Dysregulation of the complement cascade in the $ hSOD1^{G93A} $transgenic mouse model of amyotrophic lateral sclerosis
Background Components of the innate immune complement system have been implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS); however, a comprehensive examination of complement expression in this disease has not been performed. This study therefore aimed to determine the expression o...
Ausführliche Beschreibung
Gespeichert in:
| Autor*in: |
Lee, John D [verfasserIn] |
|---|
| Format: |
E-Artikel |
|---|---|
| Sprache: |
Englisch |
| Erschienen: |
2013 |
|---|
| Schlagwörter: |
|---|
| Anmerkung: |
© Lee et al.; licensee BioMed Central Ltd. 2013. This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( |
|---|
| Übergeordnetes Werk: |
Enthalten in: Journal of neuroinflammation - London : BioMed Central, 2004, 10(2013), 1 vom: 26. Sept. |
|---|---|
| Übergeordnetes Werk: |
volume:10 ; year:2013 ; number:1 ; day:26 ; month:09 |
| Links: |
|---|
| DOI / URN: |
10.1186/1742-2094-10-119 |
|---|
| Katalog-ID: |
SPR029095999 |
|---|
| LEADER | 01000caa a22002652 4500 | ||
|---|---|---|---|
| 001 | SPR029095999 | ||
| 003 | DE-627 | ||
| 005 | 20230520014040.0 | ||
| 007 | cr uuu---uuuuu | ||
| 008 | 201007s2013 xx |||||o 00| ||eng c | ||
| 024 | 7 | |a 10.1186/1742-2094-10-119 |2 doi | |
| 035 | |a (DE-627)SPR029095999 | ||
| 035 | |a (SPR)1742-2094-10-119-e | ||
| 040 | |a DE-627 |b ger |c DE-627 |e rakwb | ||
| 041 | |a eng | ||
| 100 | 1 | |a Lee, John D |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Dysregulation of the complement cascade in the $ hSOD1^{G93A} $transgenic mouse model of amyotrophic lateral sclerosis |
| 264 | 1 | |c 2013 | |
| 336 | |a Text |b txt |2 rdacontent | ||
| 337 | |a Computermedien |b c |2 rdamedia | ||
| 338 | |a Online-Ressource |b cr |2 rdacarrier | ||
| 500 | |a © Lee et al.; licensee BioMed Central Ltd. 2013. This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( | ||
| 520 | |a Background Components of the innate immune complement system have been implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS); however, a comprehensive examination of complement expression in this disease has not been performed. This study therefore aimed to determine the expression of complement components (C1qB, C4, factor B, C3/C3b, C5 and CD88) and regulators (CD55 and CD59a) in the lumbar spinal cord of $ hSOD1^{G93A} $ mice during defined disease stages. Methods $ hSOD1^{G93A} $ and wild-type mice were examined at four different ages of disease progression. mRNA and protein expression of complement components and regulators were examined using quantitative PCR, western blotting and ELISA. Localisation of complement components within lumbar spinal cord was investigated using immunohistochemistry. Statistical differences between $ hSOD1^{G93A} $ and wild-type mice were analysed using a two-tailed t-test at each stage of disease progression. Results We found several early complement factors increased as disease progressed, whilst complement regulators decreased; suggesting overall increased complement activation through the classical or alternative pathways in $ hSOD1^{G93A} $ mice. CD88 was also increased during disease progression, with immunolocalisation demonstrating expression on motor neurons and increasing expression on microglia surrounding the regions of motor neuron death. Conclusions These results indicate that local complement activation and increased expression of CD88 may contribute to motor neuron death and ALS pathology in the $ hSOD1^{G93A} $ mouse. Hence, reducing complement-induced inflammation could be an important therapeutic strategy to treat ALS. | ||
| 650 | 4 | |a C1q |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a C4 |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a Factor B |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a C3 |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a C5 |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a CD55 |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a CD88 |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a Motor neuron disease |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a Neuroinflammation |7 (dpeaa)DE-He213 | |
| 700 | 1 | |a Kamaruzaman, Nur A |4 aut | |
| 700 | 1 | |a Fung, Jenny NT |4 aut | |
| 700 | 1 | |a Taylor, Stephen M |4 aut | |
| 700 | 1 | |a Turner, Bradley J |4 aut | |
| 700 | 1 | |a Atkin, Julie D |4 aut | |
| 700 | 1 | |a Woodruff, Trent M |4 aut | |
| 700 | 1 | |a Noakes, Peter G |4 aut | |
| 773 | 0 | 8 | |i Enthalten in |t Journal of neuroinflammation |d London : BioMed Central, 2004 |g 10(2013), 1 vom: 26. Sept. |w (DE-627)391784781 |w (DE-600)2156455-3 |x 1742-2094 |7 nnns |
| 773 | 1 | 8 | |g volume:10 |g year:2013 |g number:1 |g day:26 |g month:09 |
| 856 | 4 | 0 | |u https://dx.doi.org/10.1186/1742-2094-10-119 |z lizenzpflichtig |3 Volltext |
| 912 | |a GBV_USEFLAG_A | ||
| 912 | |a SYSFLAG_A | ||
| 912 | |a GBV_SPRINGER | ||
| 912 | |a SSG-OLC-PHA | ||
| 912 | |a GBV_ILN_20 | ||
| 912 | |a GBV_ILN_22 | ||
| 912 | |a GBV_ILN_23 | ||
| 912 | |a GBV_ILN_24 | ||
| 912 | |a GBV_ILN_39 | ||
| 912 | |a GBV_ILN_40 | ||
| 912 | |a GBV_ILN_60 | ||
| 912 | |a GBV_ILN_62 | ||
| 912 | |a GBV_ILN_63 | ||
| 912 | |a GBV_ILN_65 | ||
| 912 | |a GBV_ILN_69 | ||
| 912 | |a GBV_ILN_73 | ||
| 912 | |a GBV_ILN_74 | ||
| 912 | |a GBV_ILN_95 | ||
| 912 | |a GBV_ILN_105 | ||
| 912 | |a GBV_ILN_110 | ||
| 912 | |a GBV_ILN_151 | ||
| 912 | |a GBV_ILN_161 | ||
| 912 | |a GBV_ILN_170 | ||
| 912 | |a GBV_ILN_206 | ||
| 912 | |a GBV_ILN_213 | ||
| 912 | |a GBV_ILN_230 | ||
| 912 | |a GBV_ILN_285 | ||
| 912 | |a GBV_ILN_293 | ||
| 912 | |a GBV_ILN_602 | ||
| 912 | |a GBV_ILN_2005 | ||
| 912 | |a GBV_ILN_2009 | ||
| 912 | |a GBV_ILN_2011 | ||
| 912 | |a GBV_ILN_2014 | ||
| 912 | |a GBV_ILN_2055 | ||
| 912 | |a GBV_ILN_2111 | ||
| 912 | |a GBV_ILN_4012 | ||
| 912 | |a GBV_ILN_4037 | ||
| 912 | |a GBV_ILN_4112 | ||
| 912 | |a GBV_ILN_4125 | ||
| 912 | |a GBV_ILN_4126 | ||
| 912 | |a GBV_ILN_4249 | ||
| 912 | |a GBV_ILN_4305 | ||
| 912 | |a GBV_ILN_4306 | ||
| 912 | |a GBV_ILN_4307 | ||
| 912 | |a GBV_ILN_4313 | ||
| 912 | |a GBV_ILN_4322 | ||
| 912 | |a GBV_ILN_4323 | ||
| 912 | |a GBV_ILN_4324 | ||
| 912 | |a GBV_ILN_4325 | ||
| 912 | |a GBV_ILN_4338 | ||
| 912 | |a GBV_ILN_4367 | ||
| 912 | |a GBV_ILN_4700 | ||
| 951 | |a AR | ||
| 952 | |d 10 |j 2013 |e 1 |b 26 |c 09 | ||
| author_variant |
j d l jd jdl n a k na nak j n f jn jnf s m t sm smt b j t bj bjt j d a jd jda t m w tm tmw p g n pg pgn |
|---|---|
| matchkey_str |
article:17422094:2013----::yrgltooteopeetacditesdg3taseimueoeoa |
| hierarchy_sort_str |
2013 |
| publishDate |
2013 |
| allfields |
10.1186/1742-2094-10-119 doi (DE-627)SPR029095999 (SPR)1742-2094-10-119-e DE-627 ger DE-627 rakwb eng Lee, John D verfasserin aut Dysregulation of the complement cascade in the $ hSOD1^{G93A} $transgenic mouse model of amyotrophic lateral sclerosis 2013 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Lee et al.; licensee BioMed Central Ltd. 2013. This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( Background Components of the innate immune complement system have been implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS); however, a comprehensive examination of complement expression in this disease has not been performed. This study therefore aimed to determine the expression of complement components (C1qB, C4, factor B, C3/C3b, C5 and CD88) and regulators (CD55 and CD59a) in the lumbar spinal cord of $ hSOD1^{G93A} $ mice during defined disease stages. Methods $ hSOD1^{G93A} $ and wild-type mice were examined at four different ages of disease progression. mRNA and protein expression of complement components and regulators were examined using quantitative PCR, western blotting and ELISA. Localisation of complement components within lumbar spinal cord was investigated using immunohistochemistry. Statistical differences between $ hSOD1^{G93A} $ and wild-type mice were analysed using a two-tailed t-test at each stage of disease progression. Results We found several early complement factors increased as disease progressed, whilst complement regulators decreased; suggesting overall increased complement activation through the classical or alternative pathways in $ hSOD1^{G93A} $ mice. CD88 was also increased during disease progression, with immunolocalisation demonstrating expression on motor neurons and increasing expression on microglia surrounding the regions of motor neuron death. Conclusions These results indicate that local complement activation and increased expression of CD88 may contribute to motor neuron death and ALS pathology in the $ hSOD1^{G93A} $ mouse. Hence, reducing complement-induced inflammation could be an important therapeutic strategy to treat ALS. C1q (dpeaa)DE-He213 C4 (dpeaa)DE-He213 Factor B (dpeaa)DE-He213 C3 (dpeaa)DE-He213 C5 (dpeaa)DE-He213 CD55 (dpeaa)DE-He213 CD88 (dpeaa)DE-He213 Motor neuron disease (dpeaa)DE-He213 Neuroinflammation (dpeaa)DE-He213 Kamaruzaman, Nur A aut Fung, Jenny NT aut Taylor, Stephen M aut Turner, Bradley J aut Atkin, Julie D aut Woodruff, Trent M aut Noakes, Peter G aut Enthalten in Journal of neuroinflammation London : BioMed Central, 2004 10(2013), 1 vom: 26. Sept. (DE-627)391784781 (DE-600)2156455-3 1742-2094 nnns volume:10 year:2013 number:1 day:26 month:09 https://dx.doi.org/10.1186/1742-2094-10-119 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 10 2013 1 26 09 |
| spelling |
10.1186/1742-2094-10-119 doi (DE-627)SPR029095999 (SPR)1742-2094-10-119-e DE-627 ger DE-627 rakwb eng Lee, John D verfasserin aut Dysregulation of the complement cascade in the $ hSOD1^{G93A} $transgenic mouse model of amyotrophic lateral sclerosis 2013 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Lee et al.; licensee BioMed Central Ltd. 2013. This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( Background Components of the innate immune complement system have been implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS); however, a comprehensive examination of complement expression in this disease has not been performed. This study therefore aimed to determine the expression of complement components (C1qB, C4, factor B, C3/C3b, C5 and CD88) and regulators (CD55 and CD59a) in the lumbar spinal cord of $ hSOD1^{G93A} $ mice during defined disease stages. Methods $ hSOD1^{G93A} $ and wild-type mice were examined at four different ages of disease progression. mRNA and protein expression of complement components and regulators were examined using quantitative PCR, western blotting and ELISA. Localisation of complement components within lumbar spinal cord was investigated using immunohistochemistry. Statistical differences between $ hSOD1^{G93A} $ and wild-type mice were analysed using a two-tailed t-test at each stage of disease progression. Results We found several early complement factors increased as disease progressed, whilst complement regulators decreased; suggesting overall increased complement activation through the classical or alternative pathways in $ hSOD1^{G93A} $ mice. CD88 was also increased during disease progression, with immunolocalisation demonstrating expression on motor neurons and increasing expression on microglia surrounding the regions of motor neuron death. Conclusions These results indicate that local complement activation and increased expression of CD88 may contribute to motor neuron death and ALS pathology in the $ hSOD1^{G93A} $ mouse. Hence, reducing complement-induced inflammation could be an important therapeutic strategy to treat ALS. C1q (dpeaa)DE-He213 C4 (dpeaa)DE-He213 Factor B (dpeaa)DE-He213 C3 (dpeaa)DE-He213 C5 (dpeaa)DE-He213 CD55 (dpeaa)DE-He213 CD88 (dpeaa)DE-He213 Motor neuron disease (dpeaa)DE-He213 Neuroinflammation (dpeaa)DE-He213 Kamaruzaman, Nur A aut Fung, Jenny NT aut Taylor, Stephen M aut Turner, Bradley J aut Atkin, Julie D aut Woodruff, Trent M aut Noakes, Peter G aut Enthalten in Journal of neuroinflammation London : BioMed Central, 2004 10(2013), 1 vom: 26. Sept. (DE-627)391784781 (DE-600)2156455-3 1742-2094 nnns volume:10 year:2013 number:1 day:26 month:09 https://dx.doi.org/10.1186/1742-2094-10-119 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 10 2013 1 26 09 |
| allfields_unstemmed |
10.1186/1742-2094-10-119 doi (DE-627)SPR029095999 (SPR)1742-2094-10-119-e DE-627 ger DE-627 rakwb eng Lee, John D verfasserin aut Dysregulation of the complement cascade in the $ hSOD1^{G93A} $transgenic mouse model of amyotrophic lateral sclerosis 2013 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Lee et al.; licensee BioMed Central Ltd. 2013. This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( Background Components of the innate immune complement system have been implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS); however, a comprehensive examination of complement expression in this disease has not been performed. This study therefore aimed to determine the expression of complement components (C1qB, C4, factor B, C3/C3b, C5 and CD88) and regulators (CD55 and CD59a) in the lumbar spinal cord of $ hSOD1^{G93A} $ mice during defined disease stages. Methods $ hSOD1^{G93A} $ and wild-type mice were examined at four different ages of disease progression. mRNA and protein expression of complement components and regulators were examined using quantitative PCR, western blotting and ELISA. Localisation of complement components within lumbar spinal cord was investigated using immunohistochemistry. Statistical differences between $ hSOD1^{G93A} $ and wild-type mice were analysed using a two-tailed t-test at each stage of disease progression. Results We found several early complement factors increased as disease progressed, whilst complement regulators decreased; suggesting overall increased complement activation through the classical or alternative pathways in $ hSOD1^{G93A} $ mice. CD88 was also increased during disease progression, with immunolocalisation demonstrating expression on motor neurons and increasing expression on microglia surrounding the regions of motor neuron death. Conclusions These results indicate that local complement activation and increased expression of CD88 may contribute to motor neuron death and ALS pathology in the $ hSOD1^{G93A} $ mouse. Hence, reducing complement-induced inflammation could be an important therapeutic strategy to treat ALS. C1q (dpeaa)DE-He213 C4 (dpeaa)DE-He213 Factor B (dpeaa)DE-He213 C3 (dpeaa)DE-He213 C5 (dpeaa)DE-He213 CD55 (dpeaa)DE-He213 CD88 (dpeaa)DE-He213 Motor neuron disease (dpeaa)DE-He213 Neuroinflammation (dpeaa)DE-He213 Kamaruzaman, Nur A aut Fung, Jenny NT aut Taylor, Stephen M aut Turner, Bradley J aut Atkin, Julie D aut Woodruff, Trent M aut Noakes, Peter G aut Enthalten in Journal of neuroinflammation London : BioMed Central, 2004 10(2013), 1 vom: 26. Sept. (DE-627)391784781 (DE-600)2156455-3 1742-2094 nnns volume:10 year:2013 number:1 day:26 month:09 https://dx.doi.org/10.1186/1742-2094-10-119 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 10 2013 1 26 09 |
| allfieldsGer |
10.1186/1742-2094-10-119 doi (DE-627)SPR029095999 (SPR)1742-2094-10-119-e DE-627 ger DE-627 rakwb eng Lee, John D verfasserin aut Dysregulation of the complement cascade in the $ hSOD1^{G93A} $transgenic mouse model of amyotrophic lateral sclerosis 2013 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Lee et al.; licensee BioMed Central Ltd. 2013. This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( Background Components of the innate immune complement system have been implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS); however, a comprehensive examination of complement expression in this disease has not been performed. This study therefore aimed to determine the expression of complement components (C1qB, C4, factor B, C3/C3b, C5 and CD88) and regulators (CD55 and CD59a) in the lumbar spinal cord of $ hSOD1^{G93A} $ mice during defined disease stages. Methods $ hSOD1^{G93A} $ and wild-type mice were examined at four different ages of disease progression. mRNA and protein expression of complement components and regulators were examined using quantitative PCR, western blotting and ELISA. Localisation of complement components within lumbar spinal cord was investigated using immunohistochemistry. Statistical differences between $ hSOD1^{G93A} $ and wild-type mice were analysed using a two-tailed t-test at each stage of disease progression. Results We found several early complement factors increased as disease progressed, whilst complement regulators decreased; suggesting overall increased complement activation through the classical or alternative pathways in $ hSOD1^{G93A} $ mice. CD88 was also increased during disease progression, with immunolocalisation demonstrating expression on motor neurons and increasing expression on microglia surrounding the regions of motor neuron death. Conclusions These results indicate that local complement activation and increased expression of CD88 may contribute to motor neuron death and ALS pathology in the $ hSOD1^{G93A} $ mouse. Hence, reducing complement-induced inflammation could be an important therapeutic strategy to treat ALS. C1q (dpeaa)DE-He213 C4 (dpeaa)DE-He213 Factor B (dpeaa)DE-He213 C3 (dpeaa)DE-He213 C5 (dpeaa)DE-He213 CD55 (dpeaa)DE-He213 CD88 (dpeaa)DE-He213 Motor neuron disease (dpeaa)DE-He213 Neuroinflammation (dpeaa)DE-He213 Kamaruzaman, Nur A aut Fung, Jenny NT aut Taylor, Stephen M aut Turner, Bradley J aut Atkin, Julie D aut Woodruff, Trent M aut Noakes, Peter G aut Enthalten in Journal of neuroinflammation London : BioMed Central, 2004 10(2013), 1 vom: 26. Sept. (DE-627)391784781 (DE-600)2156455-3 1742-2094 nnns volume:10 year:2013 number:1 day:26 month:09 https://dx.doi.org/10.1186/1742-2094-10-119 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 10 2013 1 26 09 |
| allfieldsSound |
10.1186/1742-2094-10-119 doi (DE-627)SPR029095999 (SPR)1742-2094-10-119-e DE-627 ger DE-627 rakwb eng Lee, John D verfasserin aut Dysregulation of the complement cascade in the $ hSOD1^{G93A} $transgenic mouse model of amyotrophic lateral sclerosis 2013 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Lee et al.; licensee BioMed Central Ltd. 2013. This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( Background Components of the innate immune complement system have been implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS); however, a comprehensive examination of complement expression in this disease has not been performed. This study therefore aimed to determine the expression of complement components (C1qB, C4, factor B, C3/C3b, C5 and CD88) and regulators (CD55 and CD59a) in the lumbar spinal cord of $ hSOD1^{G93A} $ mice during defined disease stages. Methods $ hSOD1^{G93A} $ and wild-type mice were examined at four different ages of disease progression. mRNA and protein expression of complement components and regulators were examined using quantitative PCR, western blotting and ELISA. Localisation of complement components within lumbar spinal cord was investigated using immunohistochemistry. Statistical differences between $ hSOD1^{G93A} $ and wild-type mice were analysed using a two-tailed t-test at each stage of disease progression. Results We found several early complement factors increased as disease progressed, whilst complement regulators decreased; suggesting overall increased complement activation through the classical or alternative pathways in $ hSOD1^{G93A} $ mice. CD88 was also increased during disease progression, with immunolocalisation demonstrating expression on motor neurons and increasing expression on microglia surrounding the regions of motor neuron death. Conclusions These results indicate that local complement activation and increased expression of CD88 may contribute to motor neuron death and ALS pathology in the $ hSOD1^{G93A} $ mouse. Hence, reducing complement-induced inflammation could be an important therapeutic strategy to treat ALS. C1q (dpeaa)DE-He213 C4 (dpeaa)DE-He213 Factor B (dpeaa)DE-He213 C3 (dpeaa)DE-He213 C5 (dpeaa)DE-He213 CD55 (dpeaa)DE-He213 CD88 (dpeaa)DE-He213 Motor neuron disease (dpeaa)DE-He213 Neuroinflammation (dpeaa)DE-He213 Kamaruzaman, Nur A aut Fung, Jenny NT aut Taylor, Stephen M aut Turner, Bradley J aut Atkin, Julie D aut Woodruff, Trent M aut Noakes, Peter G aut Enthalten in Journal of neuroinflammation London : BioMed Central, 2004 10(2013), 1 vom: 26. Sept. (DE-627)391784781 (DE-600)2156455-3 1742-2094 nnns volume:10 year:2013 number:1 day:26 month:09 https://dx.doi.org/10.1186/1742-2094-10-119 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 10 2013 1 26 09 |
| language |
English |
| source |
Enthalten in Journal of neuroinflammation 10(2013), 1 vom: 26. Sept. volume:10 year:2013 number:1 day:26 month:09 |
| sourceStr |
Enthalten in Journal of neuroinflammation 10(2013), 1 vom: 26. Sept. volume:10 year:2013 number:1 day:26 month:09 |
| format_phy_str_mv |
Article |
| institution |
findex.gbv.de |
| topic_facet |
C1q C4 Factor B C3 C5 CD55 CD88 Motor neuron disease Neuroinflammation |
| isfreeaccess_bool |
false |
| container_title |
Journal of neuroinflammation |
| authorswithroles_txt_mv |
Lee, John D @@aut@@ Kamaruzaman, Nur A @@aut@@ Fung, Jenny NT @@aut@@ Taylor, Stephen M @@aut@@ Turner, Bradley J @@aut@@ Atkin, Julie D @@aut@@ Woodruff, Trent M @@aut@@ Noakes, Peter G @@aut@@ |
| publishDateDaySort_date |
2013-09-26T00:00:00Z |
| hierarchy_top_id |
391784781 |
| id |
SPR029095999 |
| language_de |
englisch |
| fullrecord |
<?xml version="1.0" encoding="UTF-8"?><collection xmlns="http://www.loc.gov/MARC21/slim"><record><leader>01000caa a22002652 4500</leader><controlfield tag="001">SPR029095999</controlfield><controlfield tag="003">DE-627</controlfield><controlfield tag="005">20230520014040.0</controlfield><controlfield tag="007">cr uuu---uuuuu</controlfield><controlfield tag="008">201007s2013 xx |||||o 00| ||eng c</controlfield><datafield tag="024" ind1="7" ind2=" "><subfield code="a">10.1186/1742-2094-10-119</subfield><subfield code="2">doi</subfield></datafield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(DE-627)SPR029095999</subfield></datafield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(SPR)1742-2094-10-119-e</subfield></datafield><datafield tag="040" ind1=" " ind2=" "><subfield code="a">DE-627</subfield><subfield code="b">ger</subfield><subfield code="c">DE-627</subfield><subfield code="e">rakwb</subfield></datafield><datafield tag="041" ind1=" " ind2=" "><subfield code="a">eng</subfield></datafield><datafield tag="100" ind1="1" ind2=" "><subfield code="a">Lee, John D</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="245" ind1="1" ind2="0"><subfield code="a">Dysregulation of the complement cascade in the $ hSOD1^{G93A} $transgenic mouse model of amyotrophic lateral sclerosis</subfield></datafield><datafield tag="264" ind1=" " ind2="1"><subfield code="c">2013</subfield></datafield><datafield tag="336" ind1=" " ind2=" "><subfield code="a">Text</subfield><subfield code="b">txt</subfield><subfield code="2">rdacontent</subfield></datafield><datafield tag="337" ind1=" " ind2=" "><subfield code="a">Computermedien</subfield><subfield code="b">c</subfield><subfield code="2">rdamedia</subfield></datafield><datafield tag="338" ind1=" " ind2=" "><subfield code="a">Online-Ressource</subfield><subfield code="b">cr</subfield><subfield code="2">rdacarrier</subfield></datafield><datafield tag="500" ind1=" " ind2=" "><subfield code="a">© Lee et al.; licensee BioMed Central Ltd. 2013. This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (</subfield></datafield><datafield tag="520" ind1=" " ind2=" "><subfield code="a">Background Components of the innate immune complement system have been implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS); however, a comprehensive examination of complement expression in this disease has not been performed. This study therefore aimed to determine the expression of complement components (C1qB, C4, factor B, C3/C3b, C5 and CD88) and regulators (CD55 and CD59a) in the lumbar spinal cord of $ hSOD1^{G93A} $ mice during defined disease stages. Methods $ hSOD1^{G93A} $ and wild-type mice were examined at four different ages of disease progression. mRNA and protein expression of complement components and regulators were examined using quantitative PCR, western blotting and ELISA. Localisation of complement components within lumbar spinal cord was investigated using immunohistochemistry. Statistical differences between $ hSOD1^{G93A} $ and wild-type mice were analysed using a two-tailed t-test at each stage of disease progression. Results We found several early complement factors increased as disease progressed, whilst complement regulators decreased; suggesting overall increased complement activation through the classical or alternative pathways in $ hSOD1^{G93A} $ mice. CD88 was also increased during disease progression, with immunolocalisation demonstrating expression on motor neurons and increasing expression on microglia surrounding the regions of motor neuron death. Conclusions These results indicate that local complement activation and increased expression of CD88 may contribute to motor neuron death and ALS pathology in the $ hSOD1^{G93A} $ mouse. Hence, reducing complement-induced inflammation could be an important therapeutic strategy to treat ALS.</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">C1q</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">C4</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Factor B</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">C3</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">C5</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">CD55</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">CD88</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Motor neuron disease</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Neuroinflammation</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Kamaruzaman, Nur A</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Fung, Jenny NT</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Taylor, Stephen M</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Turner, Bradley J</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Atkin, Julie D</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Woodruff, Trent M</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Noakes, Peter G</subfield><subfield code="4">aut</subfield></datafield><datafield tag="773" ind1="0" ind2="8"><subfield code="i">Enthalten in</subfield><subfield code="t">Journal of neuroinflammation</subfield><subfield code="d">London : BioMed Central, 2004</subfield><subfield code="g">10(2013), 1 vom: 26. Sept.</subfield><subfield code="w">(DE-627)391784781</subfield><subfield code="w">(DE-600)2156455-3</subfield><subfield code="x">1742-2094</subfield><subfield code="7">nnns</subfield></datafield><datafield tag="773" ind1="1" ind2="8"><subfield code="g">volume:10</subfield><subfield code="g">year:2013</subfield><subfield code="g">number:1</subfield><subfield code="g">day:26</subfield><subfield code="g">month:09</subfield></datafield><datafield tag="856" ind1="4" ind2="0"><subfield code="u">https://dx.doi.org/10.1186/1742-2094-10-119</subfield><subfield code="z">lizenzpflichtig</subfield><subfield code="3">Volltext</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_USEFLAG_A</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">SYSFLAG_A</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_SPRINGER</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">SSG-OLC-PHA</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_20</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_22</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_23</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_24</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_39</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_40</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_60</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_62</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_63</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_65</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_69</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_73</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_74</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_95</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_105</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_110</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_151</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_161</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_170</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_206</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_213</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_230</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_285</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_293</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_602</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_2005</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_2009</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_2011</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_2014</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_2055</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_2111</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4012</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4037</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4112</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4125</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4126</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4249</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4305</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4306</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4307</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4313</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4322</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4323</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4324</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4325</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4338</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4367</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4700</subfield></datafield><datafield tag="951" ind1=" " ind2=" "><subfield code="a">AR</subfield></datafield><datafield tag="952" ind1=" " ind2=" "><subfield code="d">10</subfield><subfield code="j">2013</subfield><subfield code="e">1</subfield><subfield code="b">26</subfield><subfield code="c">09</subfield></datafield></record></collection>
|
| author |
Lee, John D |
| spellingShingle |
Lee, John D misc C1q misc C4 misc Factor B misc C3 misc C5 misc CD55 misc CD88 misc Motor neuron disease misc Neuroinflammation Dysregulation of the complement cascade in the $ hSOD1^{G93A} $transgenic mouse model of amyotrophic lateral sclerosis |
| authorStr |
Lee, John D |
| ppnlink_with_tag_str_mv |
@@773@@(DE-627)391784781 |
| format |
electronic Article |
| delete_txt_mv |
keep |
| author_role |
aut aut aut aut aut aut aut aut |
| collection |
springer |
| remote_str |
true |
| illustrated |
Not Illustrated |
| issn |
1742-2094 |
| topic_title |
Dysregulation of the complement cascade in the $ hSOD1^{G93A} $transgenic mouse model of amyotrophic lateral sclerosis C1q (dpeaa)DE-He213 C4 (dpeaa)DE-He213 Factor B (dpeaa)DE-He213 C3 (dpeaa)DE-He213 C5 (dpeaa)DE-He213 CD55 (dpeaa)DE-He213 CD88 (dpeaa)DE-He213 Motor neuron disease (dpeaa)DE-He213 Neuroinflammation (dpeaa)DE-He213 |
| topic |
misc C1q misc C4 misc Factor B misc C3 misc C5 misc CD55 misc CD88 misc Motor neuron disease misc Neuroinflammation |
| topic_unstemmed |
misc C1q misc C4 misc Factor B misc C3 misc C5 misc CD55 misc CD88 misc Motor neuron disease misc Neuroinflammation |
| topic_browse |
misc C1q misc C4 misc Factor B misc C3 misc C5 misc CD55 misc CD88 misc Motor neuron disease misc Neuroinflammation |
| format_facet |
Elektronische Aufsätze Aufsätze Elektronische Ressource |
| format_main_str_mv |
Text Zeitschrift/Artikel |
| carriertype_str_mv |
cr |
| hierarchy_parent_title |
Journal of neuroinflammation |
| hierarchy_parent_id |
391784781 |
| hierarchy_top_title |
Journal of neuroinflammation |
| isfreeaccess_txt |
false |
| familylinks_str_mv |
(DE-627)391784781 (DE-600)2156455-3 |
| title |
Dysregulation of the complement cascade in the $ hSOD1^{G93A} $transgenic mouse model of amyotrophic lateral sclerosis |
| ctrlnum |
(DE-627)SPR029095999 (SPR)1742-2094-10-119-e |
| title_full |
Dysregulation of the complement cascade in the $ hSOD1^{G93A} $transgenic mouse model of amyotrophic lateral sclerosis |
| author_sort |
Lee, John D |
| journal |
Journal of neuroinflammation |
| journalStr |
Journal of neuroinflammation |
| lang_code |
eng |
| isOA_bool |
false |
| recordtype |
marc |
| publishDateSort |
2013 |
| contenttype_str_mv |
txt |
| author_browse |
Lee, John D Kamaruzaman, Nur A Fung, Jenny NT Taylor, Stephen M Turner, Bradley J Atkin, Julie D Woodruff, Trent M Noakes, Peter G |
| container_volume |
10 |
| format_se |
Elektronische Aufsätze |
| author-letter |
Lee, John D |
| doi_str_mv |
10.1186/1742-2094-10-119 |
| title_sort |
dysregulation of the complement cascade in the $ hsod1^{g93a} $transgenic mouse model of amyotrophic lateral sclerosis |
| title_auth |
Dysregulation of the complement cascade in the $ hSOD1^{G93A} $transgenic mouse model of amyotrophic lateral sclerosis |
| abstract |
Background Components of the innate immune complement system have been implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS); however, a comprehensive examination of complement expression in this disease has not been performed. This study therefore aimed to determine the expression of complement components (C1qB, C4, factor B, C3/C3b, C5 and CD88) and regulators (CD55 and CD59a) in the lumbar spinal cord of $ hSOD1^{G93A} $ mice during defined disease stages. Methods $ hSOD1^{G93A} $ and wild-type mice were examined at four different ages of disease progression. mRNA and protein expression of complement components and regulators were examined using quantitative PCR, western blotting and ELISA. Localisation of complement components within lumbar spinal cord was investigated using immunohistochemistry. Statistical differences between $ hSOD1^{G93A} $ and wild-type mice were analysed using a two-tailed t-test at each stage of disease progression. Results We found several early complement factors increased as disease progressed, whilst complement regulators decreased; suggesting overall increased complement activation through the classical or alternative pathways in $ hSOD1^{G93A} $ mice. CD88 was also increased during disease progression, with immunolocalisation demonstrating expression on motor neurons and increasing expression on microglia surrounding the regions of motor neuron death. Conclusions These results indicate that local complement activation and increased expression of CD88 may contribute to motor neuron death and ALS pathology in the $ hSOD1^{G93A} $ mouse. Hence, reducing complement-induced inflammation could be an important therapeutic strategy to treat ALS. © Lee et al.; licensee BioMed Central Ltd. 2013. This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( |
| abstractGer |
Background Components of the innate immune complement system have been implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS); however, a comprehensive examination of complement expression in this disease has not been performed. This study therefore aimed to determine the expression of complement components (C1qB, C4, factor B, C3/C3b, C5 and CD88) and regulators (CD55 and CD59a) in the lumbar spinal cord of $ hSOD1^{G93A} $ mice during defined disease stages. Methods $ hSOD1^{G93A} $ and wild-type mice were examined at four different ages of disease progression. mRNA and protein expression of complement components and regulators were examined using quantitative PCR, western blotting and ELISA. Localisation of complement components within lumbar spinal cord was investigated using immunohistochemistry. Statistical differences between $ hSOD1^{G93A} $ and wild-type mice were analysed using a two-tailed t-test at each stage of disease progression. Results We found several early complement factors increased as disease progressed, whilst complement regulators decreased; suggesting overall increased complement activation through the classical or alternative pathways in $ hSOD1^{G93A} $ mice. CD88 was also increased during disease progression, with immunolocalisation demonstrating expression on motor neurons and increasing expression on microglia surrounding the regions of motor neuron death. Conclusions These results indicate that local complement activation and increased expression of CD88 may contribute to motor neuron death and ALS pathology in the $ hSOD1^{G93A} $ mouse. Hence, reducing complement-induced inflammation could be an important therapeutic strategy to treat ALS. © Lee et al.; licensee BioMed Central Ltd. 2013. This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( |
| abstract_unstemmed |
Background Components of the innate immune complement system have been implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS); however, a comprehensive examination of complement expression in this disease has not been performed. This study therefore aimed to determine the expression of complement components (C1qB, C4, factor B, C3/C3b, C5 and CD88) and regulators (CD55 and CD59a) in the lumbar spinal cord of $ hSOD1^{G93A} $ mice during defined disease stages. Methods $ hSOD1^{G93A} $ and wild-type mice were examined at four different ages of disease progression. mRNA and protein expression of complement components and regulators were examined using quantitative PCR, western blotting and ELISA. Localisation of complement components within lumbar spinal cord was investigated using immunohistochemistry. Statistical differences between $ hSOD1^{G93A} $ and wild-type mice were analysed using a two-tailed t-test at each stage of disease progression. Results We found several early complement factors increased as disease progressed, whilst complement regulators decreased; suggesting overall increased complement activation through the classical or alternative pathways in $ hSOD1^{G93A} $ mice. CD88 was also increased during disease progression, with immunolocalisation demonstrating expression on motor neurons and increasing expression on microglia surrounding the regions of motor neuron death. Conclusions These results indicate that local complement activation and increased expression of CD88 may contribute to motor neuron death and ALS pathology in the $ hSOD1^{G93A} $ mouse. Hence, reducing complement-induced inflammation could be an important therapeutic strategy to treat ALS. © Lee et al.; licensee BioMed Central Ltd. 2013. This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( |
| collection_details |
GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 |
| container_issue |
1 |
| title_short |
Dysregulation of the complement cascade in the $ hSOD1^{G93A} $transgenic mouse model of amyotrophic lateral sclerosis |
| url |
https://dx.doi.org/10.1186/1742-2094-10-119 |
| remote_bool |
true |
| author2 |
Kamaruzaman, Nur A Fung, Jenny NT Taylor, Stephen M Turner, Bradley J Atkin, Julie D Woodruff, Trent M Noakes, Peter G |
| author2Str |
Kamaruzaman, Nur A Fung, Jenny NT Taylor, Stephen M Turner, Bradley J Atkin, Julie D Woodruff, Trent M Noakes, Peter G |
| ppnlink |
391784781 |
| mediatype_str_mv |
c |
| isOA_txt |
false |
| hochschulschrift_bool |
false |
| doi_str |
10.1186/1742-2094-10-119 |
| up_date |
2024-07-03T23:26:40.850Z |
| _version_ |
1803602310836781056 |
| fullrecord_marcxml |
<?xml version="1.0" encoding="UTF-8"?><collection xmlns="http://www.loc.gov/MARC21/slim"><record><leader>01000caa a22002652 4500</leader><controlfield tag="001">SPR029095999</controlfield><controlfield tag="003">DE-627</controlfield><controlfield tag="005">20230520014040.0</controlfield><controlfield tag="007">cr uuu---uuuuu</controlfield><controlfield tag="008">201007s2013 xx |||||o 00| ||eng c</controlfield><datafield tag="024" ind1="7" ind2=" "><subfield code="a">10.1186/1742-2094-10-119</subfield><subfield code="2">doi</subfield></datafield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(DE-627)SPR029095999</subfield></datafield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(SPR)1742-2094-10-119-e</subfield></datafield><datafield tag="040" ind1=" " ind2=" "><subfield code="a">DE-627</subfield><subfield code="b">ger</subfield><subfield code="c">DE-627</subfield><subfield code="e">rakwb</subfield></datafield><datafield tag="041" ind1=" " ind2=" "><subfield code="a">eng</subfield></datafield><datafield tag="100" ind1="1" ind2=" "><subfield code="a">Lee, John D</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="245" ind1="1" ind2="0"><subfield code="a">Dysregulation of the complement cascade in the $ hSOD1^{G93A} $transgenic mouse model of amyotrophic lateral sclerosis</subfield></datafield><datafield tag="264" ind1=" " ind2="1"><subfield code="c">2013</subfield></datafield><datafield tag="336" ind1=" " ind2=" "><subfield code="a">Text</subfield><subfield code="b">txt</subfield><subfield code="2">rdacontent</subfield></datafield><datafield tag="337" ind1=" " ind2=" "><subfield code="a">Computermedien</subfield><subfield code="b">c</subfield><subfield code="2">rdamedia</subfield></datafield><datafield tag="338" ind1=" " ind2=" "><subfield code="a">Online-Ressource</subfield><subfield code="b">cr</subfield><subfield code="2">rdacarrier</subfield></datafield><datafield tag="500" ind1=" " ind2=" "><subfield code="a">© Lee et al.; licensee BioMed Central Ltd. 2013. This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (</subfield></datafield><datafield tag="520" ind1=" " ind2=" "><subfield code="a">Background Components of the innate immune complement system have been implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS); however, a comprehensive examination of complement expression in this disease has not been performed. This study therefore aimed to determine the expression of complement components (C1qB, C4, factor B, C3/C3b, C5 and CD88) and regulators (CD55 and CD59a) in the lumbar spinal cord of $ hSOD1^{G93A} $ mice during defined disease stages. Methods $ hSOD1^{G93A} $ and wild-type mice were examined at four different ages of disease progression. mRNA and protein expression of complement components and regulators were examined using quantitative PCR, western blotting and ELISA. Localisation of complement components within lumbar spinal cord was investigated using immunohistochemistry. Statistical differences between $ hSOD1^{G93A} $ and wild-type mice were analysed using a two-tailed t-test at each stage of disease progression. Results We found several early complement factors increased as disease progressed, whilst complement regulators decreased; suggesting overall increased complement activation through the classical or alternative pathways in $ hSOD1^{G93A} $ mice. CD88 was also increased during disease progression, with immunolocalisation demonstrating expression on motor neurons and increasing expression on microglia surrounding the regions of motor neuron death. Conclusions These results indicate that local complement activation and increased expression of CD88 may contribute to motor neuron death and ALS pathology in the $ hSOD1^{G93A} $ mouse. Hence, reducing complement-induced inflammation could be an important therapeutic strategy to treat ALS.</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">C1q</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">C4</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Factor B</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">C3</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">C5</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">CD55</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">CD88</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Motor neuron disease</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Neuroinflammation</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Kamaruzaman, Nur A</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Fung, Jenny NT</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Taylor, Stephen M</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Turner, Bradley J</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Atkin, Julie D</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Woodruff, Trent M</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Noakes, Peter G</subfield><subfield code="4">aut</subfield></datafield><datafield tag="773" ind1="0" ind2="8"><subfield code="i">Enthalten in</subfield><subfield code="t">Journal of neuroinflammation</subfield><subfield code="d">London : BioMed Central, 2004</subfield><subfield code="g">10(2013), 1 vom: 26. Sept.</subfield><subfield code="w">(DE-627)391784781</subfield><subfield code="w">(DE-600)2156455-3</subfield><subfield code="x">1742-2094</subfield><subfield code="7">nnns</subfield></datafield><datafield tag="773" ind1="1" ind2="8"><subfield code="g">volume:10</subfield><subfield code="g">year:2013</subfield><subfield code="g">number:1</subfield><subfield code="g">day:26</subfield><subfield code="g">month:09</subfield></datafield><datafield tag="856" ind1="4" ind2="0"><subfield code="u">https://dx.doi.org/10.1186/1742-2094-10-119</subfield><subfield code="z">lizenzpflichtig</subfield><subfield code="3">Volltext</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_USEFLAG_A</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">SYSFLAG_A</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_SPRINGER</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">SSG-OLC-PHA</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_20</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_22</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_23</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_24</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_39</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_40</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_60</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_62</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_63</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_65</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_69</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_73</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_74</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_95</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_105</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_110</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_151</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_161</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_170</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_206</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_213</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_230</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_285</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_293</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_602</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_2005</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_2009</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_2011</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_2014</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_2055</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_2111</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4012</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4037</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4112</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4125</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4126</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4249</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4305</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4306</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4307</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4313</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4322</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4323</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4324</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4325</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4338</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4367</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4700</subfield></datafield><datafield tag="951" ind1=" " ind2=" "><subfield code="a">AR</subfield></datafield><datafield tag="952" ind1=" " ind2=" "><subfield code="d">10</subfield><subfield code="j">2013</subfield><subfield code="e">1</subfield><subfield code="b">26</subfield><subfield code="c">09</subfield></datafield></record></collection>
|
| score |
7.3989544 |