Treatment effect of coenzyme $ Q_{10} $ and an antioxidant cocktail in fibroblasts of patients with Sanfilippo disease
Abstract Coenzyme $ Q_{10} $ ($ CoQ_{10} $) plays a key role in the exchange of electrons in lysosomal membrane, which contributes to protons’ translocation into the lumen and to the acidification of intra-lysosomal medium, which is essential for the proteolytic function of hydrolases responsible -w...
Ausführliche Beschreibung
Gespeichert in:
| Autor*in: |
Matalonga, Leslie [verfasserIn] Arias, Angela [verfasserIn] Coll, María Josep [verfasserIn] Garcia-Villoria, Judit [verfasserIn] Gort, Laura [verfasserIn] Ribes, Antonia [verfasserIn] |
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| Format: |
E-Artikel |
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| Sprache: |
Englisch |
| Erschienen: |
2013 |
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| Schlagwörter: |
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| Übergeordnetes Werk: |
Enthalten in: Journal of inherited metabolic disease - Hoboken, NJ : Wiley, 1978, 37(2013), 3 vom: 18. Dez., Seite 439-446 |
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| Übergeordnetes Werk: |
volume:37 ; year:2013 ; number:3 ; day:18 ; month:12 ; pages:439-446 |
| Links: |
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| DOI / URN: |
10.1007/s10545-013-9668-1 |
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| Katalog-ID: |
SPR011384697 |
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| 245 | 1 | 0 | |a Treatment effect of coenzyme $ Q_{10} $ and an antioxidant cocktail in fibroblasts of patients with Sanfilippo disease |
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| 520 | |a Abstract Coenzyme $ Q_{10} $ ($ CoQ_{10} $) plays a key role in the exchange of electrons in lysosomal membrane, which contributes to protons’ translocation into the lumen and to the acidification of intra-lysosomal medium, which is essential for the proteolytic function of hydrolases responsible -when deficient- of a wide range of inherited lysosomal diseases such as Sanfilippo syndromes. Our aim was to evaluate whether treatment with $ CoQ_{10} $ or with an antioxidant cocktail (α-tocopherol, N-acetylcysteine and α-lipoic acid) were able to ameliorate the biochemical phenotype in cultured fibroblasts of Sanfilippo patients. Basal $ CoQ_{10} $ was analyzed in fibroblasts and Sanfilippo A patients showed decreased basal levels. However, no dysfunction in the $ CoQ_{10} $ biosynthesis pathways was found, revealing for the first time a secondary $ CoQ_{10} $ deficiency in Sanfilippo A fibroblasts. Cultured fibroblasts from five patients affected by Sanfilippo A and B diseases were treated with $ CoQ_{10} $ and an antioxidant cocktail. Upon $ CoQ_{10} $ treatment, none of the Sanfilippo A fibroblasts increased their residual enzymatic activity, but the two Sanfilippo B cell lines showed a statistically significant increase of their residual activity. The antioxidant treatment had no effect on the residual activity in all tested cell lines. Moreover, one Sanfilippo A and two Sanfilippo B fibroblasts showed a statistically significant reduction of glycosaminoglycans accumulation both, after 50 μmol/L $ CoQ_{10} $ and antioxidant treatment. Fibroblasts responsive to treatment enhanced their exocytosis levels. Our results are encouraging as some cellular alterations observed in Sanfilippo syndrome can be partially restored by $ CoQ_{10} $ or other antioxidant treatment in some patients. | ||
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| 650 | 4 | |a CoQ10 Level |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a CoQ10 Deficiency |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a Nonsense Mediate mRNA Decay |7 (dpeaa)DE-He213 | |
| 650 | 4 | |a Lysosomal Disease |7 (dpeaa)DE-He213 | |
| 700 | 1 | |a Arias, Angela |e verfasserin |4 aut | |
| 700 | 1 | |a Coll, María Josep |e verfasserin |4 aut | |
| 700 | 1 | |a Garcia-Villoria, Judit |e verfasserin |4 aut | |
| 700 | 1 | |a Gort, Laura |e verfasserin |4 aut | |
| 700 | 1 | |a Ribes, Antonia |e verfasserin |4 aut | |
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10.1007/s10545-013-9668-1 doi (DE-627)SPR011384697 (SPR)s10545-013-9668-1-e DE-627 ger DE-627 rakwb eng 610 ASE 44.48 bkl 44.33 bkl Matalonga, Leslie verfasserin aut Treatment effect of coenzyme $ Q_{10} $ and an antioxidant cocktail in fibroblasts of patients with Sanfilippo disease 2013 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Abstract Coenzyme $ Q_{10} $ ($ CoQ_{10} $) plays a key role in the exchange of electrons in lysosomal membrane, which contributes to protons’ translocation into the lumen and to the acidification of intra-lysosomal medium, which is essential for the proteolytic function of hydrolases responsible -when deficient- of a wide range of inherited lysosomal diseases such as Sanfilippo syndromes. Our aim was to evaluate whether treatment with $ CoQ_{10} $ or with an antioxidant cocktail (α-tocopherol, N-acetylcysteine and α-lipoic acid) were able to ameliorate the biochemical phenotype in cultured fibroblasts of Sanfilippo patients. Basal $ CoQ_{10} $ was analyzed in fibroblasts and Sanfilippo A patients showed decreased basal levels. However, no dysfunction in the $ CoQ_{10} $ biosynthesis pathways was found, revealing for the first time a secondary $ CoQ_{10} $ deficiency in Sanfilippo A fibroblasts. Cultured fibroblasts from five patients affected by Sanfilippo A and B diseases were treated with $ CoQ_{10} $ and an antioxidant cocktail. Upon $ CoQ_{10} $ treatment, none of the Sanfilippo A fibroblasts increased their residual enzymatic activity, but the two Sanfilippo B cell lines showed a statistically significant increase of their residual activity. The antioxidant treatment had no effect on the residual activity in all tested cell lines. Moreover, one Sanfilippo A and two Sanfilippo B fibroblasts showed a statistically significant reduction of glycosaminoglycans accumulation both, after 50 μmol/L $ CoQ_{10} $ and antioxidant treatment. Fibroblasts responsive to treatment enhanced their exocytosis levels. Our results are encouraging as some cellular alterations observed in Sanfilippo syndrome can be partially restored by $ CoQ_{10} $ or other antioxidant treatment in some patients. CoQ10 (dpeaa)DE-He213 CoQ10 Level (dpeaa)DE-He213 CoQ10 Deficiency (dpeaa)DE-He213 Nonsense Mediate mRNA Decay (dpeaa)DE-He213 Lysosomal Disease (dpeaa)DE-He213 Arias, Angela verfasserin aut Coll, María Josep verfasserin aut Garcia-Villoria, Judit verfasserin aut Gort, Laura verfasserin aut Ribes, Antonia verfasserin aut Enthalten in Journal of inherited metabolic disease Hoboken, NJ : Wiley, 1978 37(2013), 3 vom: 18. Dez., Seite 439-446 (DE-627)320457753 (DE-600)2006875-X 1573-2665 nnns volume:37 year:2013 number:3 day:18 month:12 pages:439-446 https://dx.doi.org/10.1007/s10545-013-9668-1 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_266 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2070 GBV_ILN_2086 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2116 GBV_ILN_2118 GBV_ILN_2119 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 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_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 44.48 ASE 44.33 ASE AR 37 2013 3 18 12 439-446 |
| spelling |
10.1007/s10545-013-9668-1 doi (DE-627)SPR011384697 (SPR)s10545-013-9668-1-e DE-627 ger DE-627 rakwb eng 610 ASE 44.48 bkl 44.33 bkl Matalonga, Leslie verfasserin aut Treatment effect of coenzyme $ Q_{10} $ and an antioxidant cocktail in fibroblasts of patients with Sanfilippo disease 2013 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Abstract Coenzyme $ Q_{10} $ ($ CoQ_{10} $) plays a key role in the exchange of electrons in lysosomal membrane, which contributes to protons’ translocation into the lumen and to the acidification of intra-lysosomal medium, which is essential for the proteolytic function of hydrolases responsible -when deficient- of a wide range of inherited lysosomal diseases such as Sanfilippo syndromes. Our aim was to evaluate whether treatment with $ CoQ_{10} $ or with an antioxidant cocktail (α-tocopherol, N-acetylcysteine and α-lipoic acid) were able to ameliorate the biochemical phenotype in cultured fibroblasts of Sanfilippo patients. Basal $ CoQ_{10} $ was analyzed in fibroblasts and Sanfilippo A patients showed decreased basal levels. However, no dysfunction in the $ CoQ_{10} $ biosynthesis pathways was found, revealing for the first time a secondary $ CoQ_{10} $ deficiency in Sanfilippo A fibroblasts. Cultured fibroblasts from five patients affected by Sanfilippo A and B diseases were treated with $ CoQ_{10} $ and an antioxidant cocktail. Upon $ CoQ_{10} $ treatment, none of the Sanfilippo A fibroblasts increased their residual enzymatic activity, but the two Sanfilippo B cell lines showed a statistically significant increase of their residual activity. The antioxidant treatment had no effect on the residual activity in all tested cell lines. Moreover, one Sanfilippo A and two Sanfilippo B fibroblasts showed a statistically significant reduction of glycosaminoglycans accumulation both, after 50 μmol/L $ CoQ_{10} $ and antioxidant treatment. Fibroblasts responsive to treatment enhanced their exocytosis levels. Our results are encouraging as some cellular alterations observed in Sanfilippo syndrome can be partially restored by $ CoQ_{10} $ or other antioxidant treatment in some patients. CoQ10 (dpeaa)DE-He213 CoQ10 Level (dpeaa)DE-He213 CoQ10 Deficiency (dpeaa)DE-He213 Nonsense Mediate mRNA Decay (dpeaa)DE-He213 Lysosomal Disease (dpeaa)DE-He213 Arias, Angela verfasserin aut Coll, María Josep verfasserin aut Garcia-Villoria, Judit verfasserin aut Gort, Laura verfasserin aut Ribes, Antonia verfasserin aut Enthalten in Journal of inherited metabolic disease Hoboken, NJ : Wiley, 1978 37(2013), 3 vom: 18. Dez., Seite 439-446 (DE-627)320457753 (DE-600)2006875-X 1573-2665 nnns volume:37 year:2013 number:3 day:18 month:12 pages:439-446 https://dx.doi.org/10.1007/s10545-013-9668-1 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_266 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2070 GBV_ILN_2086 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2116 GBV_ILN_2118 GBV_ILN_2119 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 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_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 44.48 ASE 44.33 ASE AR 37 2013 3 18 12 439-446 |
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10.1007/s10545-013-9668-1 doi (DE-627)SPR011384697 (SPR)s10545-013-9668-1-e DE-627 ger DE-627 rakwb eng 610 ASE 44.48 bkl 44.33 bkl Matalonga, Leslie verfasserin aut Treatment effect of coenzyme $ Q_{10} $ and an antioxidant cocktail in fibroblasts of patients with Sanfilippo disease 2013 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Abstract Coenzyme $ Q_{10} $ ($ CoQ_{10} $) plays a key role in the exchange of electrons in lysosomal membrane, which contributes to protons’ translocation into the lumen and to the acidification of intra-lysosomal medium, which is essential for the proteolytic function of hydrolases responsible -when deficient- of a wide range of inherited lysosomal diseases such as Sanfilippo syndromes. Our aim was to evaluate whether treatment with $ CoQ_{10} $ or with an antioxidant cocktail (α-tocopherol, N-acetylcysteine and α-lipoic acid) were able to ameliorate the biochemical phenotype in cultured fibroblasts of Sanfilippo patients. Basal $ CoQ_{10} $ was analyzed in fibroblasts and Sanfilippo A patients showed decreased basal levels. However, no dysfunction in the $ CoQ_{10} $ biosynthesis pathways was found, revealing for the first time a secondary $ CoQ_{10} $ deficiency in Sanfilippo A fibroblasts. Cultured fibroblasts from five patients affected by Sanfilippo A and B diseases were treated with $ CoQ_{10} $ and an antioxidant cocktail. Upon $ CoQ_{10} $ treatment, none of the Sanfilippo A fibroblasts increased their residual enzymatic activity, but the two Sanfilippo B cell lines showed a statistically significant increase of their residual activity. The antioxidant treatment had no effect on the residual activity in all tested cell lines. Moreover, one Sanfilippo A and two Sanfilippo B fibroblasts showed a statistically significant reduction of glycosaminoglycans accumulation both, after 50 μmol/L $ CoQ_{10} $ and antioxidant treatment. Fibroblasts responsive to treatment enhanced their exocytosis levels. Our results are encouraging as some cellular alterations observed in Sanfilippo syndrome can be partially restored by $ CoQ_{10} $ or other antioxidant treatment in some patients. CoQ10 (dpeaa)DE-He213 CoQ10 Level (dpeaa)DE-He213 CoQ10 Deficiency (dpeaa)DE-He213 Nonsense Mediate mRNA Decay (dpeaa)DE-He213 Lysosomal Disease (dpeaa)DE-He213 Arias, Angela verfasserin aut Coll, María Josep verfasserin aut Garcia-Villoria, Judit verfasserin aut Gort, Laura verfasserin aut Ribes, Antonia verfasserin aut Enthalten in Journal of inherited metabolic disease Hoboken, NJ : Wiley, 1978 37(2013), 3 vom: 18. Dez., Seite 439-446 (DE-627)320457753 (DE-600)2006875-X 1573-2665 nnns volume:37 year:2013 number:3 day:18 month:12 pages:439-446 https://dx.doi.org/10.1007/s10545-013-9668-1 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_266 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2070 GBV_ILN_2086 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2116 GBV_ILN_2118 GBV_ILN_2119 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 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_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 44.48 ASE 44.33 ASE AR 37 2013 3 18 12 439-446 |
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10.1007/s10545-013-9668-1 doi (DE-627)SPR011384697 (SPR)s10545-013-9668-1-e DE-627 ger DE-627 rakwb eng 610 ASE 44.48 bkl 44.33 bkl Matalonga, Leslie verfasserin aut Treatment effect of coenzyme $ Q_{10} $ and an antioxidant cocktail in fibroblasts of patients with Sanfilippo disease 2013 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Abstract Coenzyme $ Q_{10} $ ($ CoQ_{10} $) plays a key role in the exchange of electrons in lysosomal membrane, which contributes to protons’ translocation into the lumen and to the acidification of intra-lysosomal medium, which is essential for the proteolytic function of hydrolases responsible -when deficient- of a wide range of inherited lysosomal diseases such as Sanfilippo syndromes. Our aim was to evaluate whether treatment with $ CoQ_{10} $ or with an antioxidant cocktail (α-tocopherol, N-acetylcysteine and α-lipoic acid) were able to ameliorate the biochemical phenotype in cultured fibroblasts of Sanfilippo patients. Basal $ CoQ_{10} $ was analyzed in fibroblasts and Sanfilippo A patients showed decreased basal levels. However, no dysfunction in the $ CoQ_{10} $ biosynthesis pathways was found, revealing for the first time a secondary $ CoQ_{10} $ deficiency in Sanfilippo A fibroblasts. Cultured fibroblasts from five patients affected by Sanfilippo A and B diseases were treated with $ CoQ_{10} $ and an antioxidant cocktail. Upon $ CoQ_{10} $ treatment, none of the Sanfilippo A fibroblasts increased their residual enzymatic activity, but the two Sanfilippo B cell lines showed a statistically significant increase of their residual activity. The antioxidant treatment had no effect on the residual activity in all tested cell lines. Moreover, one Sanfilippo A and two Sanfilippo B fibroblasts showed a statistically significant reduction of glycosaminoglycans accumulation both, after 50 μmol/L $ CoQ_{10} $ and antioxidant treatment. Fibroblasts responsive to treatment enhanced their exocytosis levels. Our results are encouraging as some cellular alterations observed in Sanfilippo syndrome can be partially restored by $ CoQ_{10} $ or other antioxidant treatment in some patients. CoQ10 (dpeaa)DE-He213 CoQ10 Level (dpeaa)DE-He213 CoQ10 Deficiency (dpeaa)DE-He213 Nonsense Mediate mRNA Decay (dpeaa)DE-He213 Lysosomal Disease (dpeaa)DE-He213 Arias, Angela verfasserin aut Coll, María Josep verfasserin aut Garcia-Villoria, Judit verfasserin aut Gort, Laura verfasserin aut Ribes, Antonia verfasserin aut Enthalten in Journal of inherited metabolic disease Hoboken, NJ : Wiley, 1978 37(2013), 3 vom: 18. Dez., Seite 439-446 (DE-627)320457753 (DE-600)2006875-X 1573-2665 nnns volume:37 year:2013 number:3 day:18 month:12 pages:439-446 https://dx.doi.org/10.1007/s10545-013-9668-1 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_266 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2070 GBV_ILN_2086 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2116 GBV_ILN_2118 GBV_ILN_2119 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 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_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 44.48 ASE 44.33 ASE AR 37 2013 3 18 12 439-446 |
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10.1007/s10545-013-9668-1 doi (DE-627)SPR011384697 (SPR)s10545-013-9668-1-e DE-627 ger DE-627 rakwb eng 610 ASE 44.48 bkl 44.33 bkl Matalonga, Leslie verfasserin aut Treatment effect of coenzyme $ Q_{10} $ and an antioxidant cocktail in fibroblasts of patients with Sanfilippo disease 2013 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Abstract Coenzyme $ Q_{10} $ ($ CoQ_{10} $) plays a key role in the exchange of electrons in lysosomal membrane, which contributes to protons’ translocation into the lumen and to the acidification of intra-lysosomal medium, which is essential for the proteolytic function of hydrolases responsible -when deficient- of a wide range of inherited lysosomal diseases such as Sanfilippo syndromes. Our aim was to evaluate whether treatment with $ CoQ_{10} $ or with an antioxidant cocktail (α-tocopherol, N-acetylcysteine and α-lipoic acid) were able to ameliorate the biochemical phenotype in cultured fibroblasts of Sanfilippo patients. Basal $ CoQ_{10} $ was analyzed in fibroblasts and Sanfilippo A patients showed decreased basal levels. However, no dysfunction in the $ CoQ_{10} $ biosynthesis pathways was found, revealing for the first time a secondary $ CoQ_{10} $ deficiency in Sanfilippo A fibroblasts. Cultured fibroblasts from five patients affected by Sanfilippo A and B diseases were treated with $ CoQ_{10} $ and an antioxidant cocktail. Upon $ CoQ_{10} $ treatment, none of the Sanfilippo A fibroblasts increased their residual enzymatic activity, but the two Sanfilippo B cell lines showed a statistically significant increase of their residual activity. The antioxidant treatment had no effect on the residual activity in all tested cell lines. Moreover, one Sanfilippo A and two Sanfilippo B fibroblasts showed a statistically significant reduction of glycosaminoglycans accumulation both, after 50 μmol/L $ CoQ_{10} $ and antioxidant treatment. Fibroblasts responsive to treatment enhanced their exocytosis levels. Our results are encouraging as some cellular alterations observed in Sanfilippo syndrome can be partially restored by $ CoQ_{10} $ or other antioxidant treatment in some patients. CoQ10 (dpeaa)DE-He213 CoQ10 Level (dpeaa)DE-He213 CoQ10 Deficiency (dpeaa)DE-He213 Nonsense Mediate mRNA Decay (dpeaa)DE-He213 Lysosomal Disease (dpeaa)DE-He213 Arias, Angela verfasserin aut Coll, María Josep verfasserin aut Garcia-Villoria, Judit verfasserin aut Gort, Laura verfasserin aut Ribes, Antonia verfasserin aut Enthalten in Journal of inherited metabolic disease Hoboken, NJ : Wiley, 1978 37(2013), 3 vom: 18. Dez., Seite 439-446 (DE-627)320457753 (DE-600)2006875-X 1573-2665 nnns volume:37 year:2013 number:3 day:18 month:12 pages:439-446 https://dx.doi.org/10.1007/s10545-013-9668-1 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_266 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2070 GBV_ILN_2086 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2116 GBV_ILN_2118 GBV_ILN_2119 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 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_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 44.48 ASE 44.33 ASE AR 37 2013 3 18 12 439-446 |
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Enthalten in Journal of inherited metabolic disease 37(2013), 3 vom: 18. Dez., Seite 439-446 volume:37 year:2013 number:3 day:18 month:12 pages:439-446 |
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Enthalten in Journal of inherited metabolic disease 37(2013), 3 vom: 18. Dez., Seite 439-446 volume:37 year:2013 number:3 day:18 month:12 pages:439-446 |
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CoQ10 CoQ10 Level CoQ10 Deficiency Nonsense Mediate mRNA Decay Lysosomal Disease |
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Journal of inherited metabolic disease |
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Matalonga, Leslie @@aut@@ Arias, Angela @@aut@@ Coll, María Josep @@aut@@ Garcia-Villoria, Judit @@aut@@ Gort, Laura @@aut@@ Ribes, Antonia @@aut@@ |
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Our aim was to evaluate whether treatment with $ CoQ_{10} $ or with an antioxidant cocktail (α-tocopherol, N-acetylcysteine and α-lipoic acid) were able to ameliorate the biochemical phenotype in cultured fibroblasts of Sanfilippo patients. Basal $ CoQ_{10} $ was analyzed in fibroblasts and Sanfilippo A patients showed decreased basal levels. However, no dysfunction in the $ CoQ_{10} $ biosynthesis pathways was found, revealing for the first time a secondary $ CoQ_{10} $ deficiency in Sanfilippo A fibroblasts. Cultured fibroblasts from five patients affected by Sanfilippo A and B diseases were treated with $ CoQ_{10} $ and an antioxidant cocktail. Upon $ CoQ_{10} $ treatment, none of the Sanfilippo A fibroblasts increased their residual enzymatic activity, but the two Sanfilippo B cell lines showed a statistically significant increase of their residual activity. The antioxidant treatment had no effect on the residual activity in all tested cell lines. Moreover, one Sanfilippo A and two Sanfilippo B fibroblasts showed a statistically significant reduction of glycosaminoglycans accumulation both, after 50 μmol/L $ CoQ_{10} $ and antioxidant treatment. Fibroblasts responsive to treatment enhanced their exocytosis levels. 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Matalonga, Leslie |
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Matalonga, Leslie ddc 610 bkl 44.48 bkl 44.33 misc CoQ10 misc CoQ10 Level misc CoQ10 Deficiency misc Nonsense Mediate mRNA Decay misc Lysosomal Disease Treatment effect of coenzyme $ Q_{10} $ and an antioxidant cocktail in fibroblasts of patients with Sanfilippo disease |
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610 ASE 44.48 bkl 44.33 bkl Treatment effect of coenzyme $ Q_{10} $ and an antioxidant cocktail in fibroblasts of patients with Sanfilippo disease CoQ10 (dpeaa)DE-He213 CoQ10 Level (dpeaa)DE-He213 CoQ10 Deficiency (dpeaa)DE-He213 Nonsense Mediate mRNA Decay (dpeaa)DE-He213 Lysosomal Disease (dpeaa)DE-He213 |
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Matalonga, Leslie Arias, Angela Coll, María Josep Garcia-Villoria, Judit Gort, Laura Ribes, Antonia |
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treatment effect of coenzyme $ q_{10} $ and an antioxidant cocktail in fibroblasts of patients with sanfilippo disease |
| title_auth |
Treatment effect of coenzyme $ Q_{10} $ and an antioxidant cocktail in fibroblasts of patients with Sanfilippo disease |
| abstract |
Abstract Coenzyme $ Q_{10} $ ($ CoQ_{10} $) plays a key role in the exchange of electrons in lysosomal membrane, which contributes to protons’ translocation into the lumen and to the acidification of intra-lysosomal medium, which is essential for the proteolytic function of hydrolases responsible -when deficient- of a wide range of inherited lysosomal diseases such as Sanfilippo syndromes. Our aim was to evaluate whether treatment with $ CoQ_{10} $ or with an antioxidant cocktail (α-tocopherol, N-acetylcysteine and α-lipoic acid) were able to ameliorate the biochemical phenotype in cultured fibroblasts of Sanfilippo patients. Basal $ CoQ_{10} $ was analyzed in fibroblasts and Sanfilippo A patients showed decreased basal levels. However, no dysfunction in the $ CoQ_{10} $ biosynthesis pathways was found, revealing for the first time a secondary $ CoQ_{10} $ deficiency in Sanfilippo A fibroblasts. Cultured fibroblasts from five patients affected by Sanfilippo A and B diseases were treated with $ CoQ_{10} $ and an antioxidant cocktail. Upon $ CoQ_{10} $ treatment, none of the Sanfilippo A fibroblasts increased their residual enzymatic activity, but the two Sanfilippo B cell lines showed a statistically significant increase of their residual activity. The antioxidant treatment had no effect on the residual activity in all tested cell lines. Moreover, one Sanfilippo A and two Sanfilippo B fibroblasts showed a statistically significant reduction of glycosaminoglycans accumulation both, after 50 μmol/L $ CoQ_{10} $ and antioxidant treatment. Fibroblasts responsive to treatment enhanced their exocytosis levels. Our results are encouraging as some cellular alterations observed in Sanfilippo syndrome can be partially restored by $ CoQ_{10} $ or other antioxidant treatment in some patients. |
| abstractGer |
Abstract Coenzyme $ Q_{10} $ ($ CoQ_{10} $) plays a key role in the exchange of electrons in lysosomal membrane, which contributes to protons’ translocation into the lumen and to the acidification of intra-lysosomal medium, which is essential for the proteolytic function of hydrolases responsible -when deficient- of a wide range of inherited lysosomal diseases such as Sanfilippo syndromes. Our aim was to evaluate whether treatment with $ CoQ_{10} $ or with an antioxidant cocktail (α-tocopherol, N-acetylcysteine and α-lipoic acid) were able to ameliorate the biochemical phenotype in cultured fibroblasts of Sanfilippo patients. Basal $ CoQ_{10} $ was analyzed in fibroblasts and Sanfilippo A patients showed decreased basal levels. However, no dysfunction in the $ CoQ_{10} $ biosynthesis pathways was found, revealing for the first time a secondary $ CoQ_{10} $ deficiency in Sanfilippo A fibroblasts. Cultured fibroblasts from five patients affected by Sanfilippo A and B diseases were treated with $ CoQ_{10} $ and an antioxidant cocktail. Upon $ CoQ_{10} $ treatment, none of the Sanfilippo A fibroblasts increased their residual enzymatic activity, but the two Sanfilippo B cell lines showed a statistically significant increase of their residual activity. The antioxidant treatment had no effect on the residual activity in all tested cell lines. Moreover, one Sanfilippo A and two Sanfilippo B fibroblasts showed a statistically significant reduction of glycosaminoglycans accumulation both, after 50 μmol/L $ CoQ_{10} $ and antioxidant treatment. Fibroblasts responsive to treatment enhanced their exocytosis levels. Our results are encouraging as some cellular alterations observed in Sanfilippo syndrome can be partially restored by $ CoQ_{10} $ or other antioxidant treatment in some patients. |
| abstract_unstemmed |
Abstract Coenzyme $ Q_{10} $ ($ CoQ_{10} $) plays a key role in the exchange of electrons in lysosomal membrane, which contributes to protons’ translocation into the lumen and to the acidification of intra-lysosomal medium, which is essential for the proteolytic function of hydrolases responsible -when deficient- of a wide range of inherited lysosomal diseases such as Sanfilippo syndromes. Our aim was to evaluate whether treatment with $ CoQ_{10} $ or with an antioxidant cocktail (α-tocopherol, N-acetylcysteine and α-lipoic acid) were able to ameliorate the biochemical phenotype in cultured fibroblasts of Sanfilippo patients. Basal $ CoQ_{10} $ was analyzed in fibroblasts and Sanfilippo A patients showed decreased basal levels. However, no dysfunction in the $ CoQ_{10} $ biosynthesis pathways was found, revealing for the first time a secondary $ CoQ_{10} $ deficiency in Sanfilippo A fibroblasts. Cultured fibroblasts from five patients affected by Sanfilippo A and B diseases were treated with $ CoQ_{10} $ and an antioxidant cocktail. Upon $ CoQ_{10} $ treatment, none of the Sanfilippo A fibroblasts increased their residual enzymatic activity, but the two Sanfilippo B cell lines showed a statistically significant increase of their residual activity. The antioxidant treatment had no effect on the residual activity in all tested cell lines. Moreover, one Sanfilippo A and two Sanfilippo B fibroblasts showed a statistically significant reduction of glycosaminoglycans accumulation both, after 50 μmol/L $ CoQ_{10} $ and antioxidant treatment. Fibroblasts responsive to treatment enhanced their exocytosis levels. Our results are encouraging as some cellular alterations observed in Sanfilippo syndrome can be partially restored by $ CoQ_{10} $ or other antioxidant treatment in some patients. |
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Treatment effect of coenzyme $ Q_{10} $ and an antioxidant cocktail in fibroblasts of patients with Sanfilippo disease |
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| score |
7.398367 |