Early Dysfunction of Substantia Nigra Dopamine Neurons in the ParkinQ311X Mouse
Mutations in the <i<PARK2</i< gene encoding the protein parkin cause autosomal recessive juvenile parkinsonism (ARJP), a neurodegenerative disease characterized by early dysfunction and loss of dopamine (DA) neurons in the substantia nigra pars compacta (SNc). No therapy is currently ava...
Ausführliche Beschreibung
Gespeichert in:
| Autor*in: |
Maria Regoni [verfasserIn] Letizia Zanetti [verfasserIn] Stefano Comai [verfasserIn] Daniela Mercatelli [verfasserIn] Salvatore Novello [verfasserIn] Federica Albanese [verfasserIn] Laura Croci [verfasserIn] Gian Giacomo Consalez [verfasserIn] Andrea Ciammola [verfasserIn] Flavia Valtorta [verfasserIn] Michele Morari [verfasserIn] Jenny Sassone [verfasserIn] |
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| Format: |
E-Artikel |
|---|---|
| Sprache: |
Englisch |
| Erschienen: |
2021 |
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| Schlagwörter: |
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| Übergeordnetes Werk: |
In: Biomedicines - MDPI AG, 2014, 9(2021), 5, p 514 |
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| Übergeordnetes Werk: |
volume:9 ; year:2021 ; number:5, p 514 |
| Links: |
|---|
| DOI / URN: |
10.3390/biomedicines9050514 |
|---|
| Katalog-ID: |
DOAJ062068733 |
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| 520 | |a Mutations in the <i<PARK2</i< gene encoding the protein parkin cause autosomal recessive juvenile parkinsonism (ARJP), a neurodegenerative disease characterized by early dysfunction and loss of dopamine (DA) neurons in the substantia nigra pars compacta (SNc). No therapy is currently available to prevent or slow down the neurodegeneration in ARJP patients. Preclinical models are key to clarifying the early events that lead to neurodegeneration and reveal the potential of novel neuroprotective strategies. ParkinQ311X is a transgenic mouse model expressing in DA neurons a mutant parkin variant found in ARJP patients. This model was previously reported to show the neuropathological hallmark of the disease, i.e., the progressive loss of DA neurons. However, the early dysfunctions that precede neurodegeneration have never been investigated. Here, we analyzed SNc DA neurons in parkinQ311X mice and found early features of mitochondrial dysfunction, extensive cytoplasmic vacuolization, and dysregulation of spontaneous in vivo firing activity. These data suggest that the parkinQ311X mouse recapitulates key features of ARJP and provides a useful tool for studying the neurodegenerative mechanisms underlying the human disease and for screening potential neuroprotective drugs. | ||
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10.3390/biomedicines9050514 doi (DE-627)DOAJ062068733 (DE-599)DOAJ12033736213a4a09a364896d9ec296cc DE-627 ger DE-627 rakwb eng QH301-705.5 Maria Regoni verfasserin aut Early Dysfunction of Substantia Nigra Dopamine Neurons in the ParkinQ311X Mouse 2021 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Mutations in the <i<PARK2</i< gene encoding the protein parkin cause autosomal recessive juvenile parkinsonism (ARJP), a neurodegenerative disease characterized by early dysfunction and loss of dopamine (DA) neurons in the substantia nigra pars compacta (SNc). No therapy is currently available to prevent or slow down the neurodegeneration in ARJP patients. Preclinical models are key to clarifying the early events that lead to neurodegeneration and reveal the potential of novel neuroprotective strategies. ParkinQ311X is a transgenic mouse model expressing in DA neurons a mutant parkin variant found in ARJP patients. This model was previously reported to show the neuropathological hallmark of the disease, i.e., the progressive loss of DA neurons. However, the early dysfunctions that precede neurodegeneration have never been investigated. Here, we analyzed SNc DA neurons in parkinQ311X mice and found early features of mitochondrial dysfunction, extensive cytoplasmic vacuolization, and dysregulation of spontaneous in vivo firing activity. These data suggest that the parkinQ311X mouse recapitulates key features of ARJP and provides a useful tool for studying the neurodegenerative mechanisms underlying the human disease and for screening potential neuroprotective drugs. Parkinson’s disease dopaminergic neurons parkinQ311X mouse early dysfunction mitochondria cytoplasmic vacuolization Biology (General) Letizia Zanetti verfasserin aut Stefano Comai verfasserin aut Daniela Mercatelli verfasserin aut Salvatore Novello verfasserin aut Federica Albanese verfasserin aut Laura Croci verfasserin aut Gian Giacomo Consalez verfasserin aut Andrea Ciammola verfasserin aut Flavia Valtorta verfasserin aut Michele Morari verfasserin aut Jenny Sassone verfasserin aut In Biomedicines MDPI AG, 2014 9(2021), 5, p 514 (DE-627)750370483 (DE-600)2720867-9 22279059 nnns volume:9 year:2021 number:5, p 514 https://doi.org/10.3390/biomedicines9050514 kostenfrei https://doaj.org/article/12033736213a4a09a364896d9ec296cc kostenfrei https://www.mdpi.com/2227-9059/9/5/514 kostenfrei https://doaj.org/toc/2227-9059 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 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_2014 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 9 2021 5, p 514 |
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10.3390/biomedicines9050514 doi (DE-627)DOAJ062068733 (DE-599)DOAJ12033736213a4a09a364896d9ec296cc DE-627 ger DE-627 rakwb eng QH301-705.5 Maria Regoni verfasserin aut Early Dysfunction of Substantia Nigra Dopamine Neurons in the ParkinQ311X Mouse 2021 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Mutations in the <i<PARK2</i< gene encoding the protein parkin cause autosomal recessive juvenile parkinsonism (ARJP), a neurodegenerative disease characterized by early dysfunction and loss of dopamine (DA) neurons in the substantia nigra pars compacta (SNc). No therapy is currently available to prevent or slow down the neurodegeneration in ARJP patients. Preclinical models are key to clarifying the early events that lead to neurodegeneration and reveal the potential of novel neuroprotective strategies. ParkinQ311X is a transgenic mouse model expressing in DA neurons a mutant parkin variant found in ARJP patients. This model was previously reported to show the neuropathological hallmark of the disease, i.e., the progressive loss of DA neurons. However, the early dysfunctions that precede neurodegeneration have never been investigated. Here, we analyzed SNc DA neurons in parkinQ311X mice and found early features of mitochondrial dysfunction, extensive cytoplasmic vacuolization, and dysregulation of spontaneous in vivo firing activity. These data suggest that the parkinQ311X mouse recapitulates key features of ARJP and provides a useful tool for studying the neurodegenerative mechanisms underlying the human disease and for screening potential neuroprotective drugs. Parkinson’s disease dopaminergic neurons parkinQ311X mouse early dysfunction mitochondria cytoplasmic vacuolization Biology (General) Letizia Zanetti verfasserin aut Stefano Comai verfasserin aut Daniela Mercatelli verfasserin aut Salvatore Novello verfasserin aut Federica Albanese verfasserin aut Laura Croci verfasserin aut Gian Giacomo Consalez verfasserin aut Andrea Ciammola verfasserin aut Flavia Valtorta verfasserin aut Michele Morari verfasserin aut Jenny Sassone verfasserin aut In Biomedicines MDPI AG, 2014 9(2021), 5, p 514 (DE-627)750370483 (DE-600)2720867-9 22279059 nnns volume:9 year:2021 number:5, p 514 https://doi.org/10.3390/biomedicines9050514 kostenfrei https://doaj.org/article/12033736213a4a09a364896d9ec296cc kostenfrei https://www.mdpi.com/2227-9059/9/5/514 kostenfrei https://doaj.org/toc/2227-9059 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 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_2014 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 9 2021 5, p 514 |
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10.3390/biomedicines9050514 doi (DE-627)DOAJ062068733 (DE-599)DOAJ12033736213a4a09a364896d9ec296cc DE-627 ger DE-627 rakwb eng QH301-705.5 Maria Regoni verfasserin aut Early Dysfunction of Substantia Nigra Dopamine Neurons in the ParkinQ311X Mouse 2021 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Mutations in the <i<PARK2</i< gene encoding the protein parkin cause autosomal recessive juvenile parkinsonism (ARJP), a neurodegenerative disease characterized by early dysfunction and loss of dopamine (DA) neurons in the substantia nigra pars compacta (SNc). No therapy is currently available to prevent or slow down the neurodegeneration in ARJP patients. Preclinical models are key to clarifying the early events that lead to neurodegeneration and reveal the potential of novel neuroprotective strategies. ParkinQ311X is a transgenic mouse model expressing in DA neurons a mutant parkin variant found in ARJP patients. This model was previously reported to show the neuropathological hallmark of the disease, i.e., the progressive loss of DA neurons. However, the early dysfunctions that precede neurodegeneration have never been investigated. Here, we analyzed SNc DA neurons in parkinQ311X mice and found early features of mitochondrial dysfunction, extensive cytoplasmic vacuolization, and dysregulation of spontaneous in vivo firing activity. These data suggest that the parkinQ311X mouse recapitulates key features of ARJP and provides a useful tool for studying the neurodegenerative mechanisms underlying the human disease and for screening potential neuroprotective drugs. Parkinson’s disease dopaminergic neurons parkinQ311X mouse early dysfunction mitochondria cytoplasmic vacuolization Biology (General) Letizia Zanetti verfasserin aut Stefano Comai verfasserin aut Daniela Mercatelli verfasserin aut Salvatore Novello verfasserin aut Federica Albanese verfasserin aut Laura Croci verfasserin aut Gian Giacomo Consalez verfasserin aut Andrea Ciammola verfasserin aut Flavia Valtorta verfasserin aut Michele Morari verfasserin aut Jenny Sassone verfasserin aut In Biomedicines MDPI AG, 2014 9(2021), 5, p 514 (DE-627)750370483 (DE-600)2720867-9 22279059 nnns volume:9 year:2021 number:5, p 514 https://doi.org/10.3390/biomedicines9050514 kostenfrei https://doaj.org/article/12033736213a4a09a364896d9ec296cc kostenfrei https://www.mdpi.com/2227-9059/9/5/514 kostenfrei https://doaj.org/toc/2227-9059 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 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_2014 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 9 2021 5, p 514 |
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Early Dysfunction of Substantia Nigra Dopamine Neurons in the ParkinQ311X Mouse |
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Mutations in the <i<PARK2</i< gene encoding the protein parkin cause autosomal recessive juvenile parkinsonism (ARJP), a neurodegenerative disease characterized by early dysfunction and loss of dopamine (DA) neurons in the substantia nigra pars compacta (SNc). No therapy is currently available to prevent or slow down the neurodegeneration in ARJP patients. Preclinical models are key to clarifying the early events that lead to neurodegeneration and reveal the potential of novel neuroprotective strategies. ParkinQ311X is a transgenic mouse model expressing in DA neurons a mutant parkin variant found in ARJP patients. This model was previously reported to show the neuropathological hallmark of the disease, i.e., the progressive loss of DA neurons. However, the early dysfunctions that precede neurodegeneration have never been investigated. Here, we analyzed SNc DA neurons in parkinQ311X mice and found early features of mitochondrial dysfunction, extensive cytoplasmic vacuolization, and dysregulation of spontaneous in vivo firing activity. These data suggest that the parkinQ311X mouse recapitulates key features of ARJP and provides a useful tool for studying the neurodegenerative mechanisms underlying the human disease and for screening potential neuroprotective drugs. |
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Mutations in the <i<PARK2</i< gene encoding the protein parkin cause autosomal recessive juvenile parkinsonism (ARJP), a neurodegenerative disease characterized by early dysfunction and loss of dopamine (DA) neurons in the substantia nigra pars compacta (SNc). No therapy is currently available to prevent or slow down the neurodegeneration in ARJP patients. Preclinical models are key to clarifying the early events that lead to neurodegeneration and reveal the potential of novel neuroprotective strategies. ParkinQ311X is a transgenic mouse model expressing in DA neurons a mutant parkin variant found in ARJP patients. This model was previously reported to show the neuropathological hallmark of the disease, i.e., the progressive loss of DA neurons. However, the early dysfunctions that precede neurodegeneration have never been investigated. Here, we analyzed SNc DA neurons in parkinQ311X mice and found early features of mitochondrial dysfunction, extensive cytoplasmic vacuolization, and dysregulation of spontaneous in vivo firing activity. These data suggest that the parkinQ311X mouse recapitulates key features of ARJP and provides a useful tool for studying the neurodegenerative mechanisms underlying the human disease and for screening potential neuroprotective drugs. |
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Mutations in the <i<PARK2</i< gene encoding the protein parkin cause autosomal recessive juvenile parkinsonism (ARJP), a neurodegenerative disease characterized by early dysfunction and loss of dopamine (DA) neurons in the substantia nigra pars compacta (SNc). No therapy is currently available to prevent or slow down the neurodegeneration in ARJP patients. Preclinical models are key to clarifying the early events that lead to neurodegeneration and reveal the potential of novel neuroprotective strategies. ParkinQ311X is a transgenic mouse model expressing in DA neurons a mutant parkin variant found in ARJP patients. This model was previously reported to show the neuropathological hallmark of the disease, i.e., the progressive loss of DA neurons. However, the early dysfunctions that precede neurodegeneration have never been investigated. Here, we analyzed SNc DA neurons in parkinQ311X mice and found early features of mitochondrial dysfunction, extensive cytoplasmic vacuolization, and dysregulation of spontaneous in vivo firing activity. These data suggest that the parkinQ311X mouse recapitulates key features of ARJP and provides a useful tool for studying the neurodegenerative mechanisms underlying the human disease and for screening potential neuroprotective drugs. |
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No therapy is currently available to prevent or slow down the neurodegeneration in ARJP patients. Preclinical models are key to clarifying the early events that lead to neurodegeneration and reveal the potential of novel neuroprotective strategies. ParkinQ311X is a transgenic mouse model expressing in DA neurons a mutant parkin variant found in ARJP patients. This model was previously reported to show the neuropathological hallmark of the disease, i.e., the progressive loss of DA neurons. However, the early dysfunctions that precede neurodegeneration have never been investigated. Here, we analyzed SNc DA neurons in parkinQ311X mice and found early features of mitochondrial dysfunction, extensive cytoplasmic vacuolization, and dysregulation of spontaneous in vivo firing activity. 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