Deciphering the role of PGC-1α in neurological disorders: from mitochondrial dysfunction to synaptic failure

The onset and mechanisms underlying neurodegenerative diseases remain uncertain. The main features of neurodegenerative diseases have been related with cellular and molecular events like neuronal loss, mitochondrial dysfunction and aberrant accumulation of misfolded proteins or peptides in specific areas of the brain. The most prevalent neurodegenerative diseases belonging to age-related pathologies are Alzheimer’s disease, Huntington’s disease, Parkinson’s disease and amyotrophic lateral sclerosis. Interestingly, mitochondrial dysfunction has been observed to occur during the early onset of several neuropathological events associated to neurodegenerative diseases. The master regulator of mitochondrial quality control and energetic metabolism is the transcriptional coactivator peroxisome proliferator-activated receptor gamma coactivator 1-alpha (PGC-1α). Additionally, it has been observed that PGC-1α appears to be a key factor in maintaining neuronal survival and synaptic transmission. In fact, PGC-1α downregulation in different brain areas (hippocampus, substantia nigra, cortex, striatum and spinal cord) that occurs in function of neurological damage including oxidative stress, neuronal loss, and motor disorders has been seen in several animal and cellular models of neurodegenerative diseases. Current evidence indicates that PGC-1α upregulation may serve as a potent therapeutic approach against development and progression of neuronal damage. Remarkably, increasing evidence shows that PGC-1α deficient mice have neurodegenerative diseases-like features, as well as neurological abnormalities. Finally, we discuss recent studies showing novel specific PGC-1α isoforms in the central nervous system that appear to exert a key role in the age of onset of neurodegenerative diseases and have a neuroprotective function in the central nervous system, thus opening a new molecular strategy for treatment of neurodegenerative diseases. The purpose of this review is to provide an up-to-date overview of the PGC-1α role in the physiopathology of neurodegenerative diseases, as well as establish the importance of PGC-1α function in synaptic transmission and neuronal survival. Ausführliche Beschreibung

Gespeichert in:

Autor*in:	Jessica D Panes [verfasserIn] Aline Wendt [verfasserIn] Oscar Ramirez-Molina [verfasserIn] Patricio A Castro [verfasserIn] Jorge Fuentealba [verfasserIn]

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2022

Schlagwörter:	alzheimer’s disease; amyotrophic lateral sclerosis; huntington’s disease; mitochondrial dysfunction; parkinson’s disease; pgc-1α; synaptic function; vascular dementia

Übergeordnetes Werk:	In: Neural Regeneration Research - Wolters Kluwer Medknow Publications, 2014, 17(2022), 2, Seite 237-245
Übergeordnetes Werk:	volume:17 ; year:2022 ; number:2 ; pages:237-245

Links:	Link aufrufen Link aufrufen Link aufrufen Journal toc

DOI / URN:	10.4103/1673-5374.317957

Katalog-ID:	DOAJ053770676

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spelling	10.4103/1673-5374.317957 doi (DE-627)DOAJ053770676 (DE-599)DOAJ2e8710252cbf481ebff7f4739e7e8176 DE-627 ger DE-627 rakwb eng RC346-429 Jessica D Panes verfasserin aut Deciphering the role of PGC-1α in neurological disorders: from mitochondrial dysfunction to synaptic failure 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier The onset and mechanisms underlying neurodegenerative diseases remain uncertain. The main features of neurodegenerative diseases have been related with cellular and molecular events like neuronal loss, mitochondrial dysfunction and aberrant accumulation of misfolded proteins or peptides in specific areas of the brain. The most prevalent neurodegenerative diseases belonging to age-related pathologies are Alzheimer’s disease, Huntington’s disease, Parkinson’s disease and amyotrophic lateral sclerosis. Interestingly, mitochondrial dysfunction has been observed to occur during the early onset of several neuropathological events associated to neurodegenerative diseases. The master regulator of mitochondrial quality control and energetic metabolism is the transcriptional coactivator peroxisome proliferator-activated receptor gamma coactivator 1-alpha (PGC-1α). Additionally, it has been observed that PGC-1α appears to be a key factor in maintaining neuronal survival and synaptic transmission. In fact, PGC-1α downregulation in different brain areas (hippocampus, substantia nigra, cortex, striatum and spinal cord) that occurs in function of neurological damage including oxidative stress, neuronal loss, and motor disorders has been seen in several animal and cellular models of neurodegenerative diseases. Current evidence indicates that PGC-1α upregulation may serve as a potent therapeutic approach against development and progression of neuronal damage. Remarkably, increasing evidence shows that PGC-1α deficient mice have neurodegenerative diseases-like features, as well as neurological abnormalities. Finally, we discuss recent studies showing novel specific PGC-1α isoforms in the central nervous system that appear to exert a key role in the age of onset of neurodegenerative diseases and have a neuroprotective function in the central nervous system, thus opening a new molecular strategy for treatment of neurodegenerative diseases. The purpose of this review is to provide an up-to-date overview of the PGC-1α role in the physiopathology of neurodegenerative diseases, as well as establish the importance of PGC-1α function in synaptic transmission and neuronal survival. alzheimer’s disease; amyotrophic lateral sclerosis; huntington’s disease; mitochondrial dysfunction; parkinson’s disease; pgc-1α; synaptic function; vascular dementia Neurology. Diseases of the nervous system Aline Wendt verfasserin aut Oscar Ramirez-Molina verfasserin aut Patricio A Castro verfasserin aut Jorge Fuentealba verfasserin aut In Neural Regeneration Research Wolters Kluwer Medknow Publications, 2014 17(2022), 2, Seite 237-245 (DE-627)545785499 (DE-600)2388460-5 18767958 nnns volume:17 year:2022 number:2 pages:237-245 https://doi.org/10.4103/1673-5374.317957 kostenfrei https://doaj.org/article/2e8710252cbf481ebff7f4739e7e8176 kostenfrei http://www.nrronline.org/article.asp?issn=1673-5374;year=2022;volume=17;issue=2;spage=237;epage=245;aulast=Panes kostenfrei https://doaj.org/toc/1673-5374 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_11 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_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_374 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2014 GBV_ILN_2700 GBV_ILN_2817 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 17 2022 2 237-245
allfields_unstemmed	10.4103/1673-5374.317957 doi (DE-627)DOAJ053770676 (DE-599)DOAJ2e8710252cbf481ebff7f4739e7e8176 DE-627 ger DE-627 rakwb eng RC346-429 Jessica D Panes verfasserin aut Deciphering the role of PGC-1α in neurological disorders: from mitochondrial dysfunction to synaptic failure 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier The onset and mechanisms underlying neurodegenerative diseases remain uncertain. The main features of neurodegenerative diseases have been related with cellular and molecular events like neuronal loss, mitochondrial dysfunction and aberrant accumulation of misfolded proteins or peptides in specific areas of the brain. The most prevalent neurodegenerative diseases belonging to age-related pathologies are Alzheimer’s disease, Huntington’s disease, Parkinson’s disease and amyotrophic lateral sclerosis. Interestingly, mitochondrial dysfunction has been observed to occur during the early onset of several neuropathological events associated to neurodegenerative diseases. The master regulator of mitochondrial quality control and energetic metabolism is the transcriptional coactivator peroxisome proliferator-activated receptor gamma coactivator 1-alpha (PGC-1α). Additionally, it has been observed that PGC-1α appears to be a key factor in maintaining neuronal survival and synaptic transmission. In fact, PGC-1α downregulation in different brain areas (hippocampus, substantia nigra, cortex, striatum and spinal cord) that occurs in function of neurological damage including oxidative stress, neuronal loss, and motor disorders has been seen in several animal and cellular models of neurodegenerative diseases. Current evidence indicates that PGC-1α upregulation may serve as a potent therapeutic approach against development and progression of neuronal damage. Remarkably, increasing evidence shows that PGC-1α deficient mice have neurodegenerative diseases-like features, as well as neurological abnormalities. Finally, we discuss recent studies showing novel specific PGC-1α isoforms in the central nervous system that appear to exert a key role in the age of onset of neurodegenerative diseases and have a neuroprotective function in the central nervous system, thus opening a new molecular strategy for treatment of neurodegenerative diseases. The purpose of this review is to provide an up-to-date overview of the PGC-1α role in the physiopathology of neurodegenerative diseases, as well as establish the importance of PGC-1α function in synaptic transmission and neuronal survival. alzheimer’s disease; amyotrophic lateral sclerosis; huntington’s disease; mitochondrial dysfunction; parkinson’s disease; pgc-1α; synaptic function; vascular dementia Neurology. Diseases of the nervous system Aline Wendt verfasserin aut Oscar Ramirez-Molina verfasserin aut Patricio A Castro verfasserin aut Jorge Fuentealba verfasserin aut In Neural Regeneration Research Wolters Kluwer Medknow Publications, 2014 17(2022), 2, Seite 237-245 (DE-627)545785499 (DE-600)2388460-5 18767958 nnns volume:17 year:2022 number:2 pages:237-245 https://doi.org/10.4103/1673-5374.317957 kostenfrei https://doaj.org/article/2e8710252cbf481ebff7f4739e7e8176 kostenfrei http://www.nrronline.org/article.asp?issn=1673-5374;year=2022;volume=17;issue=2;spage=237;epage=245;aulast=Panes kostenfrei https://doaj.org/toc/1673-5374 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_11 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_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_374 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2014 GBV_ILN_2700 GBV_ILN_2817 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 17 2022 2 237-245
allfieldsGer	10.4103/1673-5374.317957 doi (DE-627)DOAJ053770676 (DE-599)DOAJ2e8710252cbf481ebff7f4739e7e8176 DE-627 ger DE-627 rakwb eng RC346-429 Jessica D Panes verfasserin aut Deciphering the role of PGC-1α in neurological disorders: from mitochondrial dysfunction to synaptic failure 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier The onset and mechanisms underlying neurodegenerative diseases remain uncertain. The main features of neurodegenerative diseases have been related with cellular and molecular events like neuronal loss, mitochondrial dysfunction and aberrant accumulation of misfolded proteins or peptides in specific areas of the brain. The most prevalent neurodegenerative diseases belonging to age-related pathologies are Alzheimer’s disease, Huntington’s disease, Parkinson’s disease and amyotrophic lateral sclerosis. Interestingly, mitochondrial dysfunction has been observed to occur during the early onset of several neuropathological events associated to neurodegenerative diseases. The master regulator of mitochondrial quality control and energetic metabolism is the transcriptional coactivator peroxisome proliferator-activated receptor gamma coactivator 1-alpha (PGC-1α). Additionally, it has been observed that PGC-1α appears to be a key factor in maintaining neuronal survival and synaptic transmission. In fact, PGC-1α downregulation in different brain areas (hippocampus, substantia nigra, cortex, striatum and spinal cord) that occurs in function of neurological damage including oxidative stress, neuronal loss, and motor disorders has been seen in several animal and cellular models of neurodegenerative diseases. Current evidence indicates that PGC-1α upregulation may serve as a potent therapeutic approach against development and progression of neuronal damage. Remarkably, increasing evidence shows that PGC-1α deficient mice have neurodegenerative diseases-like features, as well as neurological abnormalities. Finally, we discuss recent studies showing novel specific PGC-1α isoforms in the central nervous system that appear to exert a key role in the age of onset of neurodegenerative diseases and have a neuroprotective function in the central nervous system, thus opening a new molecular strategy for treatment of neurodegenerative diseases. The purpose of this review is to provide an up-to-date overview of the PGC-1α role in the physiopathology of neurodegenerative diseases, as well as establish the importance of PGC-1α function in synaptic transmission and neuronal survival. alzheimer’s disease; amyotrophic lateral sclerosis; huntington’s disease; mitochondrial dysfunction; parkinson’s disease; pgc-1α; synaptic function; vascular dementia Neurology. Diseases of the nervous system Aline Wendt verfasserin aut Oscar Ramirez-Molina verfasserin aut Patricio A Castro verfasserin aut Jorge Fuentealba verfasserin aut In Neural Regeneration Research Wolters Kluwer Medknow Publications, 2014 17(2022), 2, Seite 237-245 (DE-627)545785499 (DE-600)2388460-5 18767958 nnns volume:17 year:2022 number:2 pages:237-245 https://doi.org/10.4103/1673-5374.317957 kostenfrei https://doaj.org/article/2e8710252cbf481ebff7f4739e7e8176 kostenfrei http://www.nrronline.org/article.asp?issn=1673-5374;year=2022;volume=17;issue=2;spage=237;epage=245;aulast=Panes kostenfrei https://doaj.org/toc/1673-5374 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_11 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_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_374 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2014 GBV_ILN_2700 GBV_ILN_2817 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 17 2022 2 237-245
allfieldsSound	10.4103/1673-5374.317957 doi (DE-627)DOAJ053770676 (DE-599)DOAJ2e8710252cbf481ebff7f4739e7e8176 DE-627 ger DE-627 rakwb eng RC346-429 Jessica D Panes verfasserin aut Deciphering the role of PGC-1α in neurological disorders: from mitochondrial dysfunction to synaptic failure 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier The onset and mechanisms underlying neurodegenerative diseases remain uncertain. The main features of neurodegenerative diseases have been related with cellular and molecular events like neuronal loss, mitochondrial dysfunction and aberrant accumulation of misfolded proteins or peptides in specific areas of the brain. The most prevalent neurodegenerative diseases belonging to age-related pathologies are Alzheimer’s disease, Huntington’s disease, Parkinson’s disease and amyotrophic lateral sclerosis. Interestingly, mitochondrial dysfunction has been observed to occur during the early onset of several neuropathological events associated to neurodegenerative diseases. The master regulator of mitochondrial quality control and energetic metabolism is the transcriptional coactivator peroxisome proliferator-activated receptor gamma coactivator 1-alpha (PGC-1α). Additionally, it has been observed that PGC-1α appears to be a key factor in maintaining neuronal survival and synaptic transmission. In fact, PGC-1α downregulation in different brain areas (hippocampus, substantia nigra, cortex, striatum and spinal cord) that occurs in function of neurological damage including oxidative stress, neuronal loss, and motor disorders has been seen in several animal and cellular models of neurodegenerative diseases. Current evidence indicates that PGC-1α upregulation may serve as a potent therapeutic approach against development and progression of neuronal damage. Remarkably, increasing evidence shows that PGC-1α deficient mice have neurodegenerative diseases-like features, as well as neurological abnormalities. Finally, we discuss recent studies showing novel specific PGC-1α isoforms in the central nervous system that appear to exert a key role in the age of onset of neurodegenerative diseases and have a neuroprotective function in the central nervous system, thus opening a new molecular strategy for treatment of neurodegenerative diseases. The purpose of this review is to provide an up-to-date overview of the PGC-1α role in the physiopathology of neurodegenerative diseases, as well as establish the importance of PGC-1α function in synaptic transmission and neuronal survival. alzheimer’s disease; amyotrophic lateral sclerosis; huntington’s disease; mitochondrial dysfunction; parkinson’s disease; pgc-1α; synaptic function; vascular dementia Neurology. Diseases of the nervous system Aline Wendt verfasserin aut Oscar Ramirez-Molina verfasserin aut Patricio A Castro verfasserin aut Jorge Fuentealba verfasserin aut In Neural Regeneration Research Wolters Kluwer Medknow Publications, 2014 17(2022), 2, Seite 237-245 (DE-627)545785499 (DE-600)2388460-5 18767958 nnns volume:17 year:2022 number:2 pages:237-245 https://doi.org/10.4103/1673-5374.317957 kostenfrei https://doaj.org/article/2e8710252cbf481ebff7f4739e7e8176 kostenfrei http://www.nrronline.org/article.asp?issn=1673-5374;year=2022;volume=17;issue=2;spage=237;epage=245;aulast=Panes kostenfrei https://doaj.org/toc/1673-5374 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_11 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_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_374 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2014 GBV_ILN_2700 GBV_ILN_2817 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 17 2022 2 237-245
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source	In Neural Regeneration Research 17(2022), 2, Seite 237-245 volume:17 year:2022 number:2 pages:237-245
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topic_facet	alzheimer’s disease; amyotrophic lateral sclerosis; huntington’s disease; mitochondrial dysfunction; parkinson’s disease; pgc-1α; synaptic function; vascular dementia Neurology. Diseases of the nervous system
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author	Jessica D Panes
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topic_title	RC346-429 Deciphering the role of PGC-1α in neurological disorders: from mitochondrial dysfunction to synaptic failure alzheimer’s disease; amyotrophic lateral sclerosis; huntington’s disease; mitochondrial dysfunction; parkinson’s disease; pgc-1α; synaptic function; vascular dementia
topic	misc RC346-429 misc alzheimer’s disease; amyotrophic lateral sclerosis; huntington’s disease; mitochondrial dysfunction; parkinson’s disease; pgc-1α; synaptic function; vascular dementia misc Neurology. Diseases of the nervous system
topic_unstemmed	misc RC346-429 misc alzheimer’s disease; amyotrophic lateral sclerosis; huntington’s disease; mitochondrial dysfunction; parkinson’s disease; pgc-1α; synaptic function; vascular dementia misc Neurology. Diseases of the nervous system
topic_browse	misc RC346-429 misc alzheimer’s disease; amyotrophic lateral sclerosis; huntington’s disease; mitochondrial dysfunction; parkinson’s disease; pgc-1α; synaptic function; vascular dementia misc Neurology. Diseases of the nervous system
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title	Deciphering the role of PGC-1α in neurological disorders: from mitochondrial dysfunction to synaptic failure
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title_full	Deciphering the role of PGC-1α in neurological disorders: from mitochondrial dysfunction to synaptic failure
author_sort	Jessica D Panes
journal	Neural Regeneration Research
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author_browse	Jessica D Panes Aline Wendt Oscar Ramirez-Molina Patricio A Castro Jorge Fuentealba
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title_sort	deciphering the role of pgc-1α in neurological disorders: from mitochondrial dysfunction to synaptic failure
callnumber	RC346-429
title_auth	Deciphering the role of PGC-1α in neurological disorders: from mitochondrial dysfunction to synaptic failure
abstract	The onset and mechanisms underlying neurodegenerative diseases remain uncertain. The main features of neurodegenerative diseases have been related with cellular and molecular events like neuronal loss, mitochondrial dysfunction and aberrant accumulation of misfolded proteins or peptides in specific areas of the brain. The most prevalent neurodegenerative diseases belonging to age-related pathologies are Alzheimer’s disease, Huntington’s disease, Parkinson’s disease and amyotrophic lateral sclerosis. Interestingly, mitochondrial dysfunction has been observed to occur during the early onset of several neuropathological events associated to neurodegenerative diseases. The master regulator of mitochondrial quality control and energetic metabolism is the transcriptional coactivator peroxisome proliferator-activated receptor gamma coactivator 1-alpha (PGC-1α). Additionally, it has been observed that PGC-1α appears to be a key factor in maintaining neuronal survival and synaptic transmission. In fact, PGC-1α downregulation in different brain areas (hippocampus, substantia nigra, cortex, striatum and spinal cord) that occurs in function of neurological damage including oxidative stress, neuronal loss, and motor disorders has been seen in several animal and cellular models of neurodegenerative diseases. Current evidence indicates that PGC-1α upregulation may serve as a potent therapeutic approach against development and progression of neuronal damage. Remarkably, increasing evidence shows that PGC-1α deficient mice have neurodegenerative diseases-like features, as well as neurological abnormalities. Finally, we discuss recent studies showing novel specific PGC-1α isoforms in the central nervous system that appear to exert a key role in the age of onset of neurodegenerative diseases and have a neuroprotective function in the central nervous system, thus opening a new molecular strategy for treatment of neurodegenerative diseases. The purpose of this review is to provide an up-to-date overview of the PGC-1α role in the physiopathology of neurodegenerative diseases, as well as establish the importance of PGC-1α function in synaptic transmission and neuronal survival.
abstractGer	The onset and mechanisms underlying neurodegenerative diseases remain uncertain. The main features of neurodegenerative diseases have been related with cellular and molecular events like neuronal loss, mitochondrial dysfunction and aberrant accumulation of misfolded proteins or peptides in specific areas of the brain. The most prevalent neurodegenerative diseases belonging to age-related pathologies are Alzheimer’s disease, Huntington’s disease, Parkinson’s disease and amyotrophic lateral sclerosis. Interestingly, mitochondrial dysfunction has been observed to occur during the early onset of several neuropathological events associated to neurodegenerative diseases. The master regulator of mitochondrial quality control and energetic metabolism is the transcriptional coactivator peroxisome proliferator-activated receptor gamma coactivator 1-alpha (PGC-1α). Additionally, it has been observed that PGC-1α appears to be a key factor in maintaining neuronal survival and synaptic transmission. In fact, PGC-1α downregulation in different brain areas (hippocampus, substantia nigra, cortex, striatum and spinal cord) that occurs in function of neurological damage including oxidative stress, neuronal loss, and motor disorders has been seen in several animal and cellular models of neurodegenerative diseases. Current evidence indicates that PGC-1α upregulation may serve as a potent therapeutic approach against development and progression of neuronal damage. Remarkably, increasing evidence shows that PGC-1α deficient mice have neurodegenerative diseases-like features, as well as neurological abnormalities. Finally, we discuss recent studies showing novel specific PGC-1α isoforms in the central nervous system that appear to exert a key role in the age of onset of neurodegenerative diseases and have a neuroprotective function in the central nervous system, thus opening a new molecular strategy for treatment of neurodegenerative diseases. The purpose of this review is to provide an up-to-date overview of the PGC-1α role in the physiopathology of neurodegenerative diseases, as well as establish the importance of PGC-1α function in synaptic transmission and neuronal survival.
abstract_unstemmed	The onset and mechanisms underlying neurodegenerative diseases remain uncertain. The main features of neurodegenerative diseases have been related with cellular and molecular events like neuronal loss, mitochondrial dysfunction and aberrant accumulation of misfolded proteins or peptides in specific areas of the brain. The most prevalent neurodegenerative diseases belonging to age-related pathologies are Alzheimer’s disease, Huntington’s disease, Parkinson’s disease and amyotrophic lateral sclerosis. Interestingly, mitochondrial dysfunction has been observed to occur during the early onset of several neuropathological events associated to neurodegenerative diseases. The master regulator of mitochondrial quality control and energetic metabolism is the transcriptional coactivator peroxisome proliferator-activated receptor gamma coactivator 1-alpha (PGC-1α). Additionally, it has been observed that PGC-1α appears to be a key factor in maintaining neuronal survival and synaptic transmission. In fact, PGC-1α downregulation in different brain areas (hippocampus, substantia nigra, cortex, striatum and spinal cord) that occurs in function of neurological damage including oxidative stress, neuronal loss, and motor disorders has been seen in several animal and cellular models of neurodegenerative diseases. Current evidence indicates that PGC-1α upregulation may serve as a potent therapeutic approach against development and progression of neuronal damage. Remarkably, increasing evidence shows that PGC-1α deficient mice have neurodegenerative diseases-like features, as well as neurological abnormalities. Finally, we discuss recent studies showing novel specific PGC-1α isoforms in the central nervous system that appear to exert a key role in the age of onset of neurodegenerative diseases and have a neuroprotective function in the central nervous system, thus opening a new molecular strategy for treatment of neurodegenerative diseases. The purpose of this review is to provide an up-to-date overview of the PGC-1α role in the physiopathology of neurodegenerative diseases, as well as establish the importance of PGC-1α function in synaptic transmission and neuronal survival.
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title_short	Deciphering the role of PGC-1α in neurological disorders: from mitochondrial dysfunction to synaptic failure
url	https://doi.org/10.4103/1673-5374.317957 https://doaj.org/article/2e8710252cbf481ebff7f4739e7e8176 http://www.nrronline.org/article.asp?issn=1673-5374;year=2022;volume=17;issue=2;spage=237;epage=245;aulast=Panes https://doaj.org/toc/1673-5374
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