La maladie de Parkinson au Canada (serveur d'exploration)

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Structure and Function of Parkin, PINK1, and DJ-1, the Three Musketeers of Neuroprotection

Identifieur interne : 000C64 ( Main/Exploration ); précédent : 000C63; suivant : 000C65

Structure and Function of Parkin, PINK1, and DJ-1, the Three Musketeers of Neuroprotection

Auteurs : Jean-François Trempe [Canada] ; Edward A. Fon [Canada]

Source :

RBID : PMC:3630392

Abstract

Autosomal recessive forms of Parkinson’s disease are caused by mutations in three genes: Parkin, PINK1, and DJ-1. These genes encode for proteins with distinct enzymatic activities that may work together to confer neuroprotection. Parkin is an E3 ubiquitin ligase that has been shown to ubiquitinate substrates and to trigger proteasome-dependent degradation or autophagy, two crucial homeostatic processes in neurons. PINK1 is a mitochondrial protein kinase whose activity is required for Parkin-dependent mitophagy, a process that has been linked to neurodegeneration. Finally, DJ-1 is a protein homologous to a broad class of bacterial enzymes that may function as a sensor and modulator of reactive oxygen species, which have been implicated in neurodegenerative diseases. Here, we review the literature on the structure and biochemical functions of these three proteins.


Url:
DOI: 10.3389/fneur.2013.00038
PubMed: 23626584
PubMed Central: 3630392


Affiliations:


Links toward previous steps (curation, corpus...)


Le document en format XML

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<p>Autosomal recessive forms of Parkinson’s disease are caused by mutations in three genes:
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. These genes encode for proteins with distinct enzymatic activities that may work together to confer neuroprotection. Parkin is an E3 ubiquitin ligase that has been shown to ubiquitinate substrates and to trigger proteasome-dependent degradation or autophagy, two crucial homeostatic processes in neurons. PINK1 is a mitochondrial protein kinase whose activity is required for Parkin-dependent mitophagy, a process that has been linked to neurodegeneration. Finally, DJ-1 is a protein homologous to a broad class of bacterial enzymes that may function as a sensor and modulator of reactive oxygen species, which have been implicated in neurodegenerative diseases. Here, we review the literature on the structure and biochemical functions of these three proteins.</p>
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<li>Canada</li>
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