Astrocytic proliferation and mitochondrial dysfunction induced by accumulated glutaric acidemia I (GAI) metabolites: Possible implications for GAI pathogenesis

Astrocytic proliferation and mitochondrial dysfunction induced by accumulated glutaric acidemia I (GAI) metabolites: Possible implications for GAI pathogenesis

Glutaric (GA) and 3-hydroxyglutaric (OHGA) acids accumulate in glutaric acidemia I (GAI), a neurometabolic disease characterized by acute striatal degeneration and chronic progressive cortical atrophy. To explore the hypothesis that astrocytes are involved in GAI pathogenesis and targets of accumula...

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Bibliographic Details
Main Authors: Silvia Olivera, Anabel Fernandez, Alexandra Latini, Juan Carlos Rosillo, Gabriela Casanova, Moacir Wajner, Patricia Cassina, Luis Barbeito
Format: Article
Language:English
Published: Elsevier 2008-12-01
Series:Neurobiology of Disease
Subjects:
Astrocyte proliferation
Glutaric acidemia I
Mitochondria dysfunction
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996108002192

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http://www.sciencedirect.com/science/article/pii/S0969996108002192

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