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Interaction of Glutaric Aciduria Type 1-Related glutaryl-CoA Dehydrogenase with Mitochondrial Matrix Proteins

Glutaric aciduria type 1 (GA1) is an inherited neurometabolic disorder caused by mutations in the GCDH gene encoding glutaryl-CoA dehydrogenase (GCDH), which forms homo- and heteromeric complexes in the mitochondrial matrix. GA1 patients are prone to the development of encephalopathic crises which l...

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Autori principali: Schmiesing, Jessica, Schlüter, Hartmut, Ullrich, Kurt, Braulke, Thomas, Mühlhausen, Chris
Natura: Artigo
Lingua:Inglês
Pubblicazione: Public Library of Science 2014
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC3912011/
https://ncbi.nlm.nih.gov/pubmed/24498361
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0087715
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