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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...
Autores principales: | Schmiesing, Jessica, Schlüter, Hartmut, Ullrich, Kurt, Braulke, Thomas, Mühlhausen, Chris |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3912011/ https://www.ncbi.nlm.nih.gov/pubmed/24498361 http://dx.doi.org/10.1371/journal.pone.0087715 |
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