Interaction of Glutaric Aciduria Type 1-Related glutaryl-CoA Dehydrogenase with Mitochondrial Matrix...
Interaction of Glutaric Aciduria Type 1-Related glutaryl-CoA Dehydrogenase with Mitochondrial Matrix Proteins
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United States: Public Library of Science
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Language
English
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Publisher
United States: Public Library of Science
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Contents
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 lead to an irreversible disabling dystonic movement...
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Full title
Interaction of Glutaric Aciduria Type 1-Related glutaryl-CoA Dehydrogenase with Mitochondrial Matrix Proteins
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TN_cdi_plos_journals_1494055435
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https://devfeature-collection.sl.nsw.gov.au/record/TN_cdi_plos_journals_1494055435
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ISSN
1932-6203
E-ISSN
1932-6203
DOI
10.1371/journal.pone.0087715