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...
Main Authors: | , , , , |
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Format: | Online |
Language: | English |
Published: |
Public Library of Science
2014
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Online Access: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3912011/ |