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Article

Mitochondrial import and processing of wild type and type III mutant isovaleryl-CoA dehydrogenase.

The Journal of biological chemistry - 17 Mar 2000

Volchenboum S L, Vockley J

Abstract excerpt

Isovaleric acidemia is a rare inborn error of metabolism caused by a deficiency of isovaleryl-CoA dehydrogenase (IVD), a nucleus-encoded, homotetrameric, mitochondrial flavoenzyme that catalyzes the conversion of isovaleryl-CoA to 3-methylcrotonyl-CoA. We have previously identified a nucleotide deletion in the gene for IVD in fibroblasts from a patient with isovaleric acidemia leading to a shift in reading frame...

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Source

Identifiers

PMID: 10713113
DOI: 10.1074/jbc.275.11.7958

Genes

IVD

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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Mitochondrial import and processing of wild type and type III mutant isovaleryl-CoA dehydrogenase.PMID 10713113Read ↗

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