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Article

Propionyl-CoA carboxylase pcca-1 and pccb-1 gene deletions in Caenorhabditis elegans globally impair mitochondrial energy metabolism.

Journal of inherited metabolic disease - 1 Mar 2018

Chapman Kimberly A, Ostrovsky Julian, Rao Meera, Dingley Stephen D, Polyak Erzsebet, Yudkoff Marc, Xiao Rui, Bennett Michael J, Falk Marni J

Abstract excerpt

Propionic acidemia (PA) is a classical inborn error of metabolism with high morbidity that results from the inability of the propionyl-CoA carboxylase (PCC) enzyme to convert propionyl-CoA to methylmalonyl-CoA. PA is inherited in an autosomal recessive fashion due to functional loss of both alleles of either PCCA or PCCB. These genes are highly conserved across evolutionarily diverse species and share extensive...

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Source

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Identifiers

PMID: 29159707
DOI: 10.1007/s10545-017-0111-x
PMCID: PMC5832583

Genes

PCCA
PCCB

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026.

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