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Article

Propionyl-CoA carboxylase - A review.

Molecular genetics and metabolism - 1 Dec 2017

Wongkittichote Parith, Ah Mew Nicholas, Chapman Kimberly A

Abstract excerpt

Propionyl-CoA carboxylase (PCC) is the enzyme which catalyzes the carboxylation of propionyl-CoA to methylmalonyl-CoA and is encoded by the genes PCCA and PCCB to form a hetero-dodecamer. Dysfunction of PCC leads to the inherited metabolic disorder propionic acidemia, which can result in an affected individual presenting with metabolic acidosis, hyperammonemia, lethargy, vomiting and sometimes coma and death if...

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Source

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Identifiers

PMID: 29033250
DOI: 10.1016/j.ymgme.2017.10.002
PMCID: PMC5725275

Genes

PCCA
PCCB

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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