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Literature summary for 6.4.1.3 extracted from

  • Chapman, K.; Ostrovsky, J.; Rao, M.; Dingley, S.; Polyak, E.; Yudkoff, M.; Xiao, R.; Bennett, M.; Falk, M.
    Propionyl-CoA carboxylase pcca-1 and pccb-1 gene deletions in Caenorhabditis elegans globally impair mitochondrial energy metabolism (2017), J. Inherit. Metab. Dis., 41, 157-168 .
    View publication on PubMedView publication on EuropePMC
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Localization

Localization Comment Organism GeneOntology No. Textmining
mitochondrion
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 Caenorhabditis elegans 5739
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Organism

Organism UniProt Comment Textmining
Caenorhabditis elegans Q19842 and Q20676 Q19842 i.e. subunit PccA-1, Q20676 i.e. subunit PccB-1
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Synonyms

Synonyms Comment Organism
PccA-1
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 Caenorhabditis elegans
PccB-1
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 Caenorhabditis elegans

General Information

General Information Comment Organism
physiological function animal lifespan is significantly reduced relative to wild-type worms in mutant strains lacking either subunit PccA or PccB. Mitochondrial oxidative phosphorylation capacity and efficiency of isolated mitochondria are also significantly reduced in both mutant strains. In vivo quantitation of mitochondrial physiology is normal in PccB-1 mutants, but PccA-1 deletion mutants have significantly increased mitochondrial matrix oxidant burden as well as significantly decreased mitochondrial membrane potential and mitochondrial content. Both deletion strains have decreased accumulation of malate, isotopic enrichment in citrate, and increased lactate accumulation Caenorhabditis elegans