Cytoslic carboxylases translocate to mitochondrial matrix

Stable Identifier
R-HSA-3323111
Type
Reaction [omitted]
Species
Homo sapiens
Compartment
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Once biotinylated, three halocarboxylases (hCBXs) are localised to the mitochondrial matrix. The mechanism of transfer is still unclear. Pyruvate carboxylase (PC) is required for gluconeogenesis, lipogenesis, neurotransmitter synthesis and insulin secretion; Methylcrotonyl-CoA carboxylase (MCC) is required for amino acid metabolism; propionyl-CoA carboxylase (PCC) is required for odd-chain fatty acid oxidation (Ingaramo & Beckett 2012, Hiratsuka et al. 1998, Bailey et al. 2010).

Literature References
PubMed ID Title Journal Year
9630604 Identification of holocarboxylase synthetase (HCS) proteins in human placenta

Hiratsuka, M, Sakamoto, O, Li, X, Suzuki, Y, Aoki, Y, Narisawa, K

Biochim. Biophys. Acta 1998
20153287 Holocarboxylase synthetase: correlation of protein localisation with biological function

Bailey, LM, Wallace, JC, Polyak, SW

Arch. Biochem. Biophys. 2010
22123817 Selectivity in post-translational biotin addition to five human carboxylases

Ingaramo, M, Beckett, D

J. Biol. Chem. 2012
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