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rate-limiting unreviewed

Propionyl-CoA carboxylase (PCC)

1 reactions · 1 pathways

Cofactors used

ATP Bicarbonate/CO2 Biotin (B7) · B7 (Biotin)

Clinical / pharmacological. Propionic acidaemia; biotin-dependent

What it does, reaction by reaction

1 reactions

Propionyl-CoA to Succinyl-CoA (anaplerosis) Amino Acid & Nitrogen Metabolism · Mitochondrial matrix

step 1 Irreversible rate-limiting

Propionyl-CoA + HCO3- + ATP -> D-methylmalonyl-CoA + ADP + Pi

Converts Propionyl-CoA HCO3- ATP into D-Methylmalonyl-CoA ADP Pi

› Notes

Entry point for odd-chain fatty acids, valine, isoleucine, methionine and threonine. Deficiency causes propionic acidaemia.

Showing all 1 reactions.

What accelerates and inhibits it

Regulation is pathway-specific, so each context is listed separately

0 entries

No regulation recorded for this enzyme in the source documents.

Recent literature

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Europe PMC · fetched just now · sorted by publication date

  1. 1
    Novel Na<sub>V</sub>1.6-Mitochondrial Complexes in Retinal Astrocyte Pockets Sequester GABA.

    Holden JM, Calkins DJ. · 2026-11-01

    open access unreviewed
  2. 2
    Mitochondrial proteome of Acanthamoeba delineates aerobic and anaerobic pathways dynamically regulated by oxygen.

    Stefely JA, Deng FG, Chen MZ, Nguyen K, Luce BE, Fry MY, Guo XA, Maliaris K, Rivera KD, Papanastasiou M, Issa… · 2026-10-01

    open access unreviewed
  3. 3
    Overcoming hepatic tropism: Precision engineering of lipid nanoparticles for extrahepatic RNA delivery.

    Liu Y, Guo X, Hu Q, Gan C, Nie S, Xiang J, Liu Y, Zou J, Wu X, Li C, Tang Y. · 2026-08-18

    open access unreviewed
  4. 4
    LncRNA Airn preserves cardiomyocyte fatty acid oxidation to ameliorate diabetic cardiomyopathy by modulating the QKI-PPARα axis.

    Peng T, Liu M, Yu Z, Wang X, Zhang Y, Wang D, Qi B, Ma W, Ma F, Song L, Hu J, Li Y. · 2026-08-24

    open access unreviewed
  5. 5
    Biochemical and Clinical Characterization of a Patient with Partial Biotinidase Deficiency Carrying the Rare BTD c.690C>G (p.Phe230Leu) Variant: A Case Report.

    Semeraro D, Verrocchio S, Ferrante R, Rossi C, Pieragostino D, Zappacosta A, Buccolini C, Di Michele S, Natal… · 2026-09-10

    open access unreviewed
  6. 6
    Disturbances of Mitochondrial Functions and Oxidative Stress Induction in Methylmalonic and Propionic Acidemias: A Critical Role for Metabolite Accumulation.

    Amaral AU, Wajner M. · 2026-09-20

    open access unreviewed
  7. 7
    Propionic Acidemia: An Ambispective Cohort Study on Phenotypic and Genotypic Characteristics at a Tertiary Center in Vietnam.

    Quang DM, Vu CD, Nguyen KN, Nguyen NL, Mai TTC, Huong NTT, Mo DT, Phuong LT, Tran TH, Tu NH, Khoa NX, Vuong V… · 2026-09-11

    open access unreviewed
  8. 8
    RNA therapeutics: current status and future directions.

    Yang B, Sedano MJ, Perez K, Vijayaraghavan M, Reid VA, Yallapu MM, Chauhan SC, Dhandayuthapani S, Ramos EI, G… · 2026-09-24

    open access unreviewed
  9. 9
    Propionyl-CoA catabolism is a metabolic gatekeeper for fatty acid oxidation in pancreatic cancer.

    Yu W, Wang B, Meng F, Ren X, Zhou D, Wei H, Yang Z, Sun H, Jiang M, Xia T, Cui W, Fang H. · 2026-09-11

    unreviewed
  10. 10
    Oxygen-dependent subcellular redistribution of PHD3 links the hypoxic microenvironment to mitochondrial metabolic reprogramming in ccRCC.

    Sim J, Lim S, Lee C, Kim HJ, Lee W, Kim H, Lee H, Kim K, Teli MK, Jo KB, Wright J, Park JS, Lee ME, Jung M, N… · 2026-09-02

    open access unreviewed

External claims. These come from an index outside this database and are not checked against it. Treat them as leads.