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PW-054 Amino Acid & Nitrogen Metabolism Anaplerotic unreviewed

Propionyl-CoA to Succinyl-CoA (anaplerosis)

Propionyl-CoA (odd-chain FA, Val, Ile, Met, Thr) Succinyl-CoA (TCA / gluconeogenesis)
Compartment
Mitochondrial matrix
Main tissue
Liver, most tissues
Rate-limiting
Propionyl-CoA carboxylase (PCC)
Steps
3

Reaction steps

In source order, 3 total

showing 1–3
  1. 1

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

    Propionyl-CoA carboxylase (PCC) 6.4.1.3 ST-0376 Irreversible rate-limiting Biotin (B7) ATP Bicarbonate/CO2
    In Propionyl-CoA HCO3- ATP
    Out D-Methylmalonyl-CoA ADP Pi
    › Notes

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

  2. 2

    D-Methylmalonyl-CoA <-> L-methylmalonyl-CoA

    Methylmalonyl-CoA epimerase (racemase) 5.1.99.1 ST-0377 Reversible
    In D-Methylmalonyl-CoA <
    Out L-Methylmalonyl-CoA
  3. 3

    L-Methylmalonyl-CoA -> succinyl-CoA

    Methylmalonyl-CoA mutase (MMUT) 5.4.99.2 ST-0378 Irreversible/directional Cobalamin (B12)
    In L-Methylmalonyl-CoA
    Out Succinyl-CoA
    › Notes

    Requires adenosylcobalamin. B12 deficiency raises serum methylmalonic acid, which distinguishes it from folate deficiency; inherited defects cause methylmalonic acidaemia.

Showing all 3 steps.

Recent literature

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Europe PMC · from cache · sorted by publication date

  1. 1
    Lactylation-driven therapeutic resistance in cancer: Mechanisms and therapeutic opportunities.

    Zhang W, Huang G, Tang W, Li J, Chen J, Feng Y, Li K, Pan C, Li S, Zhang H, Ye R, Long H, Yi GZ. · 2025-11-17

    open access unreviewed
  2. 2
    Hepatic ketogenesis is not required for exercise training to mitigate diet-induced liver steatosis in male mice.

    Vang CM, Ortega AF, Pfeiffer RE, Hartmann JL, Hampton GS, Wang H, Queathem ED, Crawford PA, Han X, Hughey CC. · 2026-05-14

    open access unreviewed
  3. 3
    Genome Sequence and Phylogenetic Analysis of the Sulfide-Oxidizing Heliobacterium "&lt;i&gt;Heliomicrobium sulfidophilum&lt;/i&gt;" Strain BR4.

    Kyndt JA, Petrova KO, Toshchakov SV, Bryantseva IA. · 2026-05-21

    open access unreviewed
  4. 4
    Unrestrained fatty acid oxidation triggers heart failure in mice via cardiolipin loss and mitochondrial dysfunction.

    Kim CW, Vale G, Fu X, McDonald JG, Dai C, Li C, Wang ZV, Sharma G, Khemtong C, Malloy CR, Deja S, Burgess SC,… · 2026-05-01

    cited 2× open access unreviewed
  5. 5
    Acetate uptake alleviates propionate-mediated growth restriction in &lt;i&gt;Yersinia enterocolitica&lt;/i&gt;.

    Muramatsu MK, Cole D, Perez-Orozco SJ, Winter SE. · 2026-04-15

    open access unreviewed
  6. 6
    Metagenomic insights into the effects of Clostridium butyricum and Bacillus subtilis probiotics on the gut microbiome and metabolic pathways of industrial broilers in Thailand.

    Sitthipunya A, Uthaipaisanwong P, Sinwat N, Kanjanavaikoon K, Cheevadhanarak S, Kusonmano K. · 2026-01-07

    open access unreviewed
  7. 7
    Elevated branched-chain amino acids linked to hippocampal volumes and cognitive improvement in mild cognitive impairment.

    Huang H, Cheng S, Lu W, Gao X, Liu Z, Alzheimer’s Disease Neuroimaging Initiative. · 2026-02-14

    open access unreviewed
  8. 8
    Metabolism of bile salts in the estrogen degrading bacterium Caenibius tardaugens.

    Ibero J, Hernández-Fernández G, García JL, Galán B. · 2026-02-07

    open access unreviewed
  9. 9
    Depletion of individual dietary amino acids induce distinct metabolic and chromatin states.

    Haws SA, Liu Y, Green CL, Chaiyakul K, Mishra P, Babygirija R, Armstrong EA, Mehendale AT, Ong IM, Lamming DW… · 2025-12-17

    cited 3× open access unreviewed
  10. 10
    Serum metabolomics identifies metabolic changes in obese cats fed enzymatically hydrolyzed poultry byproduct meal.

    de Andrade Príncipe L, Marchi PH, Micheletti CD, de Campos Valim JMB, Cônsolo NRB, Pedreira RS, Jeremias JT, … · 2026-01-01

    open access unreviewed

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