Skip to content
MS
Sign in
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

Live Europe PMC search

Load 10 most recent

Nothing is fetched until you ask, so the page stays fast and the request is yours rather than automatic.

Europe PMC · ten most recent, newest first