Propionyl-CoA carboxylase (PCC)
Cofactors used
Clinical / pharmacological. Propionic acidaemia; biotin-dependent
What it does, reaction by reaction
Propionyl-CoA to Succinyl-CoA (anaplerosis) Amino Acid & Nitrogen Metabolism · Mitochondrial matrix
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
No regulation recorded for this enzyme in the source documents.
Recent literature
Europe PMC · fetched just now · sorted by publication date
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1
Targeted gene editing of <i>PCCA</i> pseudoexon using CRISPR-Cas12a for potential therapy in propionic acidemia.
Álvarez M, Del Álamo JV, Richard E, Desviat LR. · 2026-06-17
unreviewed -
2
Long Term Follow‐Up After Transplantation in Propionic Acidemia: A Retrospective French Pediatric and Adult Cohort Study
Mekdade T, Bérat C, Schiff M, Gaschignard M, Bouchereau J, Arnoux J, Francoz C, Servais A, Dao M, Lacroix V, … · 2026-06-25
open access unreviewed -
3
Holocarboxylase synthetase deficiency: pathogenesis, clinical features, diagnosis, treatment, and research prospects.
ZeZhao J, Ablimit A. · 2026-06-22
unreviewed -
4
Recent Advances in Beta-Alanine Production via Enzymatic Catalysis and Microbial Whole-Cell Catalysis.
Yu J, Ma P, Zhang J, Zhang H, Tie H, Ruan H. · 2026-06-03
open access unreviewed -
5
From N-of-1 to versatility in propionic acidemia: Antisense oligonucleotide-mediated skipping of a constitutive <i>PCCA</i> pseudoexon.
Totsune E, Wada Y, Mikami-Saito Y, Arai-Ichinoi N, Saijo N, Takayama J, Maeda Y, Nakajima Y, Ohara O, Kure S,… · 2026-04-02
open access unreviewed -
6
Propionic acidemia in Mexico: Clinical and genotypic spectrum.
Vela-Amieva M, Alcántara-Ortigoza MA, Guillén-López S, López-Mejía L, González-Del Ángel A, Reyna-Fabián M, F… · 2026-04-15
open access unreviewed -
7
Energetic stress in combination with impaired fatty acid oxidation induces sequestration of CoA and adaptation of CoA metabolism.
Kiyuna LA, Odendaal C, Singh M, Gerding A, Langelaar-Makkinje M, van der Zwaag M, Drachman A, Cetkovská V, Ki… · 2026-02-07
open access unreviewed -
8
Natural Molecules for Brain Health and Resilience.
Venetsanaki V, Pardali EC, Cholevas C, Grammatikopoulou MG, Goulis DG, Theoharides TC. · 2026-05-13
open access unreviewed -
9
A Novel Variant in an Israeli Bedouin Family: The First Reported Cases of Carbonic Anhydrase VA (CA5A) Deficiency in Israel.
Abelson N, Kristal E, Hershkovitz E, Wormser O, Dolgin V, Amar S, Staretz-Chacham O. · 2026-05-01
open access unreviewed -
10
Elucidation of Enzymatic Routes Underlying Odd-Chain Fatty Acid Synthesis and Propionate Assimilation in Euglena gracilis.
Katayama R, Hayashi R, Ueda M, Sakamoto T, Nakazawa M. · 2026-05-01
unreviewed
External claims. These come from an index outside this database and are not checked against it. Treat them as leads.