Propionyl-CoA carboxylase
Clinical / pharmacological. Propionic acidaemia; biotin-dependent
What it does, reaction by reaction
Pyrimidine Degradation Nucleotide Metabolism · Cytosol
Propionyl-CoA → succinyl-CoA
Converts Propionyl-CoA into Succinyl-CoA
› Notes
Propionyl-CoA carboxylase uses ATP, bicarbonate, and biotin to convert propionyl-CoA to D-methylmalonyl-CoA; methylmalonyl-CoA epimerase produces L-methylmalonyl-CoA; and adenosylcobalamin-dependent methylmalonyl-CoA mutase rearranges it to succinyl-CoA. These downstream steps permit thymine carbon to enter the tricarboxylic acid cycle.
Showing all 1 reactions.
What accelerates and inhibits it
Regulation is pathway-specific, so each context is listed separately
Amino Acid Catabolism Overview (glucogenic vs. ketogenic amino acids; brief overview of branched-chain amino acid catabolism and phenylalanine/tyrosine catabolism as representative examples)
Propionyl-CoA availability; biotin sufficiency
Biotin deficiency; product accumulation
No dominant acute hormonal switch; flux follows odd-chain fatty-acid and amino-acid catabolism
Pyrimidine Degradation
Listed there as: Propionyl-CoA carboxylase (downstream thymine-carbon handling)
Propionyl-CoA and bicarbonate availability
Biotin deficiency and loss-of-function variants impair activity
Metabolic/nutritional state influences substrate flow; no defining acute hormonal allostery.
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
Precision metabolic therapy for propionic acidemia.
Subramaniyan B, Lu F, Li H, Paiboonrungruang C, Li Y, Xiong Z, Zhang GF, Chen X. · 2026-06-24
unreviewed -
3
Advances in vehicles for in situ delivery: From classical vectors to biologically inspired structures.
Wang H, Tang X, Pan X, Tang H, Gao J, Li Q. · 2026-03-17
open access unreviewed -
4
Enhanced spinosad production in <i>Saccharopolyspora spinosa</i> by employing mannose as an extracellular carbon reservoir and optimizing acetyl-CoA supply pathway.
Zhu Z, Zhao Q, Zhu Y, Shuai L, Li J, Liu Q, Liu X, Wang S, Jin D, Dai Z, Xia L, Liu H, Rang J. · 2026-02-09
open access unreviewed -
5
A novel TetR-type repressor directly modulates precursor supply and utilization for erythromycin biosynthesis.
Wu P, Meng Z, Shu Y, Chen K, Xu Z, Huang X, Zhang B, Liu J, Wu H. · 2026-02-03
open access unreviewed -
6
Adaptation of White Adipocytes to Cooler Temperatures: Impacts on Energy Metabolism and Protein Acetylation.
Mori H, Hariri H, Moe W, Durham S, Guzman Y, Paulsson E, Simmermon RC, Bhanderi PB, Peterson SK, Dickson MJ, … · 2026-08-01
unreviewed -
7
Reprogrammed Propionate Metabolism Alters Redox-Dependent Aldosterone Production.
Sun M, Gao M, Liu Y, Xia Z, Ma L, Shi N, Zhang Y, Sun S, Tao J, Bao M, Zhou M, Wang C, Yang T, Zhang Z, Yang … · 2026-06-04
unreviewed -
8
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 -
9
Holocarboxylase synthetase deficiency: pathogenesis, clinical features, diagnosis, treatment, and research prospects.
ZeZhao J, Ablimit A. · 2026-06-22
unreviewed -
10
Adeno-Associated Virus Gene Therapy Translation: Lessons from Early Regulatory Meetings.
Stan R, Lomash RM, Shchelochkov OA, Manoli I, Galarreta Aima CI, Mangalampalli V, Choi EY, Chandler RJ, Li L,… · 2026-06-19
unreviewed
External claims. These come from an index outside this database and are not checked against it. Treat them as leads.