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
Branched-chain amino acids and gut microbiota: coregulation and impact on neurological function via the gut-brain axis.
Li Q, Fan B, Xiao Y, Zhang Z, Zhao S, Liao B, Zeng J, Shan X, Ye H, Wen Y, Zhang L, Guo H, Xiao F, Zhu H. · 2026-07-14
open access unreviewed -
2
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 -
3
Inborn Errors of Metabolism: Pathophysiological Insights, Diagnostic Advances, and Evolving Treatment Paradigms.
Sadaria R. · 2026-06-19
open access unreviewed -
4
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 -
5
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 -
6
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 -
7
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 -
8
Neuropathology of Friedreich ataxia and its links to metabolic pathways.
Mercado-Ayón E, Lazaropoulos MP, Mercado-Ayón Y, Lynch DR. · 2025-12-25
cited 2× open access unreviewed -
9
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 -
10
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
External claims. These come from an index outside this database and are not checked against it. Treat them as leads.