This bacterial enzyme couples the decarboxylation of (S)-methylmalonyl-CoA to propanoyl-CoA to the vectorial transport of Na+ across the cytoplasmic membrane, thereby creating a sodium ion motive force that is used for ATP synthesis. It is a membrane-associated biotin protein and is strictly dependent on sodium ions for activity.
This bacterial enzyme couples the decarboxylation of (S)-methylmalonyl-CoA to propanoyl-CoA to the vectorial transport of Na+ across the cytoplasmic membrane, thereby creating a sodium ion motive force that is used for ATP synthesis. It is a membrane-associated biotin protein and is strictly dependent on sodium ions for activity.
Na+-independent carboxyltransferase activity: catalyzes the isotopic exchange between methylmalonyl-CoA and [1-14C]propionyl-CoA or between malonyl-CoA and [1-14C]acetyl-CoA, catalyzes the carboxyl transfer reaction from methylmalonyl-CoA to acetyl-CoA yielding propionyl-CoA and malonyl-CoA and vice versa
the enzyme catalyzes the only energy-conserving step during succinate fermentation. The decarboxylation of (S)-methylmalonyl-CoA is coupled to the vectorial transport of Na+ across the cytoplasmic membrane, thereby creating a sodium ion motive force that is used for ATP synthesis
the enzyme catalyzes the only energy-conserving step during succinate fermentation. The decarboxylation of (S)-methylmalonyl-CoA is coupled to the vectorial transport of Na+ across the cytoplasmic membrane, thereby creating a sodium ion motive force that is used for ATP synthesis
Determination of methylmalonyl coenzyme A by ultra high-performance liquid chromatography tandem mass spectrometry for measuring propionyl coenzyme A carboxylase activity in patients with propionic acidemia.
14-year-old Asian-American male with propionic acidaemia shows propionyl-CoA carboxylase deficiency with mutations in PCCB: IVS7+2 T-G (c.763+2 T-G) and p.R410Q (c.1229 G-A), and residual enzyme activity which likely explains the patient's mild phenotype with isolated cardiomyopathy without any documented episodes of metabolic acidosis or evidence of any neurocognitive deficits
genes involved in precursor biosyntheses for avermectin or other antibiotics, such as methylmalonyl-CoA decarboxylase, are upregulated by 2.4fold in aveI mutant at day 10, which may potentially lead to increased supply of precursors into the antibiotics
propionic acidemia, a rare autosomal recessive disorder, is caused by deficiency in propionyl-CoA carboxylase, and is categorized into two forms: an early (neonatal) onset form, in which clinical symptoms are presented within the first 90 days of life, and a late onset form. Deficiency in propionyl-CoA carboxylase leads to accumulation of toxic substances in the body, that results in severe metabolic decompensation
the propionyl-CoA carboxylase pathway does not increase the production of FK506, a polyketide macrolide with immunosuppressant activity, in Streptomyces clavuligerus CKD1119
the propionyl-CoA carboxylase pathway does not increase the production of FK506, a polyketide macrolide with immunosuppressant activity, in Streptomyces clavuligerus CKD1119
x * 56100, alpha, carboxyltransferase, + x * 41200, beta, carboxybiotin-carrier-protein decarboxylase, + x * 13100, gamma, biotin carrier protein, + x * 14200, delta, essential for the assembly of the complex, calculation of the MW from nucleotide sequence
x * 56100, alpha, carboxyltransferase, + x * 41200, beta, carboxybiotin-carrier-protein decarboxylase, + x * 13100, gamma, biotin carrier protein, + x * 14200, delta, essential for the assembly of the complex, calculation of the MW from nucleotide sequence
x * 56100, alpha, carboxyltransferase, + x * 41200, beta, carboxybiotin-carrier-protein decarboxylase, + x * 13100, gamma, biotin carrier protein, + x * 14200, delta, essential for the assembly of the complex, calculation of the MW from nucleotide sequence
x * 56100, alpha, carboxyltransferase, + x * 41200, beta, carboxybiotin-carrier-protein decarboxylase, + x * 13100, gamma, biotin carrier protein, + x * 14200, delta, essential for the assembly of the complex, calculation of the MW from nucleotide sequence
x * 56100, alpha, carboxyltransferase, + x * 41200, beta, carboxybiotin-carrier-protein decarboxylase, + x * 13100, gamma, biotin carrier protein, + x * 14200, delta, essential for the assembly of the complex, calculation of the MW from nucleotide sequence
the following functions are assigned to the different polypeptide chains: alpha /carboxyltransferase, beta /carboxybiotin-carrier-protein decarboxylase, gamma /biotin carrier protein
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EXPRESSION
ORGANISM
UNIPROT
LITERATURE
highest expression levels of PCCalpha in adult central nervous system in neocortex and hippocampal formation, thalamic and hypothalamic nuclei, midbrain nuclei such as the red nucleus and substantia nigra, as well as pons, medulla and the Purkinje and granular layers of cerebellum. PCCalpha is expressed in neurons of basal ganglia. High expression levels of PCCalpha in the whole embryonic central nervous system, both at E15.5 and E18.5, with the highest expression in the periventricular zones of telencephalon, midbrain and rhombencephalon. Choroid plexus also expresses high levels. Expression in embryonic liver increases towards E18.5. Outside the central nervous system, brown adipose tissue shows a very high expression level