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Target intelligence / Profile preview
Propionyl-CoA Carboxylase Beta Subunit (PCCB)
- Target
- PCCB
- Molecular classification
- Enzyme, Carboxylase, Metabolic enzyme
01
Overview
The propionyl-CoA carboxylase beta subunit is a crucial component of the propionyl-CoA carboxylase (PCC) enzyme. PCC, located in the mitochondria, plays a vital role in the catabolism of certain amino acids (valine, methionine, threonine, isoleucine), odd-chain fatty acids, and cholesterol. The beta subunit houses the carboxytransferase domain, responsible for the transfer of a carboxyl group to propionyl-CoA. Defects in PCCB lead to propionic acidemia, a serious metabolic disorder. Recent studies suggest possible roles beyond metabolism, potentially in immune regulation in cancer.
- Other names
- PCCase beta subunitPCCB_HUMANpropanoyl-CoA:carbon dioxide ligase beta subunit
02
Mechanism of action
N/A
03
Biological functions
CatalysisAmino acid metabolismFatty acid metabolismCholesterol metabolismCO2 ligase activityPossible immune regulation
04
Disease associations
Propionic acidemiaMetabolic disordersCancer (potential role)
05
Safety considerations
Propionic acidemia is a severe, potentially fatal metabolic disorder if untreated.
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