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PCCA
Loading...Anti-PCCA Products
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- Derivation: Mouse
- Species Reactivity: Human
- Type: Mouse IgG1, κ
- Application: WB, ELISA, IF, IHC, IP
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- Chicken Anti-PCCA Polyclonal IgY (BRD-0408MZ)
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- Species Reactivity: Human
- Type: Chicken antibody
- Application: WB
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For Research Use Only. Not For Clinical Use.
The protein encoded by this gene is the alpha subunit of the heterodimeric mitochondrial enzyme Propionyl-CoA carboxylase. PCCA encodes the biotin-binding region of this enzyme. Mutations in either PCCA or PCCB (encoding the beta subunit) lead to an enzyme deficiency resulting in propionic acidemia. Multiple transcript variants encoding different isoforms have been found for this gene.
Protein class
Disease related genes, Enzymes, Human disease related genes, Metabolic proteins, Plasma proteins, Potential drug targets
Predicted location
Intracellular
Single cell type specificity
Cell type enhanced (Microglial cells, Oligodendrocyte precursor cells, Oligodendrocytes, Inhibitory neurons, Excitatory neurons, Astrocytes)
Immune cell specificity
Low immune cell specificity
Cell line specificity
Cell line enhanced (RPTEC TERT1, SCLC-21H, U-937)
Interaction
The holoenzyme is a dodecamer composed of 6 PCCA/alpha subunits and 6 PCCB/beta subunits (PubMed:6765947, PubMed:20725044). Interacts (via the biotin carboxylation domain) with SIRT4 (PubMed:23438705). Interacts with SIRT3 and SIRT5 (PubMed:23438705).
Molecular function
Ligase
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