The gene PCCB encodes the beta subunit of propionyl-CoA carboxylase (PCC), a crucial enzyme in the mitochondria. Known as PCCase subunit beta, PCCB is involved in the catabolism of odd-chain fatty acids and branched-chain amino acids. The enzyme catalyzes the carboxylation of propionyl-CoA, which is essential for the metabolism of these compounds. PCCB functions within the holoenzyme, where it transfers the carboxyl group from biotin to propionyl-CoA. Deficiencies in PCCB can lead to propionic acidemia, a rare metabolic disorder characterized by the accumulation of propionic acid and its metabolites. Current drug development efforts focus on modulating PCCB activity to treat this condition, with mRNA therapies showing promising results in clinical trials.
Recombinant Human Propionyl-CoA carboxylase beta chain, mitochondrial (PCCB) (CSB-EP017523HU)
Validated Data
(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
PCCB Antibody (CSB-PA017523LA01HU)
Validated Data
Western Blot
Positive WB detected in: Jurkat whole cell lysate
All lanes: PCCB antibody at 7.7µg/ml
Secondary
Goat polyclonal to rabbit IgG at 1/50000 dilution
Predicted band size: 59, 61 kDa
Observed band size: 59 kDa
IHC image of CSB-PA017523LA01HU diluted at 1:300 and staining in paraffin-embedded human pancreatic cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
IHC image of CSB-PA017523LA01HU diluted at 1:300 and staining in paraffin-embedded human kidney tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
The following PCCB reagents supplied by CUSABIO are manufactured under a strict quality control system. Multiple applications have been validated and solid technical support is offered.
PCCB Antibodies for Homo sapiens (Human)
Code | Product Name | Species Reactivity | Application |
---|---|---|---|
CSB-PA017523LA01HU | PCCB Antibody | Human | ELISA, WB, IHC |
CSB-PA017523LB01HU | PCCB Antibody, HRP conjugated | Human | ELISA |
CSB-PA017523LC01HU | PCCB Antibody, FITC conjugated | Human | |
CSB-PA017523LD01HU | PCCB Antibody, Biotin conjugated | Human | ELISA |
PCCB Proteins for Homo sapiens (Human)
Code | Product Name | Source |
---|---|---|
CSB-YP017523HU CSB-BP017523HU CSB-MP017523HU CSB-EP017523HU-B |
Recombinant Human Propionyl-CoA carboxylase beta chain, mitochondrial (PCCB) | Yeast Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
CSB-EP017523HU | Recombinant Human Propionyl-CoA carboxylase beta chain, mitochondrial (PCCB) | E.coli |