Recombinant Human PCCB cell lysate

• Cat.No.: PCCB-1291HCL

Specification

• Description: The protein encoded by this gene is a subunit of the propionyl-CoA carboxylase (PCC) enzyme, which is involved in the catabolism of propionyl-CoA. PCC is a mitochondrial enzyme that probably acts as a dodecamer of six alpha subunits and six beta subunits. This gene encodes the beta subunit of PCC. Defects in this gene are a cause of propionic acidemia type II (PA-2).
• Species: Human
• Size: 100 ul
• Storage Buffer: 1X Sample Buffer (50 mM Tris-HCl, 2% SDS, 10% glycerol, 300 mM 2-mercaptoethanol, 0.01% Bromophenol blue)
• Applications: Western Blot

Gene Information

• Gene Name: PCCB propionyl CoA carboxylase, beta polypeptide [ Homo sapiens ]
• Official Symbol: PCCB
• Synonyms: PCCB; propionyl CoA carboxylase, beta polypeptide; propionyl Coenzyme A carboxylase, beta polypeptide; propionyl-CoA carboxylase beta chain, mitochondrial; PCCase subunit beta; propanoyl-CoA:carbon dioxide ligase subunit beta; DKFZp451E113
• Gene ID: 5096
• mRNA Refseq: NM_000532
• Protein Refseq: NP_000523
• MIM: 232050
• UniProt ID: P05166
• Chromosome Location: 3q21-q22
• Pathway: Fatty acid, triacylglycerol, and ketone body metabolism, organism-specific biosystem; Glyoxylate and dicarboxylate metabolism, organism-specific biosystem; Glyoxylate and dicarboxylate metabolism, conserved biosystem; Metabolic pathways, organism-specific biosystem; Metabolism, organism-specific biosystem; Metabolism of lipids and lipoproteins, organism-specific biosystem; Mitochondrial Fatty Acid Beta-Oxidation, organism-specific biosystem
• Function: ATP binding; ligase activity; nucleotide binding; propionyl-CoA carboxylase activity