Rabbit PCCB Polyclonal Antibody | anti-PCCB antibody

PCCB Antibody

Cat. Num. AAA9400762

• Reactivity: Human, Mouse, Rat
• Applications: Western Blot, Immunohistochemistry, Immunofluorescence
• Purity: Antibodies were purified by affinity purification using immunogen.
• Synonyms: PCCB; Polyclonal Antibody; PCCB Antibody; anti-PCCB antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Specificity: The antibody detects endogenous level of total PCCB protein.
• Purity/Purification: Antibodies were purified by affinity purification using immunogen.
• Form/Format: Supplied at 1.0mg/mL in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
• Concentration: 1.0 mg/ml (varies by lot)
• Sequence Length: 539

• Applicable Applications for anti-PCCB antibody: Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)
• Application Notes: Western blotting: 1:500 - 1:2000; Immunohistochemistry: 1:50 - 1:200; Immunofluorescence: 1:50 - 1:200
• Immunogen Type: Recombinant Protein
• Immunogen Description: Recombinant protein of human PCCB.
• Target Name: PCCB
• Preparation and Storage: Store at -20 degree C

Western Blot (WB)

(Western blot analysis of extracts of various cell lines, using PCCB antibody.)

Immunofluorescence (IF)

(Immunofluorescence analysis of U20S cell using PCCB antibody. Blue: DAPI for nuclear staining.)

Related Product Information for anti-PCCB antibody

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). Multiple transcript variants encoding different isoforms have been found for this gene.

Product Categories/Family for anti-PCCB antibody

Total protein Ab

NCBI and Uniprot Product Information

• NCBI GI #: 119943100
• NCBI GeneID: 5096
• NCBI Accession #: NP_000523.2
• NCBI GenBank Nucleotide #: NM_000532.4
• UniProt Accession #: P05166; Q16813; Q96CX0; B7Z2Z4
• Molecular Weight: 60,521 Da
• NCBI Official Full Name: propionyl-CoA carboxylase beta chain, mitochondrial isoform 1
• NCBI Official Synonym Full Names: propionyl CoA carboxylase, beta polypeptide
• NCBI Official Symbol: PCCB
• NCBI Protein Information: propionyl-CoA carboxylase beta chain, mitochondrial
• UniProt Protein Name: Propionyl-CoA carboxylase beta chain, mitochondrial
• Protein Family: Phycoerythrocyanin
• UniProt Gene Name: PCCB
• UniProt Synonym Gene Names: PCCase subunit beta
• UniProt Entry Name: PCCB_HUMAN

NCBI 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). Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, May 2010]

Uniprot Description

PCCB: Defects in PCCB are the cause of propionic acidemia type II (PA-2). PA-2 is a life-threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, developmental retardation, and intolerance to protein. Belongs to the AccD/PCCB family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Mitochondrial; Ligase; Carbohydrate Metabolism - propanoate; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 6.4.1.3

Chromosomal Location of Human Ortholog: 3q21-q22

Cellular Component: mitochondrion; mitochondrial matrix; cytosol

Molecular Function: propionyl-CoA carboxylase activity; ATP binding

Biological Process: vitamin metabolic process; fatty acid beta-oxidation; short-chain fatty acid catabolic process; cellular lipid metabolic process; biotin metabolic process; water-soluble vitamin metabolic process

Disease: Propionic Acidemia