Rabbit PCCB Polyclonal Antibody | anti-PCCB antibody

PCCB Antibody (Center)

Cat. Num. AAA9203067

• Reactivity: Human (Predicted Reactivity: Bovine, Mouse, Pig)
• Applications: Western Blot, ELISA, Flow Cytometry, Functional Assay
• Purity: Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)
• Synonyms: PCCB; Polyclonal Antibody; PCCB Antibody (Center); Propionyl-CoA carboxylase beta chain; mitochondrial; PCCase subunit beta; Propanoyl-CoA:carbon dioxide ligase subunit beta; anti-PCCB antibody

• Host: Rabbit
• Reactivity: Human (Predicted Reactivity: Bovine, Mouse, Pig)
• Clonality: Polyclonal
• Isotype: Rabbit Ig
• Specificity: This PCCB antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 82-107 amino acids from the Central region of human PCCB.
• Purity/Purification: Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)
• Form/Format: Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.
• Sequence Positions: 82-107
• Sequence Length: 559

• Applicable Applications for anti-PCCB antibody: Western Blot (WB), ELISA (EIA), Flow Cytometry (FC/FACS)
• Application Notes: WB~~1:1000; FC~~1:10-50
• Antigen Source: HUMAN
• Function: This is one of the 2 subunits of thebiotin-dependent propionyl-CoA carboxylase(PCC), a mitochondrial enzyme involved inthe catabolism of odd chain fatty acids,branched-chain amino acids isoleucine,threonine, methionine, and valine and othermetabolites (PubMed:6765947,PubMed:15890657).Propionyl-CoA carboxylase catalyzes thecarboxylation ofpropionyl-CoA/propanoyl-CoA toD-methylmalonyl-CoA/(S)-methylmalonyl-CoA (PubMed:6765947,PubMed:15890657). Withinthe holoenzyme, the alpha subunitcatalyzes the ATP-dependent carboxylationof the biotin carried by the biotin carboxylcarrier (BCC) domain, while the betasubunit then transfers the carboxyl groupfrom carboxylated biotin to propionyl-CoA(By similarity). Propionyl-CoA carboxylasealso significantly acts onbutyryl-CoA/butanoyl-CoA, which isconverted toethylmalonyl-CoA/(2S)-ethylmalonyl-CoA ata much lower rate (PubMed:6765947). Otheralternative minor substrates include (2E)-butenoyl-CoA/crotonoyl-CoA (By similarity).
• Cellular Location: Mitochondrion matrix
• Preparation and Storage: Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.

Western Blot (WB)

(Western blot analysis of PCCB Antibody (Center) in Jurkat cell line lysates (35ug/lane). PCCB (arrow) was detected using the purified Pab.)

Flow Cytometry (FC/FACS)

(PCCB Antibody (Center) flow cytometry analysis of Jurkat cells (bottom histogram) compared to a negative control cell (top histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.)

Related Product Information for anti-PCCB antibody

PCCB 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.

Product Categories/Family for anti-PCCB antibody

Metabolism; Signal Transduction

References

Yang,X., et.al.,Mol. Genet. Metab. 81 (4), 335-342 (2004)

NCBI and Uniprot Product Information

• NCBI GI #: 119943100
• NCBI GeneID: 5096
• NCBI Accession #: NP_000523.2; NP_001171485.1
• NCBI GenBank Nucleotide #: NM_000532.4
• UniProt Accession #: P05166; Q16813; Q96CX0; B7Z2Z4
• Molecular Weight: Calculated MW: 58216
• 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: Carbohydrate Metabolism - propanoate; Ligase; EC 6.4.1.3; Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine degradation

Chromosomal Location of Human Ortholog: 3q21-q22

Cellular Component: mitochondrion; mitochondrial matrix; cytosol

Molecular Function: propionyl-CoA carboxylase activity; ATP binding

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

Disease: Propionic Acidemia