PCCB, Polyclonal Antibody

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

Western blot analysis of extracts of various cell lines, using PCCB antibody. Immunofluorescence analysis of U20S cell using PCCB antibody. Blue: DAPI for nuclear staining.

Affinity Purification

Liquid

1mg/ml (lot specific)

Store at -20 degree C (regular) and -80 degree C (long term). Avoid freeze / thaw cycles.

Small volumes of anti-PCCB antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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.

Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)

WB: 1:500 - 1:2000
IHC: 1:50- 1:200
IF: 1:50- 1:200

60,521 Da

propionyl-CoA carboxylase beta subunit

propionyl-CoA carboxylase beta chain, mitochondrial

Propionyl-CoA carboxylase beta chain, mitochondrial

PCCase subunit beta  [Similar Products]

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]

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

Chromosomal Location of Human Ortholog: 3q22.3

Cellular Component: cytosol; mitochondrial matrix; mitochondrion

Biological Process: biotin metabolic process; short-chain fatty acid catabolic process

Disease: Propionic Acidemia

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