PCCA Antibody 3 Publications

Rabbit Polyclonal| Catalog number: 21988-1-AP

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Con: 39 μg/150 μl

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Species specificity:
human, mouse

Positive WB detected in:
L02 cells, mouse liver tissue, NIH/3T3 cells

Positive IP detected in:
L02 cells

Positive IHC detected in:
human liver tissue

Recommended dilution:
WB : 1:500-1:2000
IP : 0.5-4.0 ug for IP and 1:500-1:2000 for WB
IHC : 1:100-1:400

Product Information


Source:
Rabbit

Purification method:
Antigen affinity purification

Isotype:
IgG

Storage:
PBS with 0.02% sodium azide and 50% glycerol pH 7.3. Store at -20oC. Aliquoting is unnecessary for -20oC storage.

Immunogen Information


Full name:
propionyl Coenzyme A carboxylase, alpha polypeptide

Calculated molecular weight:
80 kDa

Observed molecular weight:
70 kDa

GenBank accession number:

Gene ID (NCBI):

Gene symbol
PCCA

Synonyms
PCCA, PCCase subunit alpha
Background

Propionyl-CoA carboxylase (PCC) catalyzes the biotin-dependent carboxylation of propionyl-CoA to D-methyl-malonyl CoA, a reaction that occurs in the mitochondrial matrix. PCC is involved in the catabolism of several essential amino acids (methionine, isoleucine, threonine and valine), as well as odd chain fatty acids and cholesterol. Deficiency of PCC results in propionic acidemia, a metabolic disorder characterized by severe metabolic ketoacidosis, vomiting, lethargy and hypotonia. PCC consists of nonidentical subunits (α and β) encoded by different genes (PCCA and PCCB, respectively). The αPCC cDNA contains an open reading frame of 2106 nucleotide bases and codes for a 702 amino acid polypeptide. The mature length subunit is 70 kDa and contains the biotin binding site.(PMID:8852656). This protein has 3 isoforms produced by alternative splicing with the molecular weight of 80 kDa, 77 kDa and 75 kDa. The full length protein has a transit peptide with 52 amino acids which will be removed in mature form. This antibody is specific to PCCA.


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