PCCA Antibody - N-terminal regio : HRP (ARP33071_P050-HRP)

Propionyl CoA carboxylase, alpha polypeptide

5095

Q8WXQ7

NP_000273

Human, Mouse, Rat, Cow, Dog, Horse, Pig, Rabbit, Zebrafish

The immunogen is a synthetic peptide directed towards the N terminal region of human PCCA

PCCA is the alpha subunit of the heterodimeric mitochondrial enzyme Propionyl-CoA carboxylase. PCCA is the biotin-binding region of this enzyme. Mutations in either PCCA or PCCB (encoding the beta subunit) lead to an enzyme deficiency resulting in propionic acidemia.The protein encoded by this gene is the alpha subunit of the heterodimeric mitochondrial enzyme Propionyl-CoA carboxylase. PCCA encodes the biotin-binding region of this enzyme. Mutations in either PCCA or PCCB (encoding the beta subunit) lead to an enzyme deficiency resulting in propionic acidemia. Two transcript variants encoding different isoforms have been found for this gene.

HUWE1; PARK2; IFIT1; UBE2N; UBC; EBNA-LP; PCCB

HRP: Horseradish Peroxidase

Polyclonal Antibody

WB, IHC

Affinity Purified

0.5 mg/ml

Cow: 86%; Dog: 86%; Horse: 83%; Human: 100%; Mouse: 77%; Pig: 86%; Rabbit: 86%; Rat: 86%; Zebrafish: 75%

Liquid. Purified antibody is supplied in high phosphate PBS, 100 mm phosphate, 150 mM NaCl, pH 7.6.

All conjugated antibodies should be stored in light-protected vials or covered with a light protecting material (i.e. aluminum foil) . Conjugated antibodies are stable for at least 12 months at 4C. If longer storage is desired (24 months), conjugates may be diluted with up to 50% glycerol and stored at -20C to -80C. Freezing and thawing conjugated antibodies will compromise enzyme activity as well as antibody binding.

75kDa

Ugarte, M., (er) Am. J. Hum. Genet. 81 (6) (2007) In press

Wet Ice

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PCCA

PCCA

Rabbit

Propionyl-CoA carboxylase alpha chain, mitochondrial

NM_000282

LYYSRQCLMVSRNLGSVGYDPNEKTFDKILVANRGEIACRVIRTCKKMGI