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PCCA Antibody - N-terminal region : FITC (ARP33071_P050-FITC)

Selected Variant

ARP33071_P050-FITC

Size: 100 µL

Product Specifications

Gene Name

Propionyl CoA carboxylase, alpha polypeptide

Gene ID

5095

Swiss Prot

Q8WXQ7

Accession Number

NP_000273

Host

Rabbit

Reactivity

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

Immunogen

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

Target

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.

Partner Proteins

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

Clonality

Polyclonal

Conjugation

FITC: Fluorescein Isothiocyanate

Type

Polyclonal Antibody

Applications

WB, IHC

Purification

Affinity Purified

Concentration

0.5 mg/ml

Homology

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

Format

Liquid. Purified antibody supplied in 1x PBS buffer.

Reconstitution

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.

Molecular Weight

75kDa

References & Citations

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

Shipping Conditions

Wet Ice

Protein Length

728

NCBI Gene Symbol

PCCA

NCBI GB Accession Number

PCCA

Host or Source

Rabbit

Protein Name

Propionyl-CoA carboxylase alpha chain, mitochondrial

Nucleotide Accession Number

NM_000282

Peptide Sequence

LYYSRQCLMVSRNLGSVGYDPNEKTFDKILVANRGEIACRVIRTCKKMGI

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