PCCA Antibody

Product Specifications

Background

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.

NCBI Gene ID

5095

Swiss Prot

Q8WXQ7

Accession Number

NP_000273

Host

Rabbit

Reactivity

Human

Immunogen

Antibody produced in rabbits immunized with a synthetic peptide corresponding a region of human PCCA.

Clonality

Polyclonal

Conjugation

Unconjugated

Type

Primary Antibodies

Field of Research

Other

Purification

Antibody is purified by peptide affinity chromatography method.

Positive Control

Tranfected 293T Cell Lysate

Concentration

Batch dependent

Buffer

Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

Modification

None

Shipping Conditions

Blue Ice

Storage Conditions

For short periods of storage (days) store at 4˚ C. For longer periods of storage, store PCCA antibody at -20˚ C. As with any antibody avoid repeat freeze-thaw cycles.

Calculated Molecular Weight

75 kDa

Applications Notes

PCCA antibody can be used for detection of PCCA by ELISA at 1:312500. PCCA antibody can be used for detection of PCCA by western blot at 1 μ g/mL, and HRP conjugated secondary antibody should be diluted 1:50,000 - 100,000.