P504S Monoclonal Antibody

Ab type

Primary antibody

Background

This gene encodes a racemase. The encoded enzyme interconverts pristanoyl-CoA and C27-bile acylCoAs between their (R)- and (S)-stereoisomers. The conversion to the (S)-stereoisomers is necessary for degradation of these substrates by peroxisomal beta-oxidation. Encoded proteins from this locus localize to both mitochondria and peroxisomes. Mutations in this gene may be associated with adult-onset sensorimotor neuropathy, pigmentary retinopathy, and adrenomyeloneuropathy due to defects in bile acid synthesis. Alternatively spliced transcript variants have been described. Read-through transcription also exists between this gene and the upstream neighboring C1QTNF3 (C1q and tumor necrosis factor related protein 3) gene.

Reactivity

Human,Mouse,Rat

Clonality

Monoclonal

Host

Mouse

Isotype

IgG

Immunogen

Purified recombinant human P504S (C-terminus) protein fragments expressed in E.coli.

Storage Stability

-20°C for one year

Concentration

1 mg/ml

MolecularWeight_Da

N/A

synonyms

AMACR; Alpha-methylacyl-CoA racemase; 2-methylacyl-CoA racemase