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.
Pack Size | 100ul, 1ml, 20ul |
---|---|
Applications | ELISA, IHC-P, WB |
Host Species | Rabbit |
Ig Isotype | IgG |
Antibody Type | Monoclonal Antibody |
Organism Species | Human |
Product Synonyms | AMACR/p504S |
Observed Mol Wt | 42kDa |
Alternative Names | RM; RACE; CBAS4; P504S; AMACRD; AMACR/p504S |
Immunogen (Antigen) | Recombinant protein |
Format | Liquid |
Buffer Formulation | 0.05% BSA, 50% glycerol, PBS with 0.05% proclin300, pH7.3 |
Reactivity | Human, Mouse, Rat |
Uniprot ID | 23600 |
Gene ID | Q9UHK6 |
Potency (Clone Number) | ARC50876 |
Purification | Antigen-specific affinity chromatography followed by Protein A affinity chromatography |
Usage | For Research Use Only. Not for diagnostics or human use. |
Shelf Life | 12 months at time of shipping |
Shipping | Shipped in Dry Ice at -20 Degree Celsius |
Storage | Store at -20 Degree Celsius. It is recommended to aliquot and store to avoid repeated freeze-thaw as it affects the stability of the antibody. |
Research Areas | Cancer; Cell Biology; Immunology |
KD/KO Validated | KD Validated |
Disclaimer | The data indicated herein are as indicated and validated in our laboratory. These reagents are for research use only and not for in-vitro diagnostics or human use. |
Reviews
There are no reviews yet.