AMACR polyclonal Antibody | BS6159

AMACR polyclonal Antibody | BS6159 | Gentaur UK, US & Europe Distribution

Host: Rabbit

Reactivity: Human,Mouse

Application: WB,IHC,IF

Application Range: WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200

Background: Racemization of 2-methyl-branched fatty acid CoA esters. Responsible for the conversion of pristanoyl-CoA and C27-bile acyl-CoAs to their (S) -stereoisomers.Defects in AMACR are the cause of alpha-methylacyl-CoA racemase deficiency (AMACRD) . AMACRD results in elevated plasma concentrations of pristanic acid C27-bile-acid intermediates. It can be associated with polyneuropathy, retinitis pigmentosa, epilepsy. Defects in AMACR are the cause of congenital bile acid synthesis defect type 4 (CBAS4) ; also known as cholestasis, intrahepatic, with defective conversion of trihydroxycoprostanic acid to cholic acid or trihydroxycoprostanic acid in bile. Clinical features include neonatal jaundice, intrahepatic cholestasis, bile duct deficiency and absence of cholic acid from bile.

Storage & Stability: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

Specificity: AMACR polyclonal Antibody detects endogenous levels of AMACR protein.

Molecular Weight: ~ 43 kDa

Note: For research use only, not for use in diagnostic procedure.

Alternative Names: Alpha-methylacyl-CoA racemase; 2-methylacyl-CoA racemase; AMACR

Immunogen: Recombinant full length Human AMACR.

Conjugate: Unconjugated

Modification: Unmodification

Purification & Purity: The Antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE) .

Pathway: