BS6159

antibody from Bioworld Technology, Inc
Targeting: AMACR P504S, RACE
Provider product page for BS6159
Western blot
Immunohistochemistry

Antibody data

Product number
BS6159 - Provider product page
Provider
Bioworld Technology, Inc
Product name
AMACR polyclonal antibody
Antibody type
Polyclonal
Antigen
Recombinant full length Human AMACR.
Description
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.
Reactivity
Human, Mouse
Host
Rabbit
Isotype
IgG
Vial size
100ul
Concentration
1 mg/ml
Storage
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
AMACR protein structure - BS6159 shown in red.