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AMACR pAb

AMACR pAb

Cat no: BS6159


Supplier: Bioworld Technology, Inc.
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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.
Catalogue number: BS6159
Reactivities: Human
Hosts: Rabbit
Applications: Immunofluorescence, Immunohistochemistry, Western Blot
Size: 100ug/100ul
Swiss prot: Q9UHK6
Dilutions: WB: 1:500~1:2000 IHC: 1:50~1:200 IF: 1:50~1:200
Form: liquid
Storage buffer: 1 mg/ml in Phosphate buffered saline (PBS) with 0.05% sodium azide, approx. pH 7.2.
Concentration: 1ug/ul
Antigen: Recombinant full length Human AMACR.
Species: AMACR pAb detects endogenous levels of AMACR protein.
Molecular weight: ~ 43 kDa
Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Storage temp: Store at 4 degrees C short term. Aliquot and store at -20 degrees C long term. Avoid freeze-thaw cycles.
Alternative names: 2 arylpropionyl CoA epimerase; 2 methylacyl CoA racemase; 2-methylacyl-CoA racemase; Alpha methylacyl CoA racemase; Alpha methylacyl Coenzyme A racemase; Alpha methylacyl-CoA racemase deficiency; included; Alpha-methylacyl-CoA racemase; Amacr; AMACR deficiency; included; AMACR; CBAS4; Da1-8; EC 5.1.99.4; Macr 1; Macr1; Methylacyl CoA racemase alpha; P504S; RACE; RM;
Additional info: For research use only, not for use in diagnostic procedure.

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