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ITM0023

  • Gene/Protein: AMACR
  • Product Description: Immunotag™ AMACR Monoclonal Antibody
  • 368.0000
    Price in reward points: 368

    Available Options

ITM0023

Immunotag™ AMACR Monoclonal Antibody
Antibody Specification
Datasheet
Target Protein AMACR
Clonality Monoclonal
Storage/Stability -20°C/1 year
Application WB,IHC-p,IF,ELISA
Recommended Dilution Western Blot: 1/500 - 1/2000. Immunohistochemistry: 1/200 - 1/1000. Immunofluorescence: 1/200 - 1/1000. ELISA: 1/10000. Not yet tested in other applications.
Concentration 1 mg/ml
Reactive Species Human,Mouse
Host Species Mouse
Immunogen Purified recombinant fragment of human AMACR expressed in E. Coli.
Specificity AMACR Monoclonal Antibody detects endogenous levels of AMACR protein.
Purification Affinity purification
Form Ascitic fluid containing 0.03% sodium azide.
Gene Name AMACR
Accession No. Q9UHK6 O09174
Alternate Names AMACR; Alpha-methylacyl-CoA racemase; 2-methylacyl-CoA racemase
Description alpha-methylacyl-CoA racemase(AMACR) Homo sapiens 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. [provided by RefSeq, Mar 2011],
Cell Pathway/ Category Primary bile acid biosynthesis,
Protein Expression Aorta,Brain,Cerebellum,Kidney,Liver,PCR rescued clones,Prostate cancer,Sali
Subcellular Localization cytoplasm,mitochondrion,peroxisome,peroxisomal matrix,
Protein Function catalytic activity:(2S)-2-methylacyl-CoA = (2R)-2-methylacyl-CoA.,disease:Defects in AMACR are the cause of alpha-methylacyl-CoA racemase deficiency (AMACRD) [MIM:604489]. AMACRD results in elevated plasma concentrations of pristanic acid C27-bile-acid intermediates. It can be associated with polyneuropathy, retinitis pigmentosa, epilepsy.,disease:Defects in AMACR are the cause of congenital bile acid synthesis defect type 4 (CBAS4) [MIM:214950]; 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.,function: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.,pathway:Lipid metabolism; bile acid biosynthesis.,pathway:Lipid metabolism; fatty acid metabolism.,similarity:Belongs to the caiB/baiF CoA-transferase family.,similarity:Contains 1 C1q domain.,similarity:Contains 1 collagen-like domain.,
Usage For Research Use Only! Not for diagnostic or therapeutic procedures.
Material Safety Data Sheet
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