概述

  • 产品名称Anti-AMACR抗体
    参阅全部 AMACR 一抗
  • 描述
    小鼠单克隆抗体to AMACR
  • 经测试应用适用于: WB, ELISAmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Recombinant full length Human AMACR protein (AAH09471, 1 a.a. ~ 199 a.a) with tag.

  • 阳性对照
    • HepG2 cell lysate.

应用

Our Abpromise guarantee covers the use of ab76930 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
WB Use a concentration of 1 - 5 µg/ml. Predicted molecular weight: 42 kDa.
ELISA Use at an assay dependent dilution.

靶标

  • 功能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.
  • 通路Lipid metabolism; bile acid biosynthesis.
    Lipid metabolism; fatty acid metabolism.
  • 疾病相关Defects in AMACR are the cause of alpha-methylacyl-CoA racemase deficiency (AMACRD) [MIM:614307]. 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) [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.
  • 序列相似性Belongs to the CaiB/BaiF CoA-transferase family.
  • 细胞定位Peroxisome. Mitochondrion.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 2 arylpropionyl CoA epimerase antibody
    • 2 methylacyl CoA racemase antibody
    • 2-methylacyl-CoA racemase antibody
    • Alpha methylacyl CoA racemase antibody
    • Alpha methylacyl Coenzyme A racemase antibody
    • Alpha methylacyl-CoA racemase deficiency, included antibody
    • Alpha-methylacyl-CoA racemase antibody
    • Amacr antibody
    • AMACR deficiency, included antibody
    • AMACR_HUMAN antibody
    • CBAS4 antibody
    • Da1-8 antibody
    • EC 5.1.99.4 antibody
    • Macr1 antibody
    • Methylacyl CoA racemase alpha antibody
    • RACE antibody
    • RM antibody
    see all

Anti-AMACR antibody 图像

  • Anti-AMACR antibody (ab76930) at 1 µg/ml + HepG2 cell lysate at 25 µg

    Secondary
    Goat Anti-Mouse IgG (H&L)-HRP Conjugate at 1/2500 dilution

    Predicted band size : 42 kDa
    Observed band size : 52 kDa (why is the actual band size different from the predicted?)

Anti-AMACR antibody (ab76930)参考文献

ab76930 has not yet been referenced specifically in any publications.

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