Overview

  • Product name

  • Description

    Rabbit polyclonal to AMACR
  • Host species

    Rabbit
  • Tested applications

    Suitable for: IHC-P, WBmore details
  • Species reactivity

    Reacts with: Human, Pig
  • Immunogen

    Recombinant fragment (His-T7-tag) corresponding to Human AMACR aa 2-148. Expressed in E.coli. N-terminal tags.
    Sequence:

    ALQGISVVELSGLAPGPFCAMVLADFGARVVRVDRPGSRYDVSRLGRGKR SLVLDLKQPRGAAVLRRLCKRSDVLLEPFRRGVMEKLQLGPEILQRENPR LIYARLSGFGQSGSFCRLAGHDINYLALSGVLSKIGRSGENPYAPLN


    Database link: Q9UHK6

  • Positive control

    • WB: Recombinant human AMACR; Pig kidney and liver lysates. IHC-P: Human kidney tissue.
  • General notes

     This product was previously labelled as AMACR, AMCR

     

Properties

Applications

Our Abpromise guarantee covers the use of ab231333 in the following tested applications.

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

Application Abreviews Notes
IHC-P Use a concentration of 5 - 20 µg/ml.
WB Use a concentration of 0.2 - 20 µg/ml. Predicted molecular weight: 42 kDa.

Target

  • 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.
    Lipid metabolism; fatty acid metabolism.
  • Involvement in disease

    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.
  • Sequence similarities

    Belongs to the CaiB/BaiF CoA-transferase family.
  • Cellular localization

    Peroxisome. Mitochondrion.
  • Information by UniProt
  • Database links

  • Alternative names

    • 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

Images

  • Formalin-fixed, paraffin-embedded human kidney tissue stained for AMACR using ab231333 at 20 μg/ml in immunohistochemical analysis. DAB staining.

  • Anti-AMACR antibody (ab231333) at 2 µg/ml + Pig kidney lysate

    Predicted band size: 42 kDa

  • Anti-AMACR antibody (ab231333) at 2 µg/ml + Pig liver lysate

    Predicted band size: 42 kDa

  • Anti-AMACR antibody (ab231333) at 2 µg/ml + Recombinant human AMACR protein

    Predicted band size: 42 kDa

References

ab231333 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab231333.
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