Overview

  • Product name
    Recombinant Human AMACR protein
  • Protein length
    Full length protein

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Species
      Human
    • Sequence
      MALQGISVMELSGLAPGPFCAMVLADFGARVVRVDRPGSRYDVSRLGRGK RSLVLDLKQPRGAAVLRRLCKRSDVLLEPFRRGVMEKLQLGPEILQRENP RLIYARLSGFGQSGSFCRLAGHDINYLALSGGRNSMFKFFSVENSEIESV GSTSRTEHVGWWSTFLYDLQDSRWGIHGCWSNRTPVLRAADQRTWTKV
    • Amino acids
      1 to 198
    • Tags
      proprietary tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab161708 in the following tested applications.

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

  • Applications

    ELISA

    Western blot

  • Form
    Liquid
  • Additional notes
    Protein concentration is above or equal to 0.05 mg/ml.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.31% Glutathione, 0.79% Tris HCl

General Info

  • 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_HUMAN
    • CBAS4
    • Da1-8
    • EC 5.1.99.4
    • Macr1
    • Methylacyl CoA racemase alpha
    • RACE
    • RM
    see all
  • 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

Images

  • SDS-PAGE - Recombinant Human AMACR protein (ab161708)
    SDS-PAGE - Recombinant Human AMACR protein (ab161708)
    ab161708 on a 12.5% SDS-PAGE stained with Coomassie Blue.

References

ab161708 has not yet been referenced specifically in any publications.

Publishing research using ab161708? Please let us know so that we can cite the reference in this datasheet.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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