Overview

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

Description

  • Nature
    Recombinant
  • Source
    Escherichia coli
  • Amino Acid Sequence
    • Accession
    • Species
      Human
    • Sequence
      MGSSHHHHHHSSGLVPRGSHMRPEFDWQDPLVLEEQLTTDEILIRDTFRT YCQERLMPRILLANRNEVFHREIISEMGELGVLGPTIKGYGCAGVSSVAY GLLARELERVDSGYRSAMSVQSSLVMHPIYAYGSEEQRQKYLPQLAKGEL LGCFGLTEPNSGSDPSSMETRAHYNSSNKSYTLNGTKTWITNSPMADLFV VWARCEDGCIRGFLLEKGMRGLSAPRIQGKFSLRASATGMIIMDGVEVPE ENVLPGASSLGGPFGCLNNARYGIAWGVLGASEFCLHTARQYALDRMQFG VPLARNQLIQKKLADMLTEITLGLHACLQLGRLKDQDKAAPEMVSLLKRN NCGKALDIARQARDMLGGNGISDEYHVIRHAMNLEAVNTYEGTHDIHALI LGRAITGIQAFTASK
    • Molecular weight
      46 kDa including tags
    • Amino acids
      45 to 438
    • Tags
      His tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab98118 in the following tested applications.

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

  • Applications

    SDS-PAGE

    Mass Spectrometry

  • Mass spectrometry
    MALDI-TOF
  • Purity
    > 90 % SDS-PAGE.
    ab98118 is purified by using conventional chromatography techniques.
  • Form
    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

    Preservative: None
    Constituents: 20% Glycerol, 5mM DTT, 200mM Sodium chloride, 20mM Tris HCl, pH 8

General Info

  • Alternative names
    • ACAD5
    • EC 1.3.99.7
    • GCD
    • Gcdh
    • GCDH_HUMAN
    • Glutaryl CoA dehydrogenase
    • Glutaryl CoA dehydrogenase, mitochondrial
    • Glutaryl Coenzyme A dehydrogenase
    • Glutaryl-CoA dehydrogenase
    • mitochondrial
    • MS781
    see all
  • Function
    Catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. Isoform Short is inactive.
  • Tissue specificity
    Isoform 1 and isoform 2 are expressed in fibroblasts and liver.
  • Pathway
    Amino-acid metabolism; lysine degradation.
    Amino-acid metabolism; tryptophan metabolism.
  • Involvement in disease
    Defects in GCDH are the cause of glutaric aciduria type 1 (GA1) [MIM:231670]. GA1 is an autosomal recessive metabolic disorder characterized by progressive dystonia and athetosis due to gliosis and neuronal loss in the basal ganglia.
  • Sequence similarities
    Belongs to the acyl-CoA dehydrogenase family.
  • Cellular localization
    Mitochondrion matrix.
  • Information by UniProt

Images

  • 15% SDS-PAGE analysis of ab98118 (3µg)

References

ab98118 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab98118.
Please use the links above to contact us or submit feedback about this product.

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

Sign up