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

Recombinant Human GCDH protein images

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

References for Recombinant Human GCDH protein (ab98118)

ab98118 has not yet been referenced specifically in any publications.

Product Wall

There are currently no Abreviews 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