Overview

  • Product nameRecombinant Human GCDH protein
  • Protein lengthFull length protein

Description

  • NatureRecombinant
  • SourceEscherichia coli
  • Amino Acid Sequence
    • AccessionQ92947
    • SpeciesHuman
    • SequenceMGSSHHHHHHSSGLVPRGSHMRPEFDWQDPLVLEEQLTTDEILIRDTFRT YCQERLMPRILLANRNEVFHREIISEMGELGVLGPTIKGYGCAGVSSVAY GLLARELERVDSGYRSAMSVQSSLVMHPIYAYGSEEQRQKYLPQLAKGEL LGCFGLTEPNSGSDPSSMETRAHYNSSNKSYTLNGTKTWITNSPMADLFV VWARCEDGCIRGFLLEKGMRGLSAPRIQGKFSLRASATGMIIMDGVEVPE ENVLPGASSLGGPFGCLNNARYGIAWGVLGASEFCLHTARQYALDRMQFG VPLARNQLIQKKLADMLTEITLGLHACLQLGRLKDQDKAAPEMVSLLKRN NCGKALDIARQARDMLGGNGISDEYHVIRHAMNLEAVNTYEGTHDIHALI LGRAITGIQAFTASK
    • Molecular weight46 kDa including tags
    • Amino acids45 to 438
    • TagsHis 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.
  • FormLiquid
  • 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
  • FunctionCatalyzes 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 specificityIsoform 1 and isoform 2 are expressed in fibroblasts and liver.
  • PathwayAmino-acid metabolism; lysine degradation.
    Amino-acid metabolism; tryptophan metabolism.
  • Involvement in diseaseDefects 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 similaritiesBelongs to the acyl-CoA dehydrogenase family.
  • Cellular localizationMitochondrion 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.

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