Overview

  • Product nameRecombinant Human GCSH protein
  • Protein lengthFull length protein

Description

  • NatureRecombinant
  • SourceEscherichia coli
  • Amino Acid Sequence
    • AccessionP23434
    • SpeciesHuman
    • SequenceMGSSHHHHHHSSGLVPRGSHMGSMSVRKFTEKHEWVTTENGIGTVGISNF AQEALGDVVYCSLPEVGTKLNKQDEFGALESVKAASELYSPLSGEVTEIN EALAENPGLVNKSCYEDGWLIKMTLSNPSELDELMSEEAYEKYIKSIEE
    • Molecular weight16 kDa including tags
    • Amino acids49 to 173
    • TagsHis tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab113856 in the following tested applications.

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

  • Applications

    Mass Spectrometry

    SDS-PAGE

  • Mass spectrometry
    MALDI-TOF
  • Purity> 95 % SDS-PAGE.
    ab113856 was purified using conventional chromatography techniques.
  • FormLiquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.

    pH: 8.00
    Constituents: 0.32% Tris HCl, 10% Glycerol, 0.02% DTT, 0.88% Sodium chloride

General Info

  • Alternative names
    • GCE
    • GCSH
    • GCSH_HUMAN
    • Glycine cleavage system H protein
    • Glycine cleavage system H protein mitochondrial
    • Glycine cleavage system protein H
    • Glycine cleavage system protein H (aminomethyl carrier)
    • Lipoic acid containing protein
    • mitochondrial
    • Mitochondrial glycine cleavage system H protein
    • NKH
    see all
  • FunctionThe glycine cleavage system catalyzes the degradation of glycine. The H protein shuttles the methylamine group of glycine from the P protein to the T protein.
  • Involvement in diseaseDefects in GCSH are a cause of non-ketotic hyperglycinemia (NKH) [MIM:605899]; also known as glycine encephalopathy (GCE). NKH is an autosomal recessive disease characterized by accumulation of a large amount of glycine in body fluid and by severe neurological symptoms.
  • Sequence similaritiesBelongs to the gcvH family.
    Contains 1 lipoyl-binding domain.
  • Cellular localizationMitochondrion.
  • Information by UniProt

Recombinant Human GCSH protein images

  • 15% SDS-PAGE analysis of 3 µg ab113856.

References for Recombinant Human GCSH protein (ab113856)

ab113856 has not yet been referenced specifically in any publications.

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