Recombinant人GCDH protein (ab98118)

概述

  • 产品名称
    Recombinant人GCDH protein
  • 蛋白长度
    Full length protein

描述

  • 性质
    Recombinant
  • 来源
    Escherichia coli
  • 氨基酸序列
    • Accession
    • 种属
      Human
    • 序列
      MGSSHHHHHHSSGLVPRGSHMRPEFDWQDPLVLEEQLTTDEILIRDTFRT YCQERLMPRILLANRNEVFHREIISEMGELGVLGPTIKGYGCAGVSSVAY GLLARELERVDSGYRSAMSVQSSLVMHPIYAYGSEEQRQKYLPQLAKGEL LGCFGLTEPNSGSDPSSMETRAHYNSSNKSYTLNGTKTWITNSPMADLFV VWARCEDGCIRGFLLEKGMRGLSAPRIQGKFSLRASATGMIIMDGVEVPE ENVLPGASSLGGPFGCLNNARYGIAWGVLGASEFCLHTARQYALDRMQFG VPLARNQLIQKKLADMLTEITLGLHACLQLGRLKDQDKAAPEMVSLLKRN NCGKALDIARQARDMLGGNGISDEYHVIRHAMNLEAVNTYEGTHDIHALI LGRAITGIQAFTASK
    • 分子量
      46 kDa including tags
    • 氨基酸
      45 to 438
    • 标签
      His tag N-Terminus

技术指标

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.

  • 应用

    SDS-PAGE

    Mass Spectrometry

  • 质谱法
    MALDI-TOF
  • 纯度
    > 90 % SDS-PAGE.
    ab98118 is purified by using conventional chromatography techniques.
  • 形式
    Liquid
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    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

常规信息

  • 别名
    • 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
  • 功能
    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.
  • 组织特异性
    Isoform 1 and isoform 2 are expressed in fibroblasts and liver.
  • 通路
    Amino-acid metabolism; lysine degradation.
    Amino-acid metabolism; tryptophan metabolism.
  • 疾病相关
    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.
  • 序列相似性
    Belongs to the acyl-CoA dehydrogenase family.
  • 细胞定位
    Mitochondrion matrix.
  • Information by UniProt

图片

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

文献

ab98118 has not yet been referenced specifically in any publications.

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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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