Recombinant人HADHSC protein (ab88004)

概述

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

描述

  • 性质Recombinant
  • 来源Saccharomyces cerevisiae
  • 氨基酸序列
    • 种属Human
    • 序列MAFVTRQFMRSVSSSSTASASAKKIIVKHVTVIGGGLMGAGIAQVAAATG HTVVLVDQ TEDILAKSKKGIEESLRKVAKKKFAENPKAGDEFVEKTLS TIATSTDAASVVHSTDLV VEAIVENLKVKNELFKRLDKFAAEHTIFAS NTSSLQITSIANATTRQDRFAGLHFFNP VPVMKLVEVIKTPMTSQKTF ESLVDFSKALGKHPVSCKDTPGFIVNRLLVPYLMEAIR LYERGDASKE DIDTAMKLGAGYPMGPFELLDYVGLDTTKFIVDGWHEMDAENPLHQPS PSLNKLVAENKFGKKTGEGFYKYK

技术指标

Our Abpromise guarantee covers the use of ab88004 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

  • 纯度> 90 % SDS-PAGE.
    ab88004 was purified by affinity chromatography.
  • 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. Avoid freeze / thaw cycles.

    Preservative: None
    Constituents: 30% Glycerol, 0.5% Triton-X-100, 50mM HEPES, 30mM Glutathione, 100mM Sodium chloride, 1mM DTT, pH 7.5

常规信息

  • 别名
    • 3 hydroxyacyl Coenzyme A dehydrogenase
    • HAD
    • HADH
    • HADH1
    • HADHSC
    • HADHSC, formerly
    • HADSC, formerly
    • HCDH
    • HCDH_HUMAN
    • HHF4
    • Hydroxyacyl CoA dehydrogenase
    • Hydroxyacyl-coenzyme A dehydrogenase
    • hydroxyacyl-coenzyme A dehydrogenase, mitochondrial
    • L 3 hydroxyacyl Coenzyme A dehydrogenase short chain
    • M SCHAD
    • Medium and short chain L 3 hydroxyacyl coenzyme A dehydrogenase
    • Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase
    • MGC8392
    • mitochondrial
    • MSCHAD
    • OTTHUMP00000162626
    • OTTHUMP00000219688
    • SCHAD
    • SCHAD, formerly
    • Short chain 3 hydroxyacyl CoA dehydrogenase mitochondrial
    • short chain 3-hydroxyacyl-coa dehydrogenase
    • Short-chain 3-hydroxyacyl-CoA dehydrogenase
    see all
  • 功能Plays an essential role in the mitochondrial beta-oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA.
  • 组织特异性Expressed in liver, kidney, pancreas, heart and skeletal muscle.
  • 通路Lipid metabolism; fatty acid beta-oxidation.
  • 疾病相关Defects in HADH are the cause of 3-alpha-hydroxyacyl-CoA dehydrogenase deficiency (HADH deficiency) [MIM:231530]. HADH deficiency is a metabolic disorder with various clinical presentations including hypoglycemia, hepatoencephalopathy, myopathy or cardiomyopathy, and in some cases sudden death.
    Defects in HADH are the cause of familial hyperinsulinemic hypoglycemia type 4 (HHF4) [MIM:609975]; also known as persistent hyperinsulinemic hypoglycemia of infancy (PHHI) or congenital hyperinsulinism. HHF is the most common cause of persistent hypoglycemia in infancy and is due to defective negative feedback regulation of insulin secretion by low glucose levels. It causes nesidioblastosis, a diffuse abnormality of the pancreas in which there is extensive, often disorganized formation of new islets. Unless early and aggressive intervention is undertaken, brain damage from recurrent episodes of hypoglycemia may occur. HHF4 should be easily recognizable by analysis of acylcarnitine species and that this disorder responds well to treatment with diazoxide. It provides the first 'experiment of nature' that links impaired fatty acid oxidation to hyperinsulinism and that provides support for the concept that a lipid signaling pathway is implicated in the control of insulin secretion.
  • 序列相似性Belongs to the 3-hydroxyacyl-CoA dehydrogenase family.
  • 细胞定位Mitochondrion matrix.
  • Information by UniProt

Recombinant Human HADHSC protein (ab88004)参考文献

ab88004 has not yet been referenced specifically in any publications.

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