製品の概要

  • 製品名Recombinant Human HADHSC protein
  • タンパク質長Full length protein

製品の詳細

  • 由来Recombinant
  • 由来Saccharomyces cerevisiae
  • アミノ酸配列
    • 生物種Human
    • 配列MAFVTRQFMRSVSSSSTASASAKKIIVKHVTVIGGGLMGAGIAQVAAATG HTVVLVDQ TEDILAKSKKGIEESLRKVAKKKFAENPKAGDEFVEKTLS TIATSTDAASVVHSTDLV VEAIVENLKVKNELFKRLDKFAAEHTIFAS NTSSLQITSIANATTRQDRFAGLHFFNP VPVMKLVEVIKTPMTSQKTF ESLVDFSKALGKHPVSCKDTPGFIVNRLLVPYLMEAIR LYERGDASKE DIDTAMKLGAGYPMGPFELLDYVGLDTTKFIVDGWHEMDAENPLHQPS PSLNKLVAENKFGKKTGEGFYKYK

特性

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.

  • アプリケーション

    SDS-PAGE

  • 精製度> 90 % SDS-PAGE.
    ab88004 was purified by affinity chromatography.
  • 製品の状態Liquid
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

    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

関連情報

  • 別名
    • 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
  • 機能Plays an essential role in the mitochondrial beta-oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA.
  • 組織特異性Expressed in liver, kidney, pancreas, heart and skeletal muscle.
  • パスウェイLipid metabolism; fatty acid beta-oxidation.
  • 関連疾患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.
  • 配列類似性Belongs to the 3-hydroxyacyl-CoA dehydrogenase family.
  • 細胞内局在Mitochondrion matrix.
  • Information by UniProt

Recombinant Human HADHSC protein (ab88004) 使用論文

ab88004 has not yet been referenced specifically in any publications.

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