製品の概要

  • 製品名Anti-HADHSC antibody
    HADHSC 一次抗体 製品一覧
  • 製品の詳細
    Goat polyclonal to HADHSC
  • アプリケーション適用あり: WBmore details
  • 種交差性
    交差種: Human
    交差が予測される動物種: Pig, Caenorhabditis elegans
  • 免疫原

    Synthetic peptide:

    C-YERGDASKEDID

    , corresponding to internal sequence amino acids 234-245 of Human HADHSC

  • ポジティブ・コントロール
    • Human heart, muscle and kidney lysates

製品の特性

関連製品

アプリケーション

Our Abpromise guarantee covers the use of ab65948 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 Abreviews 特記事項
WB
  • 追加情報WB: Use at a concentration of 0.01 - 0.03 µg/ml. Detects a band of approximately 34 kDa (predicted molecular weight: 34 kDa).

    Peptide ELISA: antibody detection limit dilution 1:64,000.

    Not yet tested in other applications.
    Optimal dilutions/concentrations should be determined by the end user.
  • ターゲット情報

    • 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
    • 参照データベース
    • 別名
      • 3 hydroxyacyl Coenzyme A dehydrogenase antibody
      • HAD antibody
      • HADH antibody
      • HADH1 antibody
      • HADHSC antibody
      • HADHSC, formerly antibody
      • HADSC, formerly antibody
      • HCDH antibody
      • HCDH_HUMAN antibody
      • HHF4 antibody
      • Hydroxyacyl CoA dehydrogenase antibody
      • Hydroxyacyl-coenzyme A dehydrogenase antibody
      • hydroxyacyl-coenzyme A dehydrogenase, mitochondrial antibody
      • L 3 hydroxyacyl Coenzyme A dehydrogenase short chain antibody
      • M SCHAD antibody
      • Medium and short chain L 3 hydroxyacyl coenzyme A dehydrogenase antibody
      • Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase antibody
      • MGC8392 antibody
      • mitochondrial antibody
      • MSCHAD antibody
      • OTTHUMP00000162626 antibody
      • OTTHUMP00000219688 antibody
      • SCHAD antibody
      • SCHAD, formerly antibody
      • Short chain 3 hydroxyacyl CoA dehydrogenase mitochondrial antibody
      • short chain 3-hydroxyacyl-coa dehydrogenase antibody
      • Short-chain 3-hydroxyacyl-CoA dehydrogenase antibody
      see all

    Anti-HADHSC antibody 画像

    • Anti-HADHSC antibody (ab65948) at 0.02 µg/ml + Human kidney lysate at 35 µg

      Predicted band size : 34 kDa
      Observed band size : 34 kDa

    Anti-HADHSC antibody (ab65948) 使用論文

    ab65948 has not yet been referenced specifically in any publications.

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