製品の概要

  • 製品名Anti-HLCS antibody
    HLCS 一次抗体 製品一覧
  • 製品の詳細
    Rabbit polyclonal to HLCS
  • アプリケーション適用あり: WB, IHC-Pmore details
  • 種交差性
    交差種: Human
    交差が予測される動物種: Mouse, Rat
  • 免疫原

    Recombinant fragment, corresponding to amino acids 544-725 of Human HLCS (BC060787).

  • ポジティブ・コントロール
    • Human Fetal Lung, Human Fetal Kidney, Human Fetal Skeletal Muscle

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab100925 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 1/500 - 1/1000. Predicted molecular weight: 81 kDa.
IHC-P 1/100 - 1/500.

ターゲット情報

  • 機能Post-translational modification of specific protein by attachment of biotin. Acts on various carboxylases such as acetyl-CoA-carboxylase, pyruvate carboxylase, propionyl CoA carboxylase, and 3-methylcrotonyl CoA carboxylase.
  • 組織特異性Mostly expressed in muscle, placenta, in lesser extent in the brain, kidney, pancreas, liver and lung.
  • 関連疾患Defects in HLCS are the cause of holocarboxylase synthetase deficiency (HLCS deficiency) [MIM:253270]; also known as biotin-responsive multiple carboxylase deficiency. HLCS deficiency is a neonatal form of multiple carboxylase deficiency, an autosomal recessive disorder characterized by metabolic ketoacidosis, hyperammonemia, excretion of abnormal organic acid metabolites and dermatitis. Clinical and biochemical symptoms improve dramatically with administration of biotin.
  • 配列類似性Belongs to the biotin--protein ligase family.
  • 細胞内局在Cytoplasm. Mitochondrion.
  • Information by UniProt
  • 参照データベース
  • 別名
    • Biotin [acetyl CoA carboxylase] ligase antibody
    • Biotin [methylcrotonoyl CoA carboxylase] ligase antibody
    • Biotin [methylmalonyl CoA carboxytransferase] ligase antibody
    • Biotin [propionyl CoA carboxylase [ATP hydrolyzing]] ligase antibody
    • Biotin apo protein ligase antibody
    • Biotin apo-protein ligase antibody
    • Biotin protein ligase antibody
    • Biotin--[acetyl-CoA-carboxylase] ligase antibody
    • BPL1_HUMAN antibody
    • HCS antibody
    • HLCS antibody
    • Holocarboxylase synthetase antibody
    • Holocarboxylase synthetase, EC 6.3.4 antibody
    see all

Anti-HLCS antibody 画像

  • All lanes : Anti-HLCS antibody (ab100925) at 1/500 dilution

    Lane 1 : Human fetal lung lysate
    Lane 2 : Human fetal kidney lysate


    Predicted band size : 81 kDa
  • Cytoplasmic staining of HLCS in a Formalin/PFA-fixed paraffin-embedded section of Human Fetal Skeletal Muscle using ab100925 at a dilution of 1/100.

Anti-HLCS antibody (ab100925) 使用論文

ab100925 has not yet been referenced specifically in any publications.

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I am sorry these antibodies (anti-HLCS and anti-HMGCS1) did not perform as stated on the datashee...

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