製品の概要

  • 製品名Anti-HMGCL antibody
    HMGCL 一次抗体 製品一覧
  • 製品の詳細
    Mouse monoclonal to HMGCL
  • アプリケーション適用あり: WBmore details
  • 種交差性
    交差種: Human
  • 免疫原

    Recombinant full length protein, corresponding to amino acids 1-326 of Human HMGCL

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab55173 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 1-5 µg/ml.


    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Involved in the catabolism of branched amino acids such as leucine.
    • 組織特異性Fibroblasts, liver and lymphoblasts.
    • パスウェイMetabolic intermediate metabolism; (S)-3-hydroxy-3-methylglutaryl-CoA degradation; acetoacetate from (S)-3-hydroxy-3-methylglutaryl-CoA: step 1/1.
    • 関連疾患Defects in HMGCL are the cause of 3-hydroxy-3-methylglutaryl-CoA lyase deficiency (HMGCLD) [MIM:246450]; also known as hydroxymethylglutaricaciduria or HL deficiency. An autosomal recessive disease affecting ketogenesis and L-leucine catabolism. The disease usually appears in the first year of life after a fasting period and its clinical acute symptoms include vomiting, seizures, metabolic acidosis, hypoketotic hypoglycemia and lethargy. These symptoms sometimes progress to coma, with fatal outcome in some cases.
    • 配列類似性Belongs to the HMG-CoA lyase family.
    • 細胞内局在Mitochondrion matrix.
    • Information by UniProt
    • 参照データベース
    • 別名
      • 3 hydroxy 3 methylglutaryl CoA lyase antibody
      • 3 hydroxy 3 methylglutaryl Coenzyme A lyase antibody
      • 3 hydroxymethyl 3 methylglutaryl Coenzyme A lyase (hydroxymethylglutaricaciduria) antibody
      • 3 hydroxymethyl 3 methylglutaryl Coenzyme A lyase antibody
      • 3-hydroxy-3-methylglutarate-CoA lyase antibody
      • HL antibody
      • HMG CoA lyase antibody
      • HMG CoA Lyase Deficiency antibody
      • HMG-CoA lyase antibody
      • HMGCL antibody
      • HMGCL_HUMAN antibody
      • Hydroxymethylglutaricaciduria antibody
      • Hydroxymethylglutaryl CoA lyase antibody
      • Hydroxymethylglutaryl CoA lyase mitochondrial antibody
      • Hydroxymethylglutaryl-CoA lyase antibody
      • Mitochondrial 3 hydroxy 3 methylglutaryl CoA lyase antibody
      • mitochondrial antibody
      • MS725 antibody
      • OTTHUMP00000044830 antibody
      see all

    Anti-HMGCL antibody 画像



    • Predicted band size : 34 kDa
      HMGCL antibody (ab55173) at 1ug/lane + A-431 cell lysate at 25ug/lane.

    Anti-HMGCL antibody (ab55173) 使用論文

    ab55173 has not yet been referenced specifically in any publications.

    Product Wall

    There are currently no Abreviews or Questions for ab55173.
    Please use the links above to contact us or submit feedback about this product.

    Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"