概述

  • 产品名称Anti-HMGCL抗体
    参阅全部 HMGCL 一抗
  • 描述
    小鼠单克隆抗体to HMGCL
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Human
  • 免疫原

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

性能

应用

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.

应用 Ab评论 说明
WB
  • 应用说明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.
  • 靶标

    • 功能Involved in the catabolism of branched amino acids such as leucine.
    • 组织特异性Fibroblasts, liver and lymphoblasts.
    • 通路Metabolic intermediate metabolism; (S)-3-hydroxy-3-methylglutaryl-CoA degradation; acetoacetate from (S)-3-hydroxy-3-methylglutaryl-CoA: step 1/1.
    • 疾病相关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.
    • 序列相似性Belongs to the HMG-CoA lyase family.
    • 细胞定位Mitochondrion matrix.
    • Information by UniProt
    • 数据库链接
    • 别名
      • 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 图像



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

    Anti-HMGCL antibody (ab55173)参考文献

    ab55173 has not yet been referenced specifically in any publications.

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