概述

  • 产品名称Anti-HMGCL抗体
    参阅全部 HMGCL 一抗
  • 描述
    兔多克隆抗体to HMGCL
  • 经测试应用适用于: WB, ICC/IFmore details
  • 种属反应性
    与反应: Human
    预测可用于: Mouse, Rat, Cow
  • 免疫原

    Recombinant protein fragment corresponding to a region within amino acids 1 and 185 of Human HMGCL (NP_000182).

  • 阳性对照
    • WB: 293T, A431, H1299, HeLa, HepG2, MOLT4 and Raji cell lysates ICC/IF: HeLa cells

性能

应用

Our Abpromise guarantee covers the use of ab97293 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 1/500 - 1/3000. Predicted molecular weight: 34 kDa.
ICC/IF 1/100 - 1/200.

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

  • Anti-HMGCL antibody (ab97293) at 1/3000 dilution + H1299 whole cell lysate at 30 µg

    Predicted band size : 34 kDa
  • ab97293, at a 1/200 dilution, staining HMGCL in paraformaldehyde fixed HeLa by Immunofluorescence analysis.
    The lower image was merged with DNA probe.

Anti-HMGCL antibody (ab97293)参考文献

ab97293 has not yet been referenced specifically in any publications.

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