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: WBmore details
  • 种属反应性
    与反应: Human
    预测可用于: Mouse, Rat, Rabbit, Horse, Guinea pig, Cow, Cat, Dog, Drosophila melanogaster, Zebrafish
  • 免疫原

    Synthetic peptide, corresponding to amino acids 81-130 of Human HMGCL

  • 阳性对照
    • Fetal liver cell lysate.

性能

应用

Our Abpromise guarantee covers the use of ab62299 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 Use a concentration of 2.5 µg/ml. Detects a band of approximately 34 kDa (predicted molecular weight: 34 kDa). Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.

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 (ab62299) at 2.5 µg/ml + Fetal liver cell lysate at 10 µg

    Secondary
    HRP conjugated anti-Rabbit IgG at 1/50000 dilution

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

Anti-HMGCL antibody (ab62299)参考文献

ab62299 has not yet been referenced specifically in any publications.

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