Anti-HMGCL抗体[9F5AB9] (ab110295)

概述

  • 产品名称Anti-HMGCL抗体[9F5AB9]
    参阅全部 HMGCL 一抗
  • 描述
    小鼠单克隆抗体[9F5AB9] to HMGCL
  • 经测试应用适用于: ICC/IF, In-Cell ELISA, IPmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Human liver mitochondria

  • 阳性对照
    • HeLa cells and human liver mitochondria.
  • 常规说明

    Product was previously marketed under the MitoSciences sub-brand.

性能

应用

Our Abpromise guarantee covers the use of ab110295 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
ICC/IF Use a concentration of 4 µg/ml.
In-Cell ELISA Use a concentration of 1 µg/ml. (0.1 µg/well)
IP Use at an assay dependent dilution.

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 [9F5AB9] 图像

  • Immunocytochemistry image of ab110295 stained Human HeLa cells. The cells were paraformaldehyde fixed (4%, 20 minutes) and Triton X-100 permeabilized (0.1%, 15 minutes). The cells were incubated with ab110295 at 5 µg/ml for 2 hours at room temperature or over night at 4°C. The secondary antibody was (green) Alexa Fluor® 488 goat anti-mouse IgG (H+L) used at a 1/1000 dilution for 1 hour. 10% Goat serum was used as the blocking agent for all blocking steps. Target protein locates mainly in mitochondria.

Anti-HMGCL antibody [9F5AB9] (ab110295)参考文献

ab110295 has not yet been referenced specifically in any publications.

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