Anti-HLCS抗体(ab100925)

概述

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

    Recombinant fragment, corresponding to amino acids 544-725 of Human HLCS (BC060787).

  • 阳性对照
    • Human Fetal Lung, Human Fetal Kidney, Human Fetal Skeletal Muscle

性能

应用

Our Abpromise guarantee covers the use of ab100925 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 - 1/1000. Predicted molecular weight: 81 kDa.
IHC-P 1/100 - 1/500.

靶标

  • 功能Post-translational modification of specific protein by attachment of biotin. Acts on various carboxylases such as acetyl-CoA-carboxylase, pyruvate carboxylase, propionyl CoA carboxylase, and 3-methylcrotonyl CoA carboxylase.
  • 组织特异性Mostly expressed in muscle, placenta, in lesser extent in the brain, kidney, pancreas, liver and lung.
  • 疾病相关Defects in HLCS are the cause of holocarboxylase synthetase deficiency (HLCS deficiency) [MIM:253270]; also known as biotin-responsive multiple carboxylase deficiency. HLCS deficiency is a neonatal form of multiple carboxylase deficiency, an autosomal recessive disorder characterized by metabolic ketoacidosis, hyperammonemia, excretion of abnormal organic acid metabolites and dermatitis. Clinical and biochemical symptoms improve dramatically with administration of biotin.
  • 序列相似性Belongs to the biotin--protein ligase family.
  • 细胞定位Cytoplasm. Mitochondrion.
  • Information by UniProt
  • 数据库链接
  • 别名
    • Biotin [acetyl CoA carboxylase] ligase antibody
    • Biotin [methylcrotonoyl CoA carboxylase] ligase antibody
    • Biotin [methylmalonyl CoA carboxytransferase] ligase antibody
    • Biotin [propionyl CoA carboxylase [ATP hydrolyzing]] ligase antibody
    • Biotin apo protein ligase antibody
    • Biotin apo-protein ligase antibody
    • Biotin protein ligase antibody
    • Biotin--[acetyl-CoA-carboxylase] ligase antibody
    • BPL1_HUMAN antibody
    • HCS antibody
    • HLCS antibody
    • Holocarboxylase synthetase antibody
    • Holocarboxylase synthetase, EC 6.3.4 antibody
    see all

Anti-HLCS antibody 图像

  • All lanes : Anti-HLCS antibody (ab100925) at 1/500 dilution

    Lane 1 : Human fetal lung lysate
    Lane 2 : Human fetal kidney lysate


    Predicted band size : 81 kDa
  • Cytoplasmic staining of HLCS in a Formalin/PFA-fixed paraffin-embedded section of Human Fetal Skeletal Muscle using ab100925 at a dilution of 1/100.

Anti-HLCS antibody (ab100925)参考文献

ab100925 has not yet been referenced specifically in any publications.

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I am sorry these antibodies (anti-HLCS and anti-HMGCS1) did not perform as stated on the datashee...

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