概述

  • 产品名称Anti-BTD抗体
    参阅全部 BTD 一抗
  • 描述
    小鼠单克隆抗体to BTD
  • 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 1-544 of Human BTD

性能

应用

Our Abpromise guarantee covers the use of ab54886 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.

    This antibody has only been tested in WB against the recombinant fragment used as immunogen. We have no data on the detection of endogenous protein.

    Not yet tested in other applications.
    Optimal dilutions/concentrations should be determined by the end user.
  • 靶标

    • 功能Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation.
    • 疾病相关Defects in BTD are the cause of biotinidase deficiency (BTD deficiency) [MIM:253260]; also called late-onset multiple carboxylase deficiency. BTD deficiency is a juvenile form of multiple carboxylase deficiency, an autosomal recessive disorder of biotin metabolism, characterized by ketoacidosis, hyperammonemia, excretion of abnormal organic acid metabolites, and dermatitis. BTD deficiency is characterized by seizures, hypotonia, skin rash, alopecia, ataxia, hearing loss, and optic atrophy. If untreated, symptoms usually become progressively worse, and coma and death may occur.
    • 序列相似性Belongs to the CN hydrolase family. BTD/VNN subfamily.
      Contains 1 CN hydrolase domain.
    • 细胞定位Secreted > extracellular space.
    • Information by UniProt
    • 数据库链接
    • 别名
      • Biotinase antibody
      • Biotinidase antibody
      • Btd antibody
      • BTD_HUMAN antibody
      • EC 3.5.1.12 antibody
      see all

    Anti-BTD antibody 图像

    • Western blot against tagged recombinant protein immunogen using ab54886 BTD antibody at 1ug/ml. Predicted band size of immunogen is 85 kDa

    Anti-BTD antibody (ab54886)参考文献

    ab54886 has not yet been referenced specifically in any publications.

    Product Wall

    Abreviews
    Abcam guarantees this product to work in the species/application used in this Abreview.
    Application Western blot
    Sample Human Recombinant protein (3 day conditioned media from trans. transfection)
    Loading amount 0.3 µg
    Specification 3 day conditioned media from trans. transfection
    Gel Running Conditions Non-reduced Denaturing (10% Tricine gel and iBLOT transfer to nitrocellulos (Invitrogen) and antibody incubation and washing using SNAP id* (Millipore))
    Blocking step Milk as blocking agent for 3 minute(s) · Concentration: 0.2% · Temperature: 22°C
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    提交于 Dec 11 2008

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