概述

  • 产品名称Anti-SUMF1抗体
    参阅全部 SUMF1 一抗
  • 描述
    兔多克隆抗体to SUMF1
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Synthetic peptide conjugated to KLH, corresponding to a region within C terminal amino acids 310 - 399 of Human SUMF1

  • 阳性对照
    • Hela cell line lysates

性能

  • 形式Liquid
  • 存放说明Shipped at 4°C. Store at 4°C (up to 6 months). Store at -20°C long term.
  • 存储溶液Preservative: 0.09% Sodium Azide
    Constituents: PBS
  • Concentration information loading...
  • 纯度Immunogen affinity purified
  • 纯化说明ab103467 is purified through a protein A column, followed by peptide affinity purification.
  • 克隆多克隆
  • 同种型IgG
  • 研究领域

应用

Our Abpromise guarantee covers the use of ab103467 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 1/100 - 1/500. Predicted molecular weight: 40 kDa.

靶标

  • 功能Using molecular oxygen and an unidentified reducing agent, oxidizes a cysteine residue in the substrate sulfatase to an active site 3-oxoalanine residue, which is also called C(alpha)-formylglycine. Known substrates include GALNS, ARSA, STS and ARSE.
  • 组织特异性Ubiquitous. Highly expressed in kidney, pancreas and liver. Detected at lower levels in leukocytes, lung, placenta, small intestine, skeletal muscle and heart.
  • 通路Protein modification; sulfatase oxidation.
  • 疾病相关Defects in SUMF1 are the cause of multiple sulfatase deficiency (MSD) [MIM:272200]. MSD is a clinically and biochemically heterogeneous disorder caused by the simultaneous impairment of all sulfatases, due to defective post-translational modification and activation. It combines features of individual sulfatase deficiencies such as metachromatic leukodystrophy, mucopolysaccharidosis, chondrodysplasia punctata, hydrocephalus, ichthyosis, neurologic deterioration and developmental delay. Inheritance is autosomal recessive.
  • 序列相似性Belongs to the sulfatase-modifying factor family.
  • 翻译后修饰N-glycosylated. Contains high-mannose-type oligosaccharides.
  • 细胞定位Endoplasmic reticulum lumen.
  • Information by UniProt
  • 数据库链接
  • 别名
    • MGC150436 antibody
    • AAPA3037 antibody
    • C alpha formylglycine generating enzyme 1 antibody
    • C-alpha-formylglycine-generating enzyme 1 antibody
    • FGE antibody
    • FGly generating enzyme antibody
    • MGC131853 antibody
    • Sulfatase modifying factor 1 [Precursor] antibody
    • Sulfatase-modifying factor 1 antibody
    • SUMF1 antibody
    • SUMF1_HUMAN antibody
    • UNQ3037 antibody
    see all

Anti-SUMF1 antibody 图像

  • Anti-SUMF1 antibody (ab103467) at 1/100 dilution + Hela cell line lysates at 35 µg

    Predicted band size : 40 kDa

Anti-SUMF1 antibody (ab103467)参考文献

ab103467 has not yet been referenced specifically in any publications.

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