概述

  • 产品名称Anti-PEX7抗体
    参阅全部 PEX7 一抗
  • 描述
    兔多克隆抗体to PEX7
  • 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, Chicken, Guinea pig, Cow, Dog
  • 免疫原

    Synthetic peptide corresponding to a region within N terminal amino acids 2-51 (SAVCGGAARM LRTPGRHGYA AEFSPYLPGR LACATAQHYG IAGCGTLLIL) of Human PEX7 (NP_000279).

  • 阳性对照
    • MCF7 cell lysate

性能

  • 形式Liquid
  • 存放说明Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid repeated freeze / thaw cycles.
  • 存储溶液Preservative: None
    Constituents: 2% Sucrose, PBS
  • Concentration information loading...
  • 纯度Immunogen affinity purified
  • 克隆多克隆
  • 同种型IgG
  • 研究领域

应用

Our Abpromise guarantee covers the use of ab90822 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 1 µg/ml. Predicted molecular weight: 36 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.

靶标

  • 功能Binds to the N-terminal PTS2-type peroxisomal targeting signal and plays an essential role in peroxisomal protein import.
  • 组织特异性Ubiquitous. Highest expression in pancreas, skeletal muscle and heart.
  • 疾病相关Defects in PEX7 are the cause of peroxisome biogenesis disorder complementation group 11 (PBD-CG11) [MIM:614879]. PBD refers to a group of peroxisomal disorders arising from a failure of protein import into the peroxisomal membrane or matrix. The PBD group is comprised of four disorders: Zellweger syndrome (ZWS), neonatal adrenoleukodystrophy (NALD), infantile Refsum disease (IRD), and classical rhizomelic chondrodysplasia punctata (RCDP). ZWS, NALD and IRD are distinct from RCDP and constitute a clinical continuum of overlapping phenotypes known as the Zellweger spectrum. The PBD group is genetically heterogeneous with at least 13 distinct genetic groups as concluded from complementation studies.
    Defects in PEX7 are the cause of rhizomelic chondrodysplasia punctata type 1 (RCDP1) [MIM:215100]. RCDP1 is characterized by rhizomelic shortening of femur and humerus, vertebral disorders, cataract, cutaneous lesions and severe mental retardation.
    Defects in PEX7 are the cause of peroxisome biogenesis disorder 9B (PBD9B) [MIM:614879]. A peroxisome biogenesis disorder with unusually mild clinical and biochemical manifestations. Affected individuals manifest a variable phenotype similar to, and in some cases indistinguishable from, classic Refsum disease. Variable features include ocular abnormalities, sensorimotor neuropathy, ichthyosis, deafness, chondrodysplasia punctata without rhizomelia or growth failure.
  • 序列相似性Belongs to the WD repeat peroxin-7 family.
    Contains 6 WD repeats.
  • 细胞定位Peroxisome. Cytoplasm.
  • Information by UniProt
  • 数据库链接
  • 别名
    • PBD9B antibody
    • PCDP1 antibody
    • Peroxin 7 antibody
    • Peroxin-7 antibody
    • Peroxisomal PTS2 receptor antibody
    • Peroxisomal targeting signal 2 receptor antibody
    • Peroxisome biogenesis factor 7 antibody
    • Peroxisome targeting signal 2 receptor antibody
    • PEX7 antibody
    • PEX7 protein antibody
    • PEX7_HUMAN antibody
    • PTS2 receptor antibody
    • PTS2R antibody
    • RCDP1 antibody
    • RD antibody
    see all

Anti-PEX7 antibody 图像

  • Anti-PEX7 antibody (ab90822) at 1 µg/ml (in 5% skim milk / PBS buffer) + MCF7 cell lysate at 10 µg

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

    Predicted band size : 36 kDa

Anti-PEX7 antibody (ab90822)参考文献

ab90822 has not yet been referenced specifically in any publications.

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