概述

  • 产品名称Anti-PEX19抗体
    参阅全部 PEX19 一抗
  • 描述
    小鼠多克隆抗体to PEX19
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Full length human PEX19 protein (NP_002848.1)

  • 阳性对照
    • Jurkat cell lysate 293T transfected cell lysate

性能

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应用

Our Abpromise guarantee covers the use of ab88365 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: 33 kDa.

靶标

  • 功能Necessary for early peroxisomal biogenesis. Acts both as a cytosolic chaperone and as an import receptor for peroxisomal membrane proteins (PMPs). Binds and stabilizes newly synthesized PMPs in the cytoplasm by interacting with their hydrophobic membrane-spanning domains, and targets them to the peroxisome membrane by binding to the integral membrane protein PEX3. Excludes CDKN2A from the nucleus and prevents its interaction with MDM2, which results in active degradation of TP53.
  • 组织特异性Ubiquitously expressed. Isoform 1 is strongly predominant in all tissues except in utero where isoform 2 is the main form.
  • 疾病相关Defects in PEX19 are the cause of peroxisome biogenesis disorder complementation group 14 (PBD-CG14) [MIM:600279]; also known as PBD-CGJ. 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 14 distinct genetic groups as concluded from complementation studies.
    Defects in PEX19 are a cause of Zellweger syndrome (ZWS) [MIM:214100]. ZWS is a fatal peroxisome biogenesis disorder characterized by dysmorphic facial features, hepatomegaly, ocular abnormalities, renal cysts, hearing impairment, profound psychomotor retardation, severe hypotonia and neonatal seizures. Death occurs within the first year of life.
  • 序列相似性Belongs to the peroxin-19 family.
  • 细胞定位Cytoplasm. Peroxisome membrane. Mainly cytoplasmic. Some fraction membrane-associated to the outer surface of peroxisomes.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 33 kDa housekeeping protein antibody
    • D1S2223E antibody
    • HK33 antibody
    • Housekeeping gene 33kD antibody
    • OK/SW-cl.22 antibody
    • PBD12A antibody
    • Peroxin 19 antibody
    • Peroxin-19 antibody
    • Peroxisomal biogenesis factor 19 antibody
    • Peroxisomal farnesylated protein antibody
    • PEX19 antibody
    • PEX19_HUMAN antibody
    • PMP1 antibody
    • PMPI antibody
    • PXF antibody
    • PXMP1 antibody
    see all

Anti-PEX19 antibody 图像

  • Anti-PEX19 antibody (ab88365) at 1 µg/ml + Jurkat cell lysate at 50 µg

    Secondary
    Goat anti-mouse IgG at 1/5000 dilution

    Predicted band size : 33 kDa
  • All lanes : Anti-PEX19 antibody (ab88365) at 1 µg/ml

    Lane 1 : PEX19 transfected 293T cell lysate
    Lane 2 : Untransfected 293T cell lysate

    Lysates/proteins at 50 µg per lane.

    Secondary
    Goat anti mouse IgG at 1/5000 dilution

    Predicted band size : 33 kDa
    Observed band size : 33 kDa

Anti-PEX19 antibody (ab88365)参考文献

ab88365 has not yet been referenced specifically in any publications.

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