概述

  • 产品名称
  • 描述
    兔多克隆抗体to PEX2
  • 经测试应用
    适用于: WBmore details
  • 种属反应性
    与反应: Mouse, Human
  • 免疫原

    KLH conjugated synthetic peptide selected from the central region of Human PEX2 (NP_000309.1)

  • 阳性对照
    • Mouse cerebellum tissue lysates

性能

应用

Our Abpromise guarantee covers the use of ab95289 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: 35 kDa.

靶标

  • 功能
    Somewhat implicated in the biogenesis of peroxisomes.
  • 疾病相关
    Defects in PEX2 are the cause of peroxisome biogenesis disorder complementation group 5 (PBD-CG5) [MIM:170993]; also known as PBD-CGF. 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 PEX2 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.
    Defects in PEX2 are a cause of infantile Refsum disease (IRD) [MIM:266510]. IRD is a mild peroxisome biogenesis disorder (PBD). Clinical features include early onset, mental retardation, minor facial dysmorphism, retinopathy, sensorineural hearing deficit, hepatomegaly, osteoporosis, failure to thrive, and hypocholesterolemia. The biochemical abnormalities include accumulation of phytanic acid, very long chain fatty acids (VLCFA), di- and trihydroxycholestanoic acid and pipecolic acid.
  • 序列相似性
    Belongs to the pex2/pex10/pex12 family.
    Contains 1 RING-type zinc finger.
  • 细胞定位
    Peroxisome membrane.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 35 kDa peroxisomal membrane protein antibody
    • PAF-1 antibody
    • PAF1 antibody
    • Peroxin-2 antibody
    • peroxisomal biogenesis factor 2 antibody
    • Peroxisomal membrane protein 3 antibody
    • peroxisomal membrane protein 3, 35kDa antibody
    • Peroxisome assembly factor 1 antibody
    • Peroxisome biogenesis factor 2 antibody
    • PEX2 antibody
    • PEX2_HUMAN antibody
    • PMP3 antibody
    • PMP35 antibody
    • PXMP3 antibody
    • RING finger protein 72 antibody
    • RNF72 antibody
    • ZWS3 antibody
    see all

图片

  • Anti-PEX2 antibody (ab95289) at 1/100 dilution + Mouse cerebellum tissue lysate at 35 µg

    Predicted band size : 35 kDa

实验方案

文献

ab95289 has not yet been referenced specifically in any publications.

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