Recombinant人PEX19 protein (ab111623)

概述

  • 产品名称Recombinant人PEX19 protein
  • 蛋白长度Full length protein

描述

  • 性质Recombinant
  • 来源Escherichia coli
  • 氨基酸序列
    • AccessionP40855
    • 种属Human
    • 序列MGSSHHHHHHSSGLVPRGSHMAAAEEGCSVGAEADRELEELLESALDDFD KAKPSPAPPSTTTAPDASGPQKRSPGDTAKDALFASQEKFFQELFDSELA SQATAEFEKAMKELAEEEPHLVEQFQKLSEAAGRVGSDMTSQQEFTSCLK ETLSGLAKNATDLQNSSMSEEELTKAMEGLGMDEGDGEGNILPIMQSIMQ NLLSKDVLYPSLKEITEKYPEWLQSHRESLPPEQFEKYQEQHSVMCKICE QFEAETPTDSETTQKARFEMVLDLMQQLQDLGHPPKELAGEMPPGLNFDL DALNLSGPPGASGEQC
    • 分子量35 kDa including tags
    • 氨基酸1 to 296
    • 标签His tag N-Terminus

技术指标

Our Abpromise guarantee covers the use of ab111623 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • 应用

    SDS-PAGE

    Mass Spectrometry

  • 质谱法
    MALDI-TOF
  • 纯度> 90 % SDS-PAGE.
    ab111623 is purified using conventional chromatography techniques.
  • 形式Liquid
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

    pH: 8.00
    Constituents: 10% Glycerol, 0.32% Tris HCl

常规信息

  • 别名
    • 33 kDa housekeeping protein
    • D1S2223E
    • HK33
    • Housekeeping gene 33kD
    • OK/SW-cl.22
    • PBD12A
    • Peroxin 19
    • Peroxin-19
    • Peroxisomal biogenesis factor 19
    • Peroxisomal farnesylated protein
    • PEX19
    • PEX19_HUMAN
    • PMP1
    • PMPI
    • PXF
    • PXMP1
    see all
  • 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

Recombinant Human PEX19 protein 图像

  • 15% SDS-PAGE showing ab111623 at approximately 34.6kDa (3µg).
    (Molecular weight on SDS-PAGE will appear higher)

Recombinant Human PEX19 protein (ab111623)参考文献

ab111623 has not yet been referenced specifically in any publications.

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