製品の概要

  • 製品名Anti-PEX19 antibody
    PEX19 一次抗体 製品一覧
  • 製品の詳細
    Rabbit polyclonal to PEX19
  • アプリケーション適用あり: WBmore details
  • 種交差性
    交差種: Human
  • 免疫原

    Synthetic peptide conjugated to KLH, corresponding to a region within N terminal amino acids 32-61 of Human PEX19 (NP_002848.1, NP_001180573.1).

  • ポジティブ・コントロール
    • HeLa cell lysate.

製品の特性

アプリケーション

Our Abpromise guarantee covers the use of ab123315 in the following tested applications.

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

アプリケーション Abreviews 特記事項
WB 1/100 - 1/500. Predicted molecular weight: 33 kDa.

ターゲット情報

  • 機能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.
  • 組織特異性Ubiquitously expressed. Isoform 1 is strongly predominant in all tissues except in utero where isoform 2 is the main form.
  • 関連疾患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.
  • 配列類似性Belongs to the peroxin-19 family.
  • 細胞内局在Cytoplasm. Peroxisome membrane. Mainly cytoplasmic. Some fraction membrane-associated to the outer surface of peroxisomes.
  • Information by UniProt
  • 参照データベース
  • 別名
    • 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 画像

  • Anti-PEX19 antibody (ab123315) at 1/100 dilution + HeLa cell lysate at 35 µg
    Developed using the ECL technique

    Predicted band size : 33 kDa

Anti-PEX19 antibody (ab123315) 使用論文

ab123315 has not yet been referenced specifically in any publications.

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