製品の概要

製品の詳細

  • 由来Synthetic
  • アミノ酸配列
    • 生物種Human
    • 配列C-DSIGKDGEKQDL
    • 領域392 to 403

特性

Our Abpromise guarantee covers the use of ab22980 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

    Blocking

  • 製品の状態Liquid
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

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

関連情報

  • 別名
    • 2610008O20Rik
    • NALD
    • OTTMUSP00000005454
    • Peroxin 13
    • Peroxin-13
    • Peroxin13
    • Peroxisomal membrane protein PEX 13
    • Peroxisomal membrane protein PEX13
    • Peroxisome biogenesis factor 13
    • PEX 13
    • pex13
    • PEX13_HUMAN
    • RP23-188K3.1
    • ZWS
    see all
  • 機能Component of the peroxisomal translocation machinery with PEX14 and PEX17. Functions as a docking factor for the predominantly cytoplasmic PTS1 receptor (PAS10/PEX5). Involved in the import of PTS1 and PTS2 proteins.
  • 関連疾患Defects in PEX13 are the cause of peroxisome biogenesis disorder complementation group 13 (PBD-CG13) [MIM:601789]; also known as PBD-CGH. PBD-CG13 is a peroxisomal disorder arising from a failure of protein import into the peroxisomal membrane or matrix. The peroxisome biogenesis disorders (PBD group) are genetically heterogeneous with at least 14 distinct genetic groups as concluded from complementation studies. Include disorders are: 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 (PBD-ZSS).
    Defects in PEX13 are a cause of adrenoleukodystrophy neonatal (NALD) [MIM:202370]. NALD is a peroxisome biogenesis disorder (PBD) characterized by the accumulation of very long-chain fatty acids, adrenal insufficiency and mental retardation.
  • 配列類似性Belongs to the peroxin-13 family.
    Contains 1 SH3 domain.
  • 細胞内局在Peroxisome membrane.
  • Information by UniProt

Human PEX13 peptide (ab22980) 使用論文

ab22980 has not yet been referenced specifically in any publications.

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