概述

  • 产品名称Anti-PANK2抗体
    参阅全部 PANK2 一抗
  • 描述
    兔多克隆抗体to PANK2
  • 经测试应用适用于: WB, ELISAmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Synthetic peptide from the central region of human PANK2 conjugated to KLH.

  • 阳性对照
    • Mouse liver tissue lysate.

性能

应用

Our Abpromise guarantee covers the use of ab71381 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
ELISA
  • 应用说明ELISA: 1/1000.
    WB: 1/100 - 1/500. Detects bands of approximately 30 and 63 kDa (predicted molecular weight: 63 kDa).


    Not yet tested in other applications.
    Optimal dilutions/concentrations should be determined by the end user.
  • 靶标

    • 功能May be the master regulator of the CoA biosynthesis.
    • 组织特异性Ubiquitous.
    • 通路Cofactor biosynthesis; coenzyme A biosynthesis; CoA from (R)-pantothenate: step 1/5.
    • 疾病相关Defects in PANK2 are the cause of neurodegeneration with brain iron accumulation type 1 (NBIA1) [MIM:234200]; also known as pantothenate kinase-associated neurodegeneration (PKAN) or Hallervorden-Spatz syndrome (HSS). It is an autosomal recessive neurodegenerative disorder associated with iron accumulation in the brain, primarily in the basal ganglia. Clinical manifestations include progressive muscle spasticity, hyperreflexia, muscle rigidity, dystonia, dysarthria, and intellectual deterioration which progresses to severe dementia over several years. It is clinically classified into classic, atypical, and intermediate phenotypes. Classic forms present with onset in the first decade, rapid progression, loss of independent ambulation within 15 years. Atypical forms have onset in the second decade, slow progression, maintenance of independent ambulation up to 40 years later. Intermediate forms manifest onset in the first decade with slow progression or onset in the second decade with rapid progression. Patients with early onset tend to also develop pigmentary retinopathy, whereas those with later onset tend to also have speech disorders and psychiatric features. All patients have the 'eye of the tiger' sign on brain MRI.
      Defects in PANK2 are the cause of hypoprebetalipoproteinemia, acanthocytosis, retinitis pigmentosa, and pallidal degeneration (HARP) [MIM:607236]. HARP is a rare syndrome with many clinical similarities to NBIA1.
    • 序列相似性Belongs to the type II pantothenate kinase family.
    • 细胞定位Cytoplasm and Mitochondrion.
    • Information by UniProt
    • 数据库链接
    • 别名
      • 4933409I19Rik antibody
      • AI642621 antibody
      • C20orf48 antibody
      • Hallervorden Spatz syndrome antibody
      • HARP antibody
      • hPANK2 antibody
      • HSS antibody
      • MGC118448 antibody
      • MGC15053 antibody
      • mitochondrial antibody
      • NBIA1 antibody
      • PANK2 antibody
      • PANK2_HUMAN antibody
      • Pantothenate kinase 2 (Hallervorden Spatz syndrome) antibody
      • Pantothenate kinase 2 antibody
      • Pantothenate kinase 2 mitochondrial antibody
      • Pantothenic acid kinase 2 antibody
      • PKAN antibody
      • RP23 387C21.4 antibody
      see all

    Anti-PANK2 antibody 图像

    • Anti-PANK2 antibody (ab71381) at 1/40 dilution + mouse liver tissue lysate at 35 µg

      Predicted band size : 63 kDa
      Observed band size : 63 kDa
      Additional bands at : 30 kDa. We are unsure as to the identity of these extra bands.

    Anti-PANK2 antibody (ab71381)参考文献

    ab71381 has not yet been referenced specifically in any publications.

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