概述

  • 产品名称Anti-MPV17抗体
    参阅全部 MPV17 一抗
  • 描述
    小鼠多克隆抗体to MPV17
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Full length protein (Human)

性能

应用

Our Abpromise guarantee covers the use of ab67466 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 1/500 - 1/1000. Detects a band of approximately 17 kDa (predicted molecular weight: 20 kDa).

靶标

  • 功能Involved in mitochondria homeostasis. May be involved in the metabolism of reactive oxygen species and control of oxidative phosphorylation and mitochondrial DNA (mtDNA) maintenance.
  • 组织特异性Ubiquitous. Expressed in pancreas, kidney, muscle, liver, lung, placenta, brain and heart.
  • 疾病相关Defects in MPV17 are a cause of hepatocerebral mitochondrial DNA depletion syndrome (MDS) [MIM:251880]. MDS is a clinically heterogeneous group of disorders characterized by a reduction in mitochondrial DNA (mtDNA) copy number. Primary mtDNA depletion is inherited as an autosomal recessive trait and may affect single organs, typically muscle or liver, or multiple tissues. Individuals with the hepatocerebral form of mitochondrial DNA depletion syndrome have early progressive liver failure and neurologic abnormalities, hypoglycemia, and increased lactate in body fluids.
    Defects in MPV17 are the cause of Navajo neurohepatopathy (NN) [MIM:256810]. NN is an autosomal recessive disease that is prevalent among Navajo children in the southwestern United States. The major clinical features are hepatopathy, peripheral neuropathy, corneal anesthesia and scarring, acral mutilation, cerebral leukoencephalopathy, failure to thrive, and recurrent metabolic acidosis with intercurrent infections. Infantile, childhood, and classic forms of NN have been described. Mitochondrial DNA depletion was detected in the livers of patients, suggesting a primary defect in mtDNA maintenance.
  • 序列相似性Belongs to the peroxisomal membrane protein PXMP2/4 family.
  • 细胞定位Mitochondrion inner membrane.
  • Information by UniProt
  • 数据库链接
  • 别名
    • Glomerulosclerosis antibody
    • Mpv17 antibody
    • Mpv17 human homolog of glomerulosclerosis and nephrotic syndrome antibody
    • MpV17 mitochondrial inner membrane protein antibody
    • MPV17_HUMAN antibody
    • MTDPS6 antibody
    • Protein Mpv17 antibody
    • SYM1 antibody
    see all

Anti-MPV17 antibody 图像

  • All lanes : Anti-MPV17 antibody (ab67466) at 1/500 dilution

    Lane 1 : MPV17 transfected 293T cell lysate
    Lane 2 : Non-transfected 293T cell lysate

    Lysates/proteins at 25 µg/ml per lane.

    Secondary
    Goat Anti-Mouse IgG (H&L)-HRP Conjugate at 1/2500 dilution

    Predicted band size : 20 kDa
    Observed band size : 17 kDa (why is the actual band size different from the predicted?)

Anti-MPV17 antibody (ab67466)参考文献

ab67466 has not yet been referenced specifically in any publications.

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