概述

  • 产品名称Anti-ATPAF2抗体
    参阅全部 ATPAF2 一抗
  • 描述
    兔多克隆抗体to ATPAF2
  • 特异性This antibody detects endogenous levels of total ATPAF2 protein.
  • 经测试应用适用于: ELISA, WBmore details
  • 种属反应性
    与反应: Human
    预测可用于: Mouse
  • 免疫原

    A synthesized peptide derived from the internal region of human ATPAF2.

  • 阳性对照
    • Extracts from Jurkat cells.

性能

应用

Our Abpromise guarantee covers the use of ab74235 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
ELISA 1/40000.
WB 1/500 - 1/1000. Detects a band of approximately 33 kDa (predicted molecular weight: 33 kDa).

靶标

  • 相关性ATPAF2 belongs to the ATP12 family. It may play a role in the assembly of the F1 component of the mitochondrial ATP synthase (ATPase). Defects in ATPAF2 are the cause of complex V mitochondrial respiratory chain ATPAF2 subunit deficiency (ATPAF2 deficiency) [MIM:604273]; also called ATP synthase deficiency or ATPase deficiency. ATPAF2 deficiency seems to be an early presenting disease in which lactic acidosis, dysmorphic features, and methyl glutaconic aciduria can be major clues in the diagnosis. Dysmorphic features include a large mouth, prominent nasal bridge, micrognathia, rocker-bottom feet and flexion contractures of the limbs associated with camptodactyly. Patients are hypertonic and have an enlarged liver, hypoplastic kidneys and elevated lactate levels in urine, plasma and cerebro spinal fluid (CSF).
  • 细胞定位Mitochondrion
  • 数据库链接
  • 别名
    • ATP synthase mitochondrial F1 complex assembly factor 2 antibody
    • ATP12 antibody
    • ATP12 homolog antibody
    • ATP12p antibody
    • LP3663 antibody
    see all

Anti-ATPAF2 antibody 图像

  • All lanes : Anti-ATPAF2 antibody (ab74235) at 1/500 dilution

    Lane 1 : Extracts from Jurkat cells
    Lane 2 : Extracts from Jurkat cells plus 5µg immunizing peptide

    Lysates/proteins at 5 µg per lane.


    Predicted band size : 33 kDa
    Observed band size : 33 kDa

Anti-ATPAF2 antibody (ab74235)参考文献

ab74235 has not yet been referenced specifically in any publications.

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