概述

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

    Synthetic peptide conjugated to KLH, corresponding to a region within C terminal amino acids 407-438 of Human MKKS (NP_740754.1, NP_061336.1).

  • 阳性对照
    • U251 cell line lysate

性能

应用

Our Abpromise guarantee covers the use of ab123980 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 1/100 - 1/500. Predicted molecular weight: 62 kDa.

靶标

  • 功能Probable molecular chaperone. Assists the folding of proteins upon ATP hydrolysis. As part of the BBS/CCT complex may play a role in the assembly of BBSome, a complex involved in ciliogenesis regulating transports vesicles to the cilia. May play a role in protein processing in limb, cardiac and reproductive system development. May play a role in cytokinesis.
  • 组织特异性Widely expressed in adult and fetal tissues.
  • 疾病相关Defects in MKKS are the cause of McKusick-Kaufman syndrome (MKKS) [MIM:236700]. MKKS is an autosomal recessive developmental disorder. It is characterized by hydrometrocolpos, postaxial polydactyly and congenital heart defects.
    Defects in MKKS are the cause of Bardet-Biedl syndrome type 6 (BBS6) [MIM:209900]. Bardet-Biedl syndrome (BBS) is a genetically heterogeneous, autosomal recessive disorder characterized by usually severe pigmentary retinopathy, early onset obesity, polydactyly, hypogenitalism, renal malformation and mental retardation. Secondary features include diabetes mellitus, hypertension and congenital heart disease. A relatively high incidence of BBS is found in the mixed Arab populations of Kuwait and in Bedouin tribes throughout the Middle East, most likely due to the high rate of consaguinity in these populations and a founder effect.
  • 序列相似性Belongs to the TCP-1 chaperonin family.
  • 结构域The substrate-binding apical domain region is sufficient for centrosomal association.
  • 细胞定位Cytoplasm > cytoskeleton > centrosome. Cytoplasm > cytosol. The majority of the protein resides within the pericentriolar material (PCM), a proteinaceous tube surrounding centrioles. During interphase, the protein is confined to the lateral surfaces of the PCM but during mitosis it relocalizes throughout the PCM and is found at the intercellular bridge. The MKSS protein is highly mobile and rapidly shuttles between the cytosol and centrosome.
  • Information by UniProt
  • 数据库链接
  • 别名
    • Bardet Biedl syndrome 6 protein antibody
    • Bardet-Biedl syndrome 6 protein antibody
    • BBS6 antibody
    • HMCS antibody
    • KMS antibody
    • McKusick Kaufman syndrome antibody
    • McKusick Kaufman/Bardet Biedl syndromes putative chaperonin antibody
    • McKusick-Kaufman/Bardet-Biedl syndromes putative chaperonin antibody
    • Mkks antibody
    • MKKS_HUMAN antibody
    • MKS antibody
    see all

Anti-MKKS antibody 图像

  • Anti-MKKS antibody (ab123980) at 1/100 dilution + U251 cell line lysate at 35 µg

    Predicted band size : 62 kDa

Anti-MKKS antibody (ab123980)参考文献

ab123980 has not yet been referenced specifically in any publications.

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