Anti-ZMPSTE24抗体- Carboxyterminal end (ab53533)

概述

  • 产品名称Anti-ZMPSTE24抗体- Carboxyterminal end
    参阅全部 ZMPSTE24 一抗
  • 描述
    山羊多克隆抗体to ZMPSTE24 - Carboxyterminal end
  • 经测试应用适用于: WB, ELISAmore details
  • 种属反应性
    与反应: Human
    预测可用于: Chimpanzee, Rhesus monkey, Orangutan
  • 免疫原

    Synthetic peptide:

    C-ERLQALKTMKQH

    , corresponding to C terminal amino acids 464-475 of Human ZMPSTE24

  • 阳性对照
    • Human Skin lysate

性能

应用

Our Abpromise guarantee covers the use of ab53533 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: Peptide ELISA: Antibody detection limit dilution 1/128000.
    WB: Use at a concentration of 0.5 - 1.5 µg/ml. Detects a band of approximately 55 kDa (predicted molecular weight: 55 kDa).


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

    • 功能Proteolytically removes the C-terminal three residues of farnesylated proteins. Acts on lamin A/C.
    • 组织特异性Widely expressed. High levels in kidney, prostate, testis and ovary.
    • 疾病相关Defects in ZMPSTE24 are the cause of mandibuloacral dysplasia with type B lipodystrophy (MADB) [MIM:608612]. Mandibuloacral dysplasia (MAD) is a rare autosomal recessive disorder characterized by mandibular and clavicular hypoplasia, acroosteolysis, delayed closure of the cranial suture, joint contractures, and types A or B patterns of lipodystrophy. Type B lipodystrophy observed in MADB, is characterized by generalized fat loss.
      Defects in ZMPSTE24 are a cause of lethal tight skin contracture syndrome (LTSCS) [MIM:275210]; also called restrictive dermopathy (RD). Lethal tight skin contracture syndrome is a rare disorder mainly characterized by intrauterine growth retardation, tight and rigid skin with erosions, prominent superficial vasculature and epidermal hyperkeratosis, facial features (small mouth, small pinched nose and micrognathia), sparse/absent eyelashes and eyebrows, mineralization defects of the skull, thin dysplastic clavicles, pulmonary hypoplasia, multiple joint contractures and an early neonatal lethal course. Liveborn children usually die within the first week of life. The overall prevalence of consanguineous cases suggested an autosomal recessive inheritance.
    • 序列相似性Belongs to the peptidase M48A family.
    • 细胞定位Endoplasmic reticulum membrane. Golgi apparatus membrane.
    • Information by UniProt
    • 数据库链接
    • 别名
      • CAAX prenyl protease 1 homolog antibody
      • FACE-1 antibody
      • FACE1 antibody
      • FACE1_HUMAN antibody
      • Farnesylated proteins converting enzyme 1 antibody
      • Farnesylated proteins-converting enzyme 1 antibody
      • Prenyl protein specific endoprotease 1 antibody
      • Prenyl protein-specific endoprotease 1 antibody
      • STE24 antibody
      • Zinc metalloproteinase Ste24 homolog antibody
      • zmpste24 antibody
      see all

    Anti-ZMPSTE24 antibody - Carboxyterminal end 图像

    • ab53533 antibody (0.5 µg/ml) staining of ZMPSTE24 in Human Skin lysate (35µg protein in RIPA buffer)

      Predicted band size : 55 kDa
      Observed band size : 55 kDa

    Anti-ZMPSTE24 antibody - Carboxyterminal end (ab53533)参考文献

    ab53533 has not yet been referenced specifically in any publications.

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