概述

  • 产品名称Anti-S2P抗体
    参阅全部 S2P 一抗
  • 描述
    小鼠单克隆抗体to S2P
  • 经测试应用适用于: WB, ELISAmore details
  • 种属反应性
    与反应: Recombinant Fragment
    预测可用于: Mouse, Cow, Human, Chinese Hamster
  • 免疫原

    Recombinant fragment: ASTLQQLSFP VRAYKRLDGS TECCNNHSLT DVCFSYRNNF NKRLHTCLPA RKAVEATQVC RTNKDCKKSS SSSFCIIPSL ETHTRLIKVK HPPQIDMLYV GHPLHLH, corresponding to amino acids 312-419 of human MBTPS2 (NP_056968) with a tag

  • 阳性对照
    • Partial recombinant human MBTPS2 protein (the immunogen)

性能

应用

Our Abpromise guarantee covers the use of ab76641 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: Use at an assay dependent dilution.
    WB: Use at a concentration of 1 - 5 µg/ml. Predicted molecular weight: 57 kDa.
    This antibody has only been tested in WB against the recombinant fragment used as immunogen. We have no data on the detection of endogenous protein.

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

    • 功能Intramembrane proteolysis of sterol-regulatory element-binding proteins (SREBPs) within the first transmembrane segment thereby releasing the N-terminal segment with a portion of the transmembrane segment attached. Site-2 cleavage comes after site-1 cleavage which takes place in the lumenal loop.
    • 组织特异性Expressed in heart, brain, placenta, lung, liver, muscle, kidney and pancreas.
    • 疾病相关Defects in MBTPS2 are the cause of ichthyosis follicularis-atrichia-photophobia syndrome (IFAPS) [MIM:308205]. A syndrome characterized by a peculiar triad of follicular ichthyosis, total or subtotal atrichia, and photophobia of varying degree. Histopathologically, the epidermal granular layer is generally well-preserved or thickened at the infundibulum. Hair follicles are poorly developed and tend to be surrounded by an inflammatory infiltrate. A subgroup of patients is described with lamellar rather than follicular ichthyosis. Non-consistent features may include growth and psychomotor retardation, aganglionic megacolon, seizures and nail dystrophy.
      Defects in MBTPS2 are a cause of keratosis follicularis spinulosa decalvans X-linked (KFSDX) [MIM:308800]. A rare disorder affecting the skin and the eye. Affected men show thickening of the skin of the neck, ears, and extremities, especially the palms and soles, loss of eyebrows, eyelashes and beard, thickening of the eyelids with blepharitis and ectropion, and corneal degeneration.
    • 序列相似性Belongs to the peptidase M50A family.
    • 细胞定位Membrane. Cytoplasm.
    • Information by UniProt
    • 数据库链接
    • 别名
      • BRESEK antibody
      • Endopeptidase S2P antibody
      • IFAP antibody
      • KFSDX antibody
      • MBTP2_HUMAN antibody
      • MBTPS2 antibody
      • Membrane bound transcription factor site 2 protease antibody
      • membrane-bound transcription factor peptidase, site 2 antibody
      • membrane-bound transcription factor protease, site 2 antibody
      • Membrane-bound transcription factor site-2 protease antibody
      • S2P antibody
      • S2P endopeptidase antibody
      • Site 2 protease antibody
      • SREBPs intramembrane protease antibody
      • Sterol regulatory element-binding proteins intramembrane protease antibody
      see all

    Anti-S2P antibody 图像

    • Anti-S2P antibody (ab76641) at 5 µg/ml + immunogen at 0.2 µg

      Secondary
      Goat Anti-Mouse IgG (H&L)-HRP Conjugate at 1/5000 dilution

      Predicted band size : 57 kDa
      Observed band size : 38 kDa (why is the actual band size different from the predicted?)

    Anti-S2P antibody (ab76641)参考文献

    ab76641 has not yet been referenced specifically in any publications.

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    Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"