Anti-ZMPSTE24 抗体 - Carboxyterminal end (ab53533)

製品の概要

  • 製品名
    Anti-ZMPSTE24 antibody - Carboxyterminal end
    ZMPSTE24 一次抗体 製品一覧
  • 製品の詳細
    Goat polyclonal to ZMPSTE24 - Carboxyterminal end
  • アプリケーション
    適用あり: WB, ELISAmore details
  • 種交差性
    交差種: Human
    交差が予測される動物種: Chimpanzee, Rhesus monkey, Orangutan
  • 免疫原

    Synthetic peptide:

    C-ERLQALKTMKQH

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

  • ポジティブ・コントロール
    • Human Skin lysate

製品の特性

アプリケーション

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.

アプリケーション Abreviews 特記事項
WB
ELISA
  • 追加情報
    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.
  • ターゲット情報

    • 機能
      Proteolytically removes the C-terminal three residues of farnesylated proteins. Acts on lamin A/C.
    • 組織特異性
      Widely expressed. High levels in kidney, prostate, testis and ovary.
    • 関連疾患
      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.
    • 配列類似性
      Belongs to the peptidase M48A family.
    • 細胞内局在
      Endoplasmic reticulum membrane. Golgi apparatus membrane.
    • Information by UniProt
    • 参照データベース
    • 別名
      • 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

    画像

    • 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

    プロトコール

    参考文献

    ab53533 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"

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