概述

  • 产品名称人Munc 13-4肽

描述

  • 性质Synthetic
  • 氨基酸序列
    • 种属Human
    • 序列C-KQASQHALRPAP
    • 氨基酸1079 to 1090

相关产品

技术指标

Our Abpromise guarantee covers the use of ab23273 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • 应用

    Blocking - Blocking peptide for Anti-Munc 13-4 antibody (ab15723)

  • 形式Liquid
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

常规信息

  • 别名
    • FHL 3
    • FHL3
    • FLJ00067
    • HLH 3
    • HLH3
    • HPLH 3
    • HPLH3
    • Jinx
    • Munc13 4
    • Munc13-4
    • Protein unc 13 homolog D
    • Protein unc-13 homolog D
    • UN13D_HUMAN
    • Unc 13 homolog D
    • UNC 13D
    • Unc-13 homolog D (C. elegans)
    • Unc13 homolog D
    • Unc13 homolog D (C elegans)
    • UNC13, C. elegans, homolog of, D
    • UNC13D
    • Unc13h4
    see all
  • 功能Plays a role in cytotoxic granule exocytosis in lymphocytes. Required for both granule maturation and granule docking and priming at the immunologic synapse. Regulates assembly of recycling and late endosomal structures, leading to the formation of an endosomal exocytic compartment that fuses with perforin-containing granules at the immunologic synapse and licences them for exocytosis. Regulates Ca(2+)-dependent secretory lysosome exocytosis in mast cells.
  • 组织特异性Expressed at high levels in spleen, thymus and leukocytes. Also expressed in lung and placenta, and at very low levels in brain, heart, skeletal muscle and kidney. Expressed in cytotoxic T-lymphocytes (CTL) and mast cells.
  • 疾病相关Defects in UNC13D are the cause of hemophagocytic lymphohistiocytosis familial type 3 (FHL3) [MIM:608898]; also known as HPLH3. Familial hemophagocytic lymphohistiocytosis (FHL) is a genetically heterogeneous, rare autosomal recessive disorder. It is characterized by immune dysregulation with hypercytokinemia and defective natural killer cell function. The clinical features of the disease include fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hypofibrinogenemia, and neurological abnormalities ranging from irritability and hypotonia to seizures, cranial nerve deficits, and ataxia. Hemophagocytosis is a prominent feature of the disease, and a non-malignant infiltration of macrophages and activated T lymphocytes in lymph nodes, spleen, and other organs is also found.
  • 序列相似性Belongs to the unc-13 family.
    Contains 2 C2 domains.
    Contains 1 MHD1 (MUNC13 homology domain 1) domain.
    Contains 1 MHD2 (MUNC13 homology domain 2) domain.
  • 结构域The MHD1 and MHD2 domains mediate localization on recycling endosomes and lysosome.
  • 细胞定位Cytoplasm. Membrane. Late endosome. Recycling endosome. Lysosome. Colocalizes with cytotoxic granules at the plasma membrane. Localizes to endosomal exocytic vesicles.
  • Information by UniProt

Human Munc 13-4 peptide (ab23273)参考文献

ab23273 has not yet been referenced specifically in any publications.

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