Recombinant Human Perforin protein (ab112345)

Overview

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Accession
    • Species
      Human
    • Sequence
      WSVRLDFGDVLLATGGPLRLQVWDQDSGRDDDLLGTCDQAPKSGSHEVRC NLNHGHLKFRYHARCLPHLGGGTCLDYVPQMLLGEPPGNRSGAVW
    • Molecular weight
      36 kDa including tags
    • Amino acids
      461 to 555

Specifications

Our Abpromise guarantee covers the use of ab112345 in the following tested applications.

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

  • Biological activity
    useful for Antibody Production and Protein Array
  • Applications

    SDS-PAGE

    ELISA

    Western blot

  • Form
    Liquid
  • Additional notes
    useful for Antibody Production and Protein Array
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.31% Glutathione, 0.79% Tris HCl
    Note: Glutathione is reduced

General Info

  • Alternative names
    • Cytolysin
    • FLH2
    • HPLH2
    • Lymphocyte pore-forming protein
    • P1
    • PERF_HUMAN
    • Perforin 1
    • perforin 1 (pore forming protein)
    • Perforin-1
    • PFP
    • PGFL
    • PIGF
    • PIGF-2
    • PLGF
    • Pore forming protein
    • prf1
    • SHGC-10760
    see all
  • Function
    Plays a key role in secretory granule-dependent cell death, and in defense against virus-infected or neoplastic cells. Plays an important role in killing other cells that are recognized as non-self by the immune system, e.g. in transplant rejection or some forms of autoimmune disease. Can insert into the membrane of target cells in its calcium-bound form, oligomerize and form large pores. Promotes cytolysis and apoptosis of target cells by facilitating the uptake of cytotoxic granzymes.
  • Involvement in disease
    Defects in PRF1 are the cause of hemophagocytic lymphohistiocytosis familial type 2 (FHL2) [MIM:603553]; also known as HPLH2. 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.
  • Sequence similarities
    Belongs to the complement C6/C7/C8/C9 family.
    Contains 1 C2 domain.
    Contains 1 EGF-like domain.
    Contains 1 MACPF domain.
  • Domain
    The C2 domain mediates calcium-dependent binding to lipid membranes. A subsequent conformation change leads to membrane insertion of beta-hairpin structures and pore formation. The pore is formed by transmembrane beta-strands.
  • Post-translational
    modifications
    N-glycosylated.
  • Cellular localization
    Cytoplasmic granule lumen. Secreted. Cell membrane. Endosome lumen. Stored in cytoplasmic granules of cytolytic T-lymphocytes and secreted into the cleft between T-lymphocyte and target cell. Inserts into the cell membrane of target cells and forms pores. Membrane insertion and pore formation requires a major conformation change. May be taken up via endocytosis involving clathrin-coated vesicles and accumulate in a first time in large early endosomes.
  • Information by UniProt

Images

  • 12.5% SDS-PAGE showing ab112345 at approximately 36.08kDa stained with Coomassie Blue.

References

ab112345 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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