Mouse anti-Human Perforin Monoclonal Antibody | anti-PRF1 antibody
Mouse Anti-Human Perforin Detection Antibody, Biotin Conjugated Clone B-D48
NCBI and Uniprot Product Information
NCBI Description
The protein encoded by this gene has structural and functional similarities to complement component 9 (C9). Like C9, this protein creates transmembrane tubules and is capable of lysing non-specifically a variety of target cells. This protein is one of the main cytolytic proteins of cytolytic granules, and it is known to be a key effector molecule for T-cell- and natural killer-cell-mediated cytolysis. Defects in this gene cause familial hemophagocytic lymphohistiocytosis type 2 (HPLH2), a rare and lethal autosomal recessive disorder of early childhood. Alternative splicing results in multiple transcript variants encoding the same protein. [provided by RefSeq, Jul 2008]
Uniprot Description
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. Ref.10 Ref.11 Ref.13 Ref.14 Ref.15 Ref.17
Subunit structure: Monomer, as sobluble protein. Homooligomer. Oligomerization is required for pore formation. Ref.14 Ref.17
Subcellular location: Cytoplasmic granule lumen. Secreted. Cell membrane; Multi-pass membrane protein. Endosome lumen. Note: 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. Ref.13 Ref.14 Ref.17
Induction: Repressed by contact with target cells. Ref.9
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. Ref.16
Post-translational modification: N-glycosylated
By similarity.
Involvement in disease: Familial hemophagocytic lymphohistiocytosis 2 (FHL2) [MIM:603553]: A rare disorder characterized by immune dysregulation with hypercytokinemia, defective function of natural killer cell, and massive infiltration of several organs by activated lymphocytes and macrophages. The clinical features of the disease include fever, hepatosplenomegaly, cytopenia, and less frequently neurological abnormalities ranging from irritability and hypotonia to seizures, cranial nerve deficits and ataxia.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.18 Ref.19
Sequence similarities: Belongs to the complement C6/C7/C8/C9 family.Contains 1 C2 domain.Contains 1 EGF-like domain.Contains 1 MACPF domain.
Research Articles on PRF1
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Product Notes
The PRF1 prf1 (Catalog #AAA335270) is an Antibody produced from Mouse and is intended for research purposes only. The product is available for immediate purchase. The Mouse Anti-Human Perforin Detection Antibody, Biotin Conjugated Clone B-D48 reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's Perforin can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA), ELISpot (ES). ELISA Detection Antibody. This Antibody can be used as Detection in a human Perforin sandwich Immunoassay to detect human Perforin in combination withhuman Perforin Capture Antibody (Cat n degree879.860.001). The suggested coating concentration range below should be optimised by each laboratory for each application. Researchers should empirically determine the suitability of the PRF1 prf1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Perforin, Monoclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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