Anti-Perforin CE/IVD for IHC - Hematopathology
Perforin is a pore forming protein with a mechanism of transmembrane channel formation similar to C9, and homology between perforin and C9 have been demonstrated. Perforin is found in the granules of cytotoxic T lymphocytes and natural killer cells, which functions to enable granzymes to enter the target cells and activate apoptosis. Perforin expression is upregulated in activated CD8+ T-cells, and these cells have been identified to have a major influence in Th1-associated inflammatory skin diseases. It has been suggested that perforin plays a role in alloimmunity, being involved in both the cytolytic process of rejection as well as downregulation of the T-cell mediated responses associated with the alloimmune response. Perforin-mediated cytotoxicity has also been linked to a number of autoimmune diseases. Defects in the perforin gene cause familial hemophagocytic lymphohistiocytosis type 2 (HPLH2), a rare and lethal autosomal recessive disorder of early childhood.
Spleen section
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