Perforin: more than just an effector molecule

Perforin mediates the destruction of virus-infected or transformed cells. However, the finding that perforin deficiency is the underlying cause of familial hemophagocytic lymphohistiocytosis indicates that it also regulates the immune response.

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Bibliographic Details
Published in:Immunology today (Amsterdam. Regular ed.) 2000-06, Vol.21 (6), p.254-256
Main Authors: Stepp, Susan E, Mathew, Porunelloor A, Bennett, Michael, de Saint Basile, Geneviève, Kumar, Vinay
Format: Article
Language:English
Subjects:
Animals
Autoimmune Diseases - genetics
Autoimmune Diseases - immunology
Autoimmunity
Child, Preschool
Chromosomes, Human, Pair 10 - genetics
Cytoplasmic Granules - secretion
Cytotoxicity, Immunologic
familial hemophagocytic lymphohistiocytosis
Fas Ligand Protein
Feedback
Hemophagocytic syndrome
Histiocytosis, Non-Langerhans-Cell - genetics
Humans
Immune regulation
Infant
Killer Cells, Natural - immunology
Lymphocyte Activation
Lymphocytic Choriomeningitis - immunology
Lymphocytic choriomeningitis virus - physiology
Lymphoproliferative Disorders - genetics
Lymphoproliferative Disorders - immunology
Membrane Glycoproteins - deficiency
Membrane Glycoproteins - genetics
Membrane Glycoproteins - immunology
Membrane Glycoproteins - physiology
Mice
Mice, Inbred C57BL
Mice, Inbred MRL lpr
Mice, Knockout
Perforin
Pore Forming Cytotoxic Proteins
T-Lymphocytes, Cytotoxic - immunology
Virus Latency
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Description
Summary:Perforin mediates the destruction of virus-infected or transformed cells. However, the finding that perforin deficiency is the underlying cause of familial hemophagocytic lymphohistiocytosis indicates that it also regulates the immune response.
ISSN:0167-5699
DOI:10.1016/S0167-5699(00)01622-4