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Gene Review

HPLH1  -  hemophagocytic lymphohistiocytosis 1

Homo sapiens

Synonyms: FHL1, HLH1
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Disease relevance of HPLH1


Psychiatry related information on HPLH1

  • We therefore assessed the potential of immunochemotherapy with a core combination of steroids and etoposide to control EBV-HLH in 17 infants and children who met stringent diagnostic criteria for this reactive disorder of the mononuclear phagocyte system [3].

High impact information on HPLH1


Chemical compound and disease context of HPLH1


Biological context of HPLH1


Anatomical context of HPLH1


Associations of HPLH1 with chemical compounds


Other interactions of HPLH1


Analytical, diagnostic and therapeutic context of HPLH1


  1. Lethal hemophagocytic lymphohistiocytosis in Hermansky-Pudlak syndrome type II. Enders, A., Zieger, B., Schwarz, K., Yoshimi, A., Speckmann, C., Knoepfle, E.M., Kontny, U., Müller, C., Nurden, A., Rohr, J., Henschen, M., Pannicke, U., Niemeyer, C., Nurden, P., Ehl, S. Blood (2006) [Pubmed]
  2. Peripheral T-cell lymphoma associated with hemophagocytic syndrome and hemophagocytic lymphohistiocytosis of children: do they share something? Aricò, M. Blood (1990) [Pubmed]
  3. Effective control of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis with immunochemotherapy. Histiocyte Society. Imashuku, S., Hibi, S., Ohara, T., Iwai, A., Sako, M., Kato, M., Arakawa, H., Sotomatsu, M., Kataoka, S., Asami, K., Hasegawa, D., Kosaka, Y., Sano, K., Igarashi, N., Maruhashi, K., Ichimi, R., Kawasaki, H., Maeda, N., Tanizawa, A., Arai, K., Abe, T., Hisakawa, H., Miyashita, H., Henter, J.I. Blood (1999) [Pubmed]
  4. A proportion of patients with lymphoma may harbor mutations of the perforin gene. Clementi, R., Locatelli, F., Dupré, L., Garaventa, A., Emmi, L., Bregni, M., Cefalo, G., Moretta, A., Danesino, C., Comis, M., Pession, A., Ramenghi, U., Maccario, R., Aricò, M., Roncarolo, M.G. Blood (2005) [Pubmed]
  5. Development of hemophagocytic lymphohistiocytosis in triplets infected with HHV-8. Grossman, W.J., Radhi, M., Schauer, D., Gerday, E., Grose, C., Goldman, F.D. Blood (2005) [Pubmed]
  6. Getting secretory granules ready for prime time. Zhang, B., Ginsburg, D. Cell (2003) [Pubmed]
  7. The functional basis for hemophagocytic lymphohistiocytosis in a patient with co-inherited missense mutations in the perforin (PFN1) gene. Voskoboinik, I., Thia, M.C., De Bono, A., Browne, K., Cretney, E., Jackson, J.T., Darcy, P.K., Jane, S.M., Smyth, M.J., Trapani, J.A. J. Exp. Med. (2004) [Pubmed]
  8. Spectrum of perforin gene mutations in familial hemophagocytic lymphohistiocytosis. Göransdotter Ericson, K., Fadeel, B., Nilsson-Ardnor, S., Söderhäll, C., Samuelsson, A., Janka, G., Schneider, M., Gürgey, A., Yalman, N., Révész, T., Egeler, R., Jahnukainen, K., Storm-Mathiesen, I., Haraldsson , A., Poole, J., de Saint Basile, G., Nordenskjöld, M., Henter, J. Am. J. Hum. Genet. (2001) [Pubmed]
  9. Immune evasion of Borrelia burgdorferi by acquisition of human complement regulators FHL-1/reconectin and Factor H. Kraiczy, P., Skerka, C., Kirschfink, M., Brade, V., Zipfel, P.F. Eur. J. Immunol. (2001) [Pubmed]
  10. Hemophagocytic lymphohistiocytosis in infancy and childhood. Imashuku, S., Hlbi, S., Todo, S. J. Pediatr. (1997) [Pubmed]
  11. Bone marrow transplantation from genetically HLA-nonidentical donors in children with fatal inherited disorders excluding severe combined immunodeficiencies: use of two monoclonal antibodies to prevent graft rejection. Jabado, N., Le Deist, F., Cant, A., De Graeff-Meeders, E.R., Fasth, A., Morgan, G., Vellodi, A., Hale, G., Bujan, W., Thomas, C., Cavazzana-Calvo, M., Wijdenes, J., Fischer, A. Pediatrics (1996) [Pubmed]
  12. Management of severe neutropenia with cyclosporin during initial treatment of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis. Imashuku, S., Hibi, S., Kuriyama, K., Tabata, Y., Hashida, T., Iwai, A., Kato, M., Yamashita, N., Oda MUchida, M., Kinugawa, N., Sawada, M., Konno, M. Leuk. Lymphoma (2000) [Pubmed]
  13. X-linked lymphoproliferative syndrome presenting with systemic lymphocytic vasculitis. Kanegane, H., Ito, Y., Ohshima, K., Shichijo, T., Tomimasu, K., Nomura, K., Futatani, T., Sumazaki, R., Miyawaki, T. Am. J. Hematol. (2005) [Pubmed]
  14. Localization of a gene for familial hemophagocytic lymphohistiocytosis at chromosome 9q21.3-22 by homozygosity mapping. Ohadi, M., Lalloz, M.R., Sham, P., Zhao, J., Dearlove, A.M., Shiach, C., Kinsey, S., Rhodes, M., Layton, D.M. Am. J. Hum. Genet. (1999) [Pubmed]
  15. Hemophagocytic lymphohistiocytosis is associated with deficiencies of cellular cytolysis but normal expression of transcripts relevant to killer-cell-induced apoptosis. Schneider, E.M., Lorenz, I., Müller-Rosenberger, M., Steinbach, G., Kron, M., Janka-Schaub, G.E. Blood (2002) [Pubmed]
  16. Molecular dissection of the signaling and costimulatory functions of CD150 (SLAM): CD150/SAP binding and CD150-mediated costimulation. Howie, D., Simarro, M., Sayos, J., Guirado, M., Sancho, J., Terhorst, C. Blood (2002) [Pubmed]
  17. Unusual immunophenotype of CD8+ T cells in familial hemophagocytic lymphohistiocytosis. Karandikar, N.J., Kroft, S.H., Yegappan, S., Rogers, B.B., Aquino, V.M., Lee, K.M., Kumar, V., Guenaga, F.J., Jaffe, E.S., Douek, D.C., McKenna, R.W. Blood (2004) [Pubmed]
  18. Successful correction of hemophagocytic lymphohistiocytosis with related or unrelated bone marrow transplantation. Baker, K.S., DeLaat, C.A., Steinbuch, M., Gross, T.G., Shapiro, R.S., Loechelt, B., Harris, R., Filipovich, A.H. Blood (1997) [Pubmed]
  19. A single amino acid change, A91V, leads to conformational changes that can impair processing to the active form of perforin. Trambas, C., Gallo, F., Pende, D., Marcenaro, S., Moretta, L., De Fusco, C., Santoro, A., Notarangelo, L., Arico, M., Griffiths, G.M. Blood (2005) [Pubmed]
  20. Frequency and severity of central nervous system lesions in hemophagocytic lymphohistiocytosis. Haddad, E., Sulis, M.L., Jabado, N., Blanche, S., Fischer, A., Tardieu, M. Blood (1997) [Pubmed]
  21. Treatment of familial hemophagocytic lymphohistiocytosis with antithymocyte globulins, steroids, and cyclosporin A. Stéphan, J.L., Donadieu, J., Ledeist, F., Blanche, S., Griscelli, C., Fischer, A. Blood (1993) [Pubmed]
  22. Treatment of hemophagocytic lymphohistiocytosis with chemotherapy and bone marrow transplantation: a single-center study of 22 cases. Blanche, S., Caniglia, M., Girault, D., Landman, J., Griscelli, C., Fischer, A. Blood (1991) [Pubmed]
  23. Requirement for etoposide in the treatment of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis. Imashuku, S., Kuriyama, K., Teramura, T., Ishii, E., Kinugawa, N., Kato, M., Sako, M., Hibi, S. J. Clin. Oncol. (2001) [Pubmed]
  24. Interleukin 1 receptor antagonist to treat cytophagic histiocytic panniculitis with secondary hemophagocytic lymphohistiocytosis. Behrens, E.M., Kreiger, P.A., Cherian, S., Cron, R.Q. J. Rheumatol. (2006) [Pubmed]
  25. Griscelli syndrome: report of the first peripheral blood stem cell transplant and the role of mutations in the RAB27A gene as an indication for BMT. Schuster, F., Stachel, D.K., Schmid, I., Baumeister, F.A., Graubner, U.B., Weiss, M., Haas, R.J., Belohradsky, B.H. Bone Marrow Transplant. (2001) [Pubmed]
  26. Elevated circulating levels of interleukin-1 receptor antagonist but not IL-1 agonists in hemophagocytic lymphohistiocytosis. Henter, J.I., Andersson, B., Elinder, G., Jakobson, A., Lübeck, P.O., Söder, O. Med. Pediatr. Oncol. (1996) [Pubmed]
  27. Adult onset and atypical presentation of hemophagocytic lymphohistiocytosis in siblings carrying PRF1 mutations. Clementi, R., Emmi, L., Maccario, R., Liotta, F., Moretta, L., Danesino, C., Aricó, M. Blood (2002) [Pubmed]
  28. Differential expression of granzymes A and B in human cytotoxic lymphocyte subsets and T regulatory cells. Grossman, W.J., Verbsky, J.W., Tollefsen, B.L., Kemper, C., Atkinson, J.P., Ley, T.J. Blood (2004) [Pubmed]
  29. Increased IL-16 levels in hemophagocytic lymphohistiocytosis. Takada, H., Ohga, S., Mizuno, Y., Nomura, A., Hara, T. J. Pediatr. Hematol. Oncol. (2004) [Pubmed]
  30. Successful stem cell transplantation following orthotopic liver transplantation from the same haploidentical family donor in a girl with hemophagocytic lymphohistiocytosis. Matthes-Martin, S., Peters, C., Königsrainer, A., Fritsch, G., Lion, T., Heitger, A., Kapelari, K., Kronberger, M., Offner, F., Wrba, F., Margreiter, R., Gadner, H. Blood (2000) [Pubmed]
  31. Perforin expression in cytotoxic lymphocytes from patients with hemophagocytic lymphohistiocytosis and their family members. Kogawa, K., Lee, S.M., Villanueva, J., Marmer, D., Sumegi, J., Filipovich, A.H. Blood (2002) [Pubmed]
  32. An animal model of hemophagocytic lymphohistiocytosis (HLH): CD8+ T cells and interferon gamma are essential for the disorder. Jordan, M.B., Hildeman, D., Kappler, J., Marrack, P. Blood (2004) [Pubmed]
  33. Primary necrotizing lymphocytic central nervous system vasculitis due to perforin deficiency in a four-year-old girl. Moshous, D., Feyen, O., Lankisch, P., Schwarz, K., Schaper, J., Schneider, M., Dilloo, D., Laws, H.J., Schwahn, B.C., Niehues, T. Arthritis Rheum. (2007) [Pubmed]
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