نتایج جستجو برای: autoimmune lymphoproliferative syndrome

تعداد نتایج: 678796  

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2007
João B Oliveira Nicolas Bidère Julie E Niemela Lixin Zheng Keiko Sakai Cynthia P Nix Robert L Danner Jennifer Barb Peter J Munson Jennifer M Puck Janet Dale Stephen E Straus Thomas A Fleisher Michael J Lenardo

The p21 RAS subfamily of small GTPases, including KRAS, HRAS, and NRAS, regulates cell proliferation, cytoskeletal organization, and other signaling networks, and is the most frequent target of activating mutations in cancer. Activating germline mutations of KRAS and HRAS cause severe developmental abnormalities leading to Noonan, cardio-facial-cutaneous, and Costello syndrome, but activating g...

Journal: :Blood 2002
Jin Hong Liu Sheng Wei Thierry Lamy Yongxiang Li P K Epling-Burnette Julie Y Djeu Thomas P Loughran

Altered expression of the Fas-Fas ligand apoptotic pathway leads to lymphoproliferative and autoimmune diseases. In lpr/lpr mice and children with autoimmune lymphoproliferative syndrome, defective apoptosis is due to Fas mutations. Large granular lymphocyte (LGL) leukemia is a clonal lymphoproliferative disorder associated with rheumatoid arthritis. Leukemic LGLs are resistant to Fas-dependent...

Journal: :Journal of immunology 1998
I R Rifkin P L Channavajhala H L Kiefer A J Carmack E Landesman-Bollag B C Beaudette B Jersky D J Salant S T Ju A Marshak-Rothstein D C Seldin

MRL-lpr/lpr mice have a Fas receptor mutation that leads to abnormalities of apoptosis, lymphoproliferation, and a lupus-like autoimmune disease associated with the production of autoantibodies. Other than Fas pathway defects, little is known about molecular abnormalities that predispose to autoimmunity. Protein kinase CK2 (also termed casein kinase II), a serine-threonine protein kinase whose ...

Journal: :Atlas of Genetics and Cytogenetics in Oncology and Haematology 2011

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