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Scholar Results 1 - 10 of about 21 citing DeFranco: Defective function of Fas in patients with type 1 diabetes associated with other.... (0.07 sec) 

Autoimmune lymphoproliferative syndromes: genetic defects of apoptosis …


F Rieux-Laucat, F Le Deist, A Fischer - Cell Death & Differentiation, 2003 - nature.com
Human and mouse natural mutants presenting with lymphoproliferative syndrome and autoimmunity
(ALPS) have enlightened the role of the Fas and FasL in lymphocyte cell death and peripheral
tolerance. Further study of the genetic basis of the human pathology led to the ...
Cited by 120 - Related articles - BL Direct - All 3 versions

Cell-death signaling and human disease


F Rieux-Laucat, A Fischer, FL Deist - Current opinion in immunology, 2003 - Elsevier
The autoimmune lymphoproliferative syndrome (ALPS) in humans and the lpr mouse strain are
the first examples of primary apoptosis defects caused by inherited death-receptor
mutations. They illustrate the role of Fas and Fas ligand in the control of autoimmune ...
Cited by 88 - Related articles - All 5 versions

Unmasking Evans syndrome: T-cell phenotype and apoptotic response reveal …

- hematologylibrary.org - Free from Publisher
DT Teachey, CS Manno, KM Axsom, T … - …, 2005 - bloodjournal.hematologylibrary.org
Autoimmune lymphoproliferative syndrome (ALPS) is a rare disorder of disrupted lymphocyte
homeostasis. Clinical manifestations of ALPS vary but typically include autoimmune
cytopenias, organomegaly, lymphadenopathy, and increased risk of malignancies. A ...
Cited by 55 - Related articles - All 9 versions

Defect in activation-induced cell death in non-obese diabetic (NOD) T lymphocytes


B Decallonne, E van Etten, A Giulietti, K Casteels, L … - Journal of Autoimmunity, 2003 - Elsevier
Activation-induced cell death (AICD) represents a major means of peripheral tolerance
induction, eliminating effector cells. NOD mice, a widely used model for autoimmune
diabetes, are characterized by high levels of circulating T lymphocytes and by resistance ...
Cited by 28 - Related articles - BL Direct - All 5 versions

Role of inherited defects decreasing Fas function in autoimmunity


U Dianzani, A Chiocchetti, U Ramenghi - Life Sciences, 2003 - Elsevier
Fas is a death receptor belonging to the TNFR superfamily and induces cell apoptosis by both
activating a caspase cascade and altering mitochondria. In the immune system, Fas is involved
in the switching-off of the immune responses and cell mediated cytotoxicity. In humans, ...
Cited by 23 - Related articles - All 4 versions

Defective lymphocyte caspase-3 expression in type 1 diabetes mellitus

- eje.org
F Vendrame, C Santangelo, R Misasi, S Dionisi, C … - European journal of …, 2005 - eje.org
Objective: Activation-induced cell death (AICD) is a major mechanism in the regulation of peripheral
tolerance and its impairment can determine the development of autoimmunity. In the present
study, in order to evaluate the role of caspase-3 in type 1 diabetes mellitus (T1DM) AICD, ...
Cited by 17 - Related articles - All 8 versions

Signaling mutations and autoimmunity


CCK Yu, AA Mamchak, AL DeFranco - B cell biology in autoimmunity, 2002 - books.google.com
Nemazee D (ed): B Cell Biology in Autoimmunity. Curr Dir Autoimmun. Basel, Karger, 2003, vol
6, pp 61-8 Signaling Mutations and Autoimmunity Calvin CK Yu, AlushaA. Mamchak, Anthony
L. DeFranco George Williams Hooper Foundation and Department of Microbiology and ...
Cited by 15 - Related articles - BL Direct - All 5 versions

Role of FAS in HIV infection


U Dianzani, T Bensi, A Savarino, S Sametti, M … - Current HIV …, 2003 - ingentaconnect.com
HIV infection is characterized by severe T cell deficiency, with a clinical picture of increased frequency
of opportunistic infections and HIV-associated neoplasms [51]. Primary infection produces an
acute mononucleosis-like illness with high blood virus titers. An immune response then ...
Cited by 14 - Related articles - BL Direct - All 2 versions

Defective function of Fas in T cells from paediatric patients with autoimmune thyroid …


G Bona, S Defranco, A Chiocchetti, M … - Clinical and …, 2003 - pubmedcentral.nih.gov
Triggering of the Fas receptor induces T cell apoptosis and is involved in shutting-off the immune
response. Inherited defects impairing Fas function cause the autoimmune lymphoproliferative
syndrome, and may play a role in other autoimmune diseases. The aim of this work was ...
Cited by 13 - Related articles - BL Direct - All 5 versions

Inherited and acquired death receptor defects in human Autoimmune …


F Rieux-Laucat - Current directions in autoimmunity, 2006 - content.karger.com
Abstract The death receptor Fas/TNFRSF6 is a key player in lymphocyte apoptosis
induction. Patients lacking a functional Fas/TNFRSF6 receptor develop a chronic lymphopro-
liferation termed Autoimmune LymphoProliferative Syndrome (ALPS), characterized by a ...
Cited by 10 - Related articles - BL Direct - All 5 versions


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