S E Straus

Summary

Affiliation: National Institutes of Health
Country: USA

Publications

  1. ncbi request reprint An inherited disorder of lymphocyte apoptosis: the autoimmune lymphoproliferative syndrome
    S E Straus
    Laboratory of Clinical Investigation, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892 1888, USA
    Ann Intern Med 130:591-601. 1999
  2. ncbi request reprint The development of lymphomas in families with autoimmune lymphoproliferative syndrome with germline Fas mutations and defective lymphocyte apoptosis
    S E Straus
    Laboratory of Clinical Investigation, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA
    Blood 98:194-200. 2001
  3. ncbi request reprint Inherited human Caspase 10 mutations underlie defective lymphocyte and dendritic cell apoptosis in autoimmune lymphoproliferative syndrome type II
    J Wang
    Laboratory of Immunology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland 20892, USA
    Cell 98:47-58. 1999
  4. pmc Autoimmune lymphoproliferative syndrome with defective Fas: genotype influences penetrance
    C E Jackson
    Branches of Genetics and Molecular Biology, National Institutes of Health, Bethesda, MD 20892, USA
    Am J Hum Genet 64:1002-14. 1999
  5. ncbi request reprint Clincal, immunologic, and genetic features of an autoimmune lymphoproliferative syndrome associated with abnormal lymphocyte apoptosis
    M C Sneller
    Laboratory of Immunoregulation, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA
    Blood 89:1341-8. 1997
  6. ncbi request reprint Immunophenotypic profiles in families with autoimmune lymphoproliferative syndrome
    J J Bleesing
    Immunology Service, Department of Laboratory Medicine, Clinical Center, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA
    Blood 98:2466-73. 2001
  7. ncbi request reprint TcR-alpha/beta(+) CD4(-)CD8(-) T cells in humans with the autoimmune lymphoproliferative syndrome express a novel CD45 isoform that is analogous to murine B220 and represents a marker of altered O-glycan biosynthesis
    J J Bleesing
    Immunology Service, Warren G Magnuson Clinical Center, Bethesda, Maryland 20892, USA
    Clin Immunol 100:314-24. 2001
  8. pmc A novel lymphoproliferative/autoimmune syndrome resembling murine lpr/gld disease
    M C Sneller
    Laboratory of Clinical Investigation, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland 20892
    J Clin Invest 90:334-41. 1992
  9. ncbi request reprint A genetic disorder of lymphocyte apoptosis involving the fas pathway: the autoimmune lymphoproliferative syndrome
    T A Fleisher
    Department of Laboratory Medicine, Warren G Magnuson Clinical Center and Laboratory of Clinical Investigation, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA
    Curr Allergy Asthma Rep 1:534-40. 2001
  10. pmc Defective CD95/APO-1/Fas signal complex formation in the human autoimmune lymphoproliferative syndrome, type Ia
    D A Martin
    Laboratory of Immunology, National Institutes of Health, Bethesda, MD 20892, USA
    Proc Natl Acad Sci U S A 96:4552-7. 1999

Detail Information

Publications26

  1. ncbi request reprint An inherited disorder of lymphocyte apoptosis: the autoimmune lymphoproliferative syndrome
    S E Straus
    Laboratory of Clinical Investigation, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892 1888, USA
    Ann Intern Med 130:591-601. 1999
    ..Defective apoptosis may also contribute to a heightened risk for lymphoma...
  2. ncbi request reprint The development of lymphomas in families with autoimmune lymphoproliferative syndrome with germline Fas mutations and defective lymphocyte apoptosis
    S E Straus
    Laboratory of Clinical Investigation, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA
    Blood 98:194-200. 2001
    ..These data implicate a role for Fas-mediated apoptosis in preventing B-cell and T-cell lymphomas. Inherited defects in receptor-mediated lymphocyte apoptosis represent a newly appreciated risk factor for lymphomas...
  3. ncbi request reprint Inherited human Caspase 10 mutations underlie defective lymphocyte and dendritic cell apoptosis in autoimmune lymphoproliferative syndrome type II
    J Wang
    Laboratory of Immunology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland 20892, USA
    Cell 98:47-58. 1999
    ..These results provide evidence that inherited nonlethal caspase abnormalities cause pleiotropic apoptosis defects underlying autoimmunity in ALPS type II...
  4. pmc Autoimmune lymphoproliferative syndrome with defective Fas: genotype influences penetrance
    C E Jackson
    Branches of Genetics and Molecular Biology, National Institutes of Health, Bethesda, MD 20892, USA
    Am J Hum Genet 64:1002-14. 1999
    ..Thus, the location of mutations within APT1 strongly influences the development and the severity of ALPS...
  5. ncbi request reprint Clincal, immunologic, and genetic features of an autoimmune lymphoproliferative syndrome associated with abnormal lymphocyte apoptosis
    M C Sneller
    Laboratory of Immunoregulation, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA
    Blood 89:1341-8. 1997
    ..Fas gene mutations account for impaired lymphocyte apoptosis in only a subset of patients with ALPS...
  6. ncbi request reprint Immunophenotypic profiles in families with autoimmune lymphoproliferative syndrome
    J J Bleesing
    Immunology Service, Department of Laboratory Medicine, Clinical Center, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA
    Blood 98:2466-73. 2001
    ....
  7. ncbi request reprint TcR-alpha/beta(+) CD4(-)CD8(-) T cells in humans with the autoimmune lymphoproliferative syndrome express a novel CD45 isoform that is analogous to murine B220 and represents a marker of altered O-glycan biosynthesis
    J J Bleesing
    Immunology Service, Warren G Magnuson Clinical Center, Bethesda, Maryland 20892, USA
    Clin Immunol 100:314-24. 2001
    ....
  8. pmc A novel lymphoproliferative/autoimmune syndrome resembling murine lpr/gld disease
    M C Sneller
    Laboratory of Clinical Investigation, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland 20892
    J Clin Invest 90:334-41. 1992
    ..The clinical and immunological features of this syndrome resemble the lymphoproliferative/autoimmune disease seen in lpr and gld mice...
  9. ncbi request reprint A genetic disorder of lymphocyte apoptosis involving the fas pathway: the autoimmune lymphoproliferative syndrome
    T A Fleisher
    Department of Laboratory Medicine, Warren G Magnuson Clinical Center and Laboratory of Clinical Investigation, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA
    Curr Allergy Asthma Rep 1:534-40. 2001
    ..The clinical features of ALPS reveal the importance of the Fas apoptotic pathway in maintaining lymphocyte homeostasis and protecting against autoimmunity and lymphoid malignancy...
  10. pmc Defective CD95/APO-1/Fas signal complex formation in the human autoimmune lymphoproliferative syndrome, type Ia
    D A Martin
    Laboratory of Immunology, National Institutes of Health, Bethesda, MD 20892, USA
    Proc Natl Acad Sci U S A 96:4552-7. 1999
    ..These data suggest that intracytoplasmic CD95 mutations in ALPS impair apoptosis chiefly by disrupting death-domain interactions with the signaling protein FADD/MORT1...
  11. ncbi request reprint Autoimmune lymphoproliferative syndrome: a syndrome associated with inherited genetic defects that impair lymphocytic apoptosis--CT and US features
    N A Avila
    Diagnostic Radiology Department, Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, MD 20892 1182, USA
    Radiology 212:257-63. 1999
    ..To describe the imaging findings in patients with autoimmune lymphoproliferative syndrome (ALPS) and to relate the findings to the clinical and genetic features of this recently recognized syndrome...
  12. pmc Mutations in the 5' end of the herpes simplex virus type 2 latency-associated transcript (LAT) promoter affect LAT expression in vivo but not the rate of spontaneous reactivation of genital herpes
    K Wang
    Laboratory of Clinical Investigation, National Institute of Allergy and Infectious Diseases, Bethesda, Maryland 20892, USA
    J Virol 71:7903-10. 1997
    ..Far greater reductions in LAT expression are necessary before reactivation rates are noticeably changed...
  13. ncbi request reprint RBC autoantibodies in autoimmune lymphoproliferative syndrome
    D F Stroncek
    Department of Transfusion Medicine, Warren G Magnuson Clinical Center, National Institute of Allergy and Infectious Diseases NIAID, The National Institutes of Health, Bethesda, Maryland 20892 1184, USA
    Transfusion 41:18-23. 2001
    ....
  14. ncbi request reprint Dominant interfering Fas gene mutations impair apoptosis in a human autoimmune lymphoproliferative syndrome
    G H Fisher
    Laboratory of Immunology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland 20892 4470, USA
    Cell 81:935-46. 1995
    ..The occurrence of Fas mutations together with abnormal T cell apoptosis in ALPS patients suggests an involvement of Fas in this recently recognized disorder of lymphocyte homeostasis and peripheral self-tolerance...
  15. ncbi request reprint Neutrophil and platelet antibodies in autoimmune lymphoproliferative syndrome
    S W Kwon
    Department of Transfusion Medicine, Warren G Magnuson Clinical Center, National Institutes of Health, Bethesda, Maryland 20892 1184, USA
    Vox Sang 85:307-12. 2003
    ..We investigated the incidence and nature of neutrophil and platelet antibodies in patients with ALPS...
  16. pmc The 2.2-kilobase latency-associated transcript of herpes simplex virus type 2 does not modulate viral replication, reactivation, or establishment of latency in transgenic mice
    K Wang
    Medical Virology Section, Laboratory of Clinical Investigation, National Institute of Allergy and Infectious Diseases, Bethesda, Maryland 20892, USA
    J Virol 75:8166-72. 2001
    ..These results imply that the phenotype of reduced reactivation associated with the LAT(-) mutant is related to a function encoded in the LAT promoter but not to the major LAT itself...
  17. ncbi request reprint Viral gene expression in rat trigeminal ganglia following neonatal infection with varicella-zoster virus
    P A Brunell
    Ahmanson Pediatrics Center, Cedars Sinai Medical Center, and University of California School of Medicine, Los Angeles, USA
    J Med Virol 58:286-90. 1999
    ..The neonatal rat may represent a useful new model for the study of VZV latency...
  18. ncbi request reprint Increases in circulating and lymphoid tissue interleukin-10 in autoimmune lymphoproliferative syndrome are associated with disease expression
    U Lopatin
    Laboratory of Clinical Investigation, Clinical Research Training Program, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland, USA
    Blood 97:3161-70. 2001
    ..Nonetheless, in vitro studies showed no influence of IL-10 on the survival of CD4(-)CD8(-) T cells. Overexpression of IL-10 in patients with inherited apoptotic defects is strongly associated with the overt manifestations of ALPS...
  19. pmc Specificity and affinity of binding of herpes simplex virus type 2 glycoprotein B to glycosaminoglycans
    R K Williams
    Medical Virology Section, National Institute of Allergy and Infectious Diseases, Bethesda, Maryland 20892, USA
    J Virol 71:1375-80. 1997
    ..The affinity and specificity of gB2 binding to glycosaminoglycans demonstrated in these studies support its role in the initial binding of HSV-2 to cells bearing heparan sulfate or dermatan sulfate glycosaminoglycans...
  20. ncbi request reprint Self-reported sensitivity to chemical exposures in five clinical populations and healthy controls
    S S Nawab
    Section on Biological Rhythms, National Institute of Mental Health, Bethesda, MD 20892 1390, USA
    Psychiatry Res 95:67-74. 2000
    ..A possible relationship between reported chemical sensitivity and hypothalamic-pituitary-adrenal (HPA)-axis functioning is discussed...
  21. ncbi request reprint Combination therapy with famciclovir and interferon-alpha for the treatment of chronic hepatitis B
    A R Marques
    Laboratory of Clinical Investigation, National Institute of Allergy andInfectious disease, National Institutes of Health, Bethesda, MD, USA
    J Infect Dis 178:1483-7. 1998
    ..The combination of famciclovir and IFN-alpha appeared to be at least additive in suppressing HBV DNA. Efficacy trials of combination therapy with famciclovir and IFN-alpha are warranted...
  22. ncbi request reprint Using an interleukin-6 challenge to evaluate neuropsychological performance in chronic fatigue syndrome
    M C Arnold
    National Institute of Neurological Disorders and Stroke, National Institute of Child Health and Human Development and National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland 20892 1440, USA
    Psychol Med 32:1075-89. 2002
    ..To induce influenza-like symptoms, we administered interleukin-6 (IL-6), a cytokine that temporarily activates the acute phase immunological and endocrine responses...
  23. ncbi request reprint Transcript mapping and transregulatory behavior of varicella-zoster virus gene 21, a latency-associated gene
    D Xia
    Laboratory of Clinical Investigation, National Institutes of Health, Bethesda, Maryland, 20892, USA
    Virology 258:304-13. 1999
    ..In transient expression assays, the ORF 21 showed no significant transregulatory activity on promoters of diverse kinetic classes. The ORF 21 promoter, however, was transactivated strongly by VZV infection or by ORF 62...
  24. ncbi request reprint Association of major histocompatibility complex determinants with the development of symptomatic and asymptomatic genital herpes simplex virus type 2 infections
    J A Lekstrom-Himes
    Medical Virology Section, Laboratory of Clinical Investigation, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA
    J Infect Dis 179:1077-85. 1999
    ..Also, HLA-Cw4 was significantly associated with HSV-2 infection. These associations indicate that immunologic factors linked to the MHC influence the risk of HSV-2 infection and disease expression...
  25. pmc Lack of interleukin-6 (IL-6) enhances susceptibility to infection but does not alter latency or reactivation of herpes simplex virus type 1 in IL-6 knockout mice
    R A LeBlanc
    Medical Virology Section, Laboratory of Clinical Investigation, National Institutes of Health, Bethesda, Maryland 20892 1888, USA
    J Virol 73:8145-51. 1999
    ..These studies indicate that while IL-6 plays a role in the protection of mice from lethal HSV infection, it does not substantively influence HSV replication, spread to the nervous system, establishment of latency, or reactivation...
  26. ncbi request reprint The clinical spectrum in a large kindred with autoimmune lymphoproliferative syndrome caused by a Fas mutation that impairs lymphocyte apoptosis
    A J Infante
    Department of Pediatrics, University of Texas Health Science Center at San Antonio 78284 7810, USA
    J Pediatr 133:629-33. 1998
    ....