KIM ERIKA NICHOLS
Affiliation: The Children's Hospital of Philadelphia
- X-linked lymphoproliferative disease: genetics and biochemistryK E Nichols
Department of Pediatric Oncology, Children s Hospital of Philadelphia, PA 19104, USA
Rev Immunogenet 2:256-66. 2000..Future studies exploring the function of DSHP during lymphocyte proliferation and activation should improve our ability to diagnose and treat XLP and possibly other human diseases associated with EBV...
- Macrophage activation and Fcgamma receptor-mediated signaling do not require expression of the SLP-76 and SLP-65 adaptorsKim E Nichols
Pediatric Oncology, Children s Hospital of Philadelphia, PA 19104, USA
J Leukoc Biol 75:541-52. 2004..Taken together, these data suggest that neither SLP-76 nor SLP-65 is required during FcgammaR-dependent signaling and functional events in macrophages...
- Molecular and cellular pathogenesis of X-linked lymphoproliferative diseaseKim E Nichols
Pediatric Oncology, Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
Immunol Rev 203:180-99. 2005..Further studies of SAP and the SLAM family receptors will provide insights into XLP and elucidate the signaling events regulating lymphocyte ontogeny and function...
- Loss of SLP-76 expression within myeloid cells confers resistance to neutrophil-mediated tissue damage while maintaining effective bacterial killingRegina A Clemens
Abramson Family Cancer Research Institute, University of Pennsylvania School of Medicine, 421 Curie Boulevard, Philadelphia, PA 19104, USA
J Immunol 178:4606-14. 2007..aureus but reduction in LPS-induced tissue damage and vascular injury...
- Regulation of cellular and humoral immune responses by the SLAM and SAP families of moleculesCindy S Ma
Garvan Institute of Medical Research, Darlinghurst, 2010, New South Wales, Australia
Annu Rev Immunol 25:337-79. 2007....
- Complementation in trans of altered thymocyte development in mice expressing mutant forms of the adaptor molecule SLP76Martha S Jordan
Cancer Biology, Abramson Family Cancer Research Institute, 427 BRBII III, University of Pennsylvania School of Medicine, Philadelphia, PA, 19104, USA
Immunity 28:359-69. 2008..Thus, the N-terminal tyrosines of SLP76 are required for thymocyte selection but can function on separate molecules to support TCR signaling...
- Impaired SLAM-SLAM homotypic interaction between invariant NKT cells and dendritic cells affects differentiation of IL-4/IL-10-secreting NKT2 cells in nonobese diabetic miceDenis V Baev
Experimental Diabetes Unit, San Raffaele Scientific Institute, Milan, Italy
J Immunol 181:869-77. 2008..Our data suggest that the genetic Slamf1 defect in NOD mice also affects SLAM expression on other immune cells such as the mDC, thus critically impairing the peripheral differentiation of iNKT cells toward a regulatory NKT2 type...
- Impaired humoral immunity in X-linked lymphoproliferative disease is associated with defective IL-10 production by CD4+ T cellsCindy S Ma
Centenary Institute of Cancer Medicine and Cell Biology, Newtown, New South Wales, Australia
J Clin Invest 115:1049-59. 2005..Thus, insufficient IL-10 production may contribute to hypogammaglobulinemia in XLP. This finding suggests new strategies for treating this immunodeficiency...
- Differential requirement for the SAP-Fyn interaction during NK T cell development and functionSelene Nunez-Cruz
Division of Oncology, Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
J Immunol 181:2311-20. 2008..Thus, SAP appears to use differential signaling mechanisms in NKT cells, with optimal ontogeny requiring Fyn binding, while functional responses occur independently of this interaction...
- Missense mutations in SH2D1A identified in patients with X-linked lymphoproliferative disease differentially affect the expression and function of SAPNathan J Hare
Lymphocyte Differentiation, Centenary Institute for Cancer Medicine and Cell Biology, Locked Bag No 6, Newtown, New South Wales 2042, Australia
Int Immunol 18:1055-65. 2006....
- SAP regulates T(H)2 differentiation and PKC-theta-mediated activation of NF-kappaB1Jennifer L Cannons
National Human Genome Research Institute, National Institutes of Health, Bethesda, MD 20892, USA
Immunity 21:693-706. 2004..SLAM engagement amplified PKC-theta recruitment in wt but not SAP- or Fyn-deficient cells, arguing that a SAP/Fyn-mediated pathway enhances PKC-theta/NF-kappaB1 activation and suggesting a role for this pathway in T(H)2 regulation...
- The adaptor protein SH2D1A regulates signaling through CD150 (SLAM) in B cellsSvitlana V Mikhalap
Signal Transduction Laboratory, R E Kavetsky Institute of Experimental Pathology, Oncology and Radiobiology National Academy of Sciences of Ukraine, 45 Vasylkivska St, Kiev 03022, Ukraine
Blood 104:4063-70. 2004..Our data support the hypothesis that CD150 and SH2D1A are coexpressed during a narrow window of B-cell maturation and SH2D1A may be involved in regulation of B-cell differentiation via switching of CD150-mediated signaling pathways...
- Summary of the 12th Nikolas Symposium dendritic cell differentiation: signals, signaling and functional consequences as clues to possible therapyKim E Nichols
Division of Pediatric Oncology, Children s of Hospital Philadelphia, Philadelphia, Pensilvania 19104, USA
J Pediatr Hematol Oncol 25:193-7. 2003
- Functional requirements for interactions between CD84 and Src homology 2 domain-containing proteins and their contribution to human T cell activationStuart G Tangye
Centenary Institute for Cancer Medicine and Cell Biology, Newtown 2042, Sydney, New South Wales, Australia
J Immunol 171:2485-95. 2003..These results reveal a novel function of CD84 on human lymphocytes and suggest that CD84 can activate human T cells via a SAP-independent mechanism...
- Treatment of primary Epstein-Barr virus infection in patients with X-linked lymphoproliferative disease using B-cell-directed therapyMichael C Milone
Department of Pathology and Laboratory Medicine, Hematologic Malignancies Program, University of Pennsylvania, Philadelphia, USA
Blood 105:994-6. 2005..These data suggest that the pre-emptive use of B-cell-directed therapy may reduce the morbidity and mortality of primary EBV infection in XLP-affected individuals...
- Coincident expression of the chemokine receptors CCR6 and CCR7 by pathologic Langerhans cells in Langerhans cell histiocytosisMark D Fleming
Department of Medical Oncology, Dana Farber Cancer Institute, Harvard Medical School, Boston, MA 02115, USA
Blood 101:2473-5. 2003..Histiocytes in Rosai-Dorfman disease and hemophagocytic syndrome also coexpressed CCR6 and CCR7, suggesting that this may be a general attribute of abnormal histiocytes...
- Enhanced T cell responses due to diacylglycerol kinase zeta deficiencyXiao Ping Zhong
The Signal Transduction Program, The Abramson Family Cancer Research Institute, Philadelphia, Pennsylvania 19104, USA
Nat Immunol 4:882-90. 2003..These results demonstrate the importance of DGKzeta as a physiological negative regulator of TCR signaling and T cell activation...
- Diacylglycerol kinase zeta regulates microbial recognition and host resistance to Toxoplasma gondiiCheng hu Liu
Department of Pediatrics Allergy and Immunology, Duke University Medical Center, Durham, NC 27710, USA
J Exp Med 204:781-92. 2007....
- Loss of circulating CD27+ memory B cells and CCR4+ T cells occurring in association with elevated EBV loads in XLP patients surviving primary EBV infectionAlejandro Malbran
Departamento de Alergia e Inmunologia, Hospital Britanico, Buenos Aires, Argentina
Blood 103:1625-31. 2004..Despite an expanded number of CD3+CD8+ lymphocytes, increased EBV loads occurred in both patients without overt clinical symptoms of mononucleosis, lymphoproliferative disease, or lymphoma...
- X-linked lymphoproliferative syndrome: an X-cellent questionAlexandra Filipovich
Cincinnati Children s Hospital Medical Center, OH, USA
Clin Immunol 119:241-4. 2006
- Selective generation of functional somatically mutated IgM+CD27+, but not Ig isotype-switched, memory B cells in X-linked lymphoproliferative diseaseCindy S Ma
Lymphocyte Differentiation Laboratory, Centenary Institute of Cancer Medicine and Cell Biology, Newtown, New South Wales, Australia
J Clin Invest 116:322-33. 2006..Production of affinity-matured IgM by IgMCD27 B cells may protect against pathogens to which a normal immune response is elicited in XLP patients...
- Expansion of functionally immature transitional B cells is associated with human-immunodeficient states characterized by impaired humoral immunityAmanda K Cuss
Centenary Institute of Cancer Medicine and Cell Biology, New South Wales, Australia
J Immunol 176:1506-16. 2006..Further investigation of transitional B cells will improve our understanding of human B cell development and how alterations to this process may precipitate immunodeficiency or autoimmunity...
- SHP-1 and SHP-2 associate with immunoreceptor tyrosine-based switch motif of programmed death 1 upon primary human T cell stimulation, but only receptor ligation prevents T cell activationJens M Chemnitz
Abramson Family Cancer Research Institute and Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
J Immunol 173:945-54. 2004..Despite this interaction, the ability of PD-1 to block T cell activation required receptor ligation, suggesting that colocalization of PD-1 with CD3 and/or CD28 may be necessary for inhibition of T cell activation...
- Regulation of NKT cell development by SAP, the protein defective in XLPKim E Nichols
Pediatric Oncology, Wood, 4th Floor, 3615 Civic Center Boulevard, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA
Nat Med 11:340-5. 2005..Thus, SAP is a crucial regulator of NKT cell ontogeny in humans and in mice. The absence of NKT cells may contribute to the phenotypes of SAP deficiency, including abnormal antiviral and antitumor immunity and hypogammaglobulinemia...
- Role of SAP,SLAM and Fyn in NKT Cell Ontogeny and ActivationKIM ERIKA NICHOLS; Fiscal Year: 2010....
- Role of SAP,SLAM and Fyn in NKT Cell Ontogeny and ActivationKim Nichols; Fiscal Year: 2007....