- LAP2zeta binds BAF and suppresses LAP2beta-mediated transcriptional repression
Sheba Cancer Research Center and the Institute of Hematology, Chaim Sheba Medical Center, Tel Hashomer, 52621, Israel
Eur J Cell Biol 87:267-78. 2008
..LAP2zeta may function by decreasing availability of BAF. These findings could have implications in the study of nuclear lamina-associated diseases and BAF-dependent retroviral integration...
- The kinetics of early T and B cell immune recovery after bone marrow transplantation in RAG-2-deficient SCID patients
Cancer Research Center, Tel Hashomer, Israel
PLoS ONE 7:e30494. 2012
- Purine nucleoside phosphorylase deficiency presenting as severe combined immune deficiency
Cancer Research Center, Pediatric Department, Pediatric Immunology Service, Jeffery Modell Foundation JMF Center, Edmond and Lily Safra Children s Hospital, Tel Aviv University, Tel Aviv, Israel
Immunol Res 56:150-4. 2013
..We suggest that the aberrant slice site was used preferentially over the normal slice site in some cells correlating with the severity of disease...
- Enhanced expression of the nuclear envelope LAP2 transcriptional repressors in normal and malignant activated lymphocytes
Sheba Cancer Research Center, Institute of Hematology, Chaim Sheba Medical Center, Tel Hashomer, Israel
Ann Hematol 86:393-401. 2007
..Based on our presented results, we believe that the LAP2-HDAC regulatory pathway should be studied as a new target for rational therapy...
- Pseudotumor cerebri after allogeneic bone marrow transplant associated with cyclosporine a use for graft-versus-host disease prophylaxis
Divisions of Immunology Allergy, The Hospital for Sick Children and the University of Toronto, 555 University Avenue, Toronto, Ontario, Canada
J Pediatr Hematol Oncol 29:66-8. 2007
..Physicians who are following patients on CsA need to be alert to the possibility of PTC. Prompt diagnosis followed by thorough evaluation and treatment are crucial for preventing visual loss and improving associated symptoms...
- Histone deacetylase inhibitors--a new tool to treat cancer
Laboratory of Molecular Hemato Oncology, Sheba Cancer Research Center, Chaim Sheba Medical Center, Tel Hashomer, Sackler School of Medicine, Tel Aviv University, Israel
Cancer Treat Rev 30:461-72. 2004
..Here, we will describe the structure of the nucleosome, review regulation of gene expression by acetylation of histones and give an update on the current phase I and phase II clinical trials with histone deacetylase inhibitors...
- Reduced central tolerance in Omenn syndrome leads to immature self-reactive oligoclonal T cells
Cancer Research Center, Safra Children s Hospital, Sheba Medical Center, Tel Hashomer and Sackler School of Medicine, Tel Aviv, Israel
J Allergy Clin Immunol 124:793-800. 2009
..Dysregulations of the central and peripheral immune tolerance, mediated by the protein autoimmune regulator (AIRE) and regulatory T cells, respectively, were proposed as possible mechanisms of this aberrant inflammatory process...
- Leptin and C-reactive protein levels correlate during minor infection in children
Department of Pediatrics, Dana Children s Hospital, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel
Isr Med Assoc J 9:76-8. 2007
..Leptin, a pleiotropic hormone, has been suggested to be part of an acute-phase response during an inflammatory stimulus. Its correlation with other acute-phase reactants during minor infection in children has not been investigated...
- Characterizing T cells in SCID patients presenting with reactive or residual T lymphocytes
Chaim Sheba Medical Center, Sackler Faculty of Medicine, Tel Aviv University, 69978 Tel Aviv, Israel
Clin Dev Immunol 2012:261470. 2012
..While all cells are functionally deficient, resulting in high susceptibility to infections, only some of these cells are causing autoimmune symptoms...
- Thymic function in MHC class II-deficient patients
Cancer Research Center, Jeffery Modell Foundation Center, Edmond and Lily Safra Children s Hospital, Sheba Medical Center, Tel Hashomer, Affiliated to Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
J Allergy Clin Immunol 131:831-9. 2013
..MHC class II (MHC-II) molecules play a pivotal role in the development, activation, and homeostasis of CD4(+) TH cells in the thymus. The absence of MHC-II molecules causes severe T-cell immunodeficiency...
- The effect of gentamicin-induced readthrough on a novel premature termination codon of CD18 leukocyte adhesion deficiency patients
Amos J Simon
Cancer Research Center, Chaim Sheba Medical Center, Tel Hashomer, Israel
PLoS ONE 5:e13659. 2010
..In other genes containing such mutations, treatments with aminoglycoside types of antibiotics (e.g., gentamicin) were reported to partially correct the premature protein termination, by induction of readthrough mechanism...
- Newborn screening for severe T and B cell immunodeficiency in Israel: a pilot study
Cancer Research Center, Safra Children s Hospital, Sheba Medical Center, Tel Hashomer, Israel
Isr Med Assoc J 15:404-9. 2013
..Enumeration of kappa-deleting recombination excision circle (KREC) copy numbers can be similarly used for early assessment of B cell lymphopenia...
- Defining combined immunodeficiency
Chaim M Roifman
Division of Immunology and Allergy, Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada
J Allergy Clin Immunol 130:177-83. 2012
..Therefore it is important to define the numbers of autologous cells usually detected in patients with CID versus those with SCID...
- Molecular assessment of thymic capacities in patients with Schimke immuno-osseous dysplasia
Cancer Research Center, Sheba Medical Center, Tel Hashomer and Sackler School of Medicine, Tel Aviv, Israel
Clin Immunol 133:375-81. 2009
..High thymic expression of SMARCAL1 mRNA raises the possibility of its importance in thymus maintenance and function...
- The role of hematopoietic stem cell transplantation in SP110 associated veno-occlusive disease with immunodeficiency syndrome
Department of Pediatrics, Hadassah University Hospital, Jerusalem, Israel
Pediatr Allergy Immunol 24:250-6. 2013
..In this study, we describe a new group of patients with VODI highlighting further clinical and immunologic aspects of this disease and re-evaluating the effectiveness of HSCT for the treatment of this disorder...
- Thymic functions and gene expression profile distinct double-negative cells from single positive cells in the autoimmune lymphoproliferative syndrome
Cancer Research Center, Sheba Medical Center, Tel Hashomer affiliated to the Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
Autoimmun Rev 11:723-30. 2012
..The origin and function of the double-negative cells are incompletely understood. Here we studied the origin of DN T cells found in patients with autoimmune lymphoproliferative syndrome...
- Nuclear envelopathies--raising the nuclear veil
Sheba Cancer Research Center, Institute of Hematology, The Chaim Sheba Medical Center and Sackler School of Medicine, Tel Aviv University, Israel
Pediatr Res 57:8R-15R. 2005
..In this review, we describe recent discoveries in the field of nuclear envelopathies and discuss current proposed pathophysiological mechanisms underlying these diseases...
- Molecular assessment of thymus capabilities in the evaluation of T-cell immunodeficiency
Cancer Research Center, Safra Children s Hospital, Chaim Sheba Medical Center, Tel Hashomer 52621, Israel
Pediatr Res 67:211-6. 2010
..Assessment of thymus capabilities by TRECs and TCR repertoire analyses is helpful in diagnosing patients with T-cell immunodeficiency and should be part of the evaluation of every patient suspected of having that condition...
- Characterization of ζ-associated protein, 70 kd (ZAP70)-deficient human lymphocytes
Chaim M Roifman
Division of Immunology and Allergy, Canadian Centre for Primary Immunodeficiency, Jeffrey Modell Research Laboratory for the diagnosis of Primary Immunodeficiency, Hospital for Sick Children, Toronto, Ontario, Canada
J Allergy Clin Immunol 126:1226-33.e1. 2010
..Patients who live beyond infancy can also experience autoimmune manifestations...
- Disturbed B and T cell homeostasis and neogenesis in patients with ataxia telangiectasia
Pediatric Immunology Unit, Jeffrey Modell Foundation JMF Center and Department of Pediatrics, Sheba Medical Center, Tel Hashomer, Israel
J Clin Immunol 34:561-72. 2014
..We examined immunologic parameters reflecting cell development and proliferation and their relevancy to the clinical phenotype in affected individuals...
- Mutation analysis should be performed to rule out gammac deficiency in children with functional severe combined immune deficiency despite apparently normal immunologic tests
Division of Immunology Allergy and the Infection, Immunity, Injury and Repair Program, The Research Institute and The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
J Pediatr 147:555-7. 2005
..Both had infections suggestive of immunodeficiency, but their immune function and lymphoid tissues were normal. They were found to have an identical R222C mutation in the gammac gene...
- Fatal lung fibrosis associated with immunodeficiency and gonadal dysgenesis in 46XX sisters--a new syndrome
Department of Pediatrics, The Canadian Centre for Primary Immunodeficiency, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
Am J Med Genet A 146:8-14. 2008
..The distinct findings in these two sisters have not been reported before and thus suggest a hitherto unknown autosomal recessive condition that includes immune dysfunction, gonadal dysgenesis, and pulmonary fibrosis...
- Severe eosinophilia in children: a diagnostic dilemma
Pediatric Department B North, Pediatric Immunology Service, Chaim Sheba Medical Center, Tel Hashomer, Israel
J Pediatr Hematol Oncol 35:303-6. 2013
..Advanced molecular assays to detect clonal expansion should be used to exclude aberrant clonal processes in such patients...
- T-cell compartment in synovial fluid of pediatric patients with JIA correlates with disease phenotype
Cancer Research Center, Sheba Medical Center, Tel Hashomer and Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel
J Clin Immunol 31:1021-8. 2011
..Oligoarthritis, which is considered a pauciarticular subtype since it involves up to four joints upon presentation, is further divided into persistent or extended forms based on disease progression...
- Novel SMARCAL1 bi-allelic mutations associated with a chromosomal breakage phenotype in a severe SIOD patient
Amos J Simon
Cancer Research Center, Chaim Sheba Medical Center, Tel Hashomer, 52621, Israel
J Clin Immunol 34:76-83. 2014
..We studied a patient with profound T-lymphocyte defect, neurodevelopmental delay, facial dysmorphism, nephrotic syndrome and spondyloepiphyseal bone dysplasia typical of SIOD...
- Hospital versus home treatment of respiratory exacerbations in cystic fibrosis
Pediatric Pulmonary Unit and the National Center for Cystic Fibrosis, Edmond and Lily Safra Children Hospital, Sheba Medical Center, Tel Hashomer, Israel
Med Sci Monit 17:CR698-703. 2011
..Treatment of respiratory exacerbations in Cystic Fibrosis (CF) is important in slowing disease progression. The treatment may be given either at home or at the hospital. The aim of our study was to compare both treatment settings...
- T-cell receptor excision circles in primary immunodeficiencies and other T-cell immune disorders
Pediatric Department B, Edmond and Lily Safra Children s Hospital, Sheba Medical Center, Tel Hashomer, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
Curr Opin Allergy Clin Immunol 11:517-24. 2011
..Their enumeration in the latest circle created during TCR delta deletion and the final TCR alpha rearrangement can determine thymus output...
- Microarray-based gene expression profiling of hematologic malignancies: basic concepts and clinical applications
Pediatric Hematology Oncology, Safra Children s Hospital, The Chaim Sheba Medical Center, Tel Hashomer and Sackler School of Medicine, Tel Aviv University, Tel Aviv 69978, Israel
Blood Rev 19:223-34. 2005
- Matrix metalloproteinases 2 and 9 are markers of inflammation but not of the degree of fibrosis in chronic hepatitis C
Department of Pediatrics, Tel Aviv Souraski Medical Center, Tel Aviv, Israel
Digestion 71:124-30. 2005
..Controversy still exists regarding the role of matrix metalloproteinases (MMPs) as valid markers of liver fibrosis...
- Epidemiologic, socioeconomic, and clinical factors associated with severity of respiratory syncytial virus infection in previously healthy infants
Department of Pediatrics, Dana Children s Hospital, Tel Aviv Sourasky Medical Center, Tel Aviv University, Tel Aviv, Israel
Clin Pediatr (Phila) 45:621-7. 2006
..Our results emphasize the complexity of predicting disease severity in previously healthy infants with RSV infection and suggest that other parameters such as host genetic background might explain the clinical variability...
- Neurologic presentation in children with ataxia-telangiectasia: is small head circumference a hallmark of the disease?
National A T Clinic, Edmond and Lilly Safra Children s Hospital, Sheba Medical Center, Ramat Gan, Israel
J Pediatr 159:466-471.e1. 2011
..To define the neurologic characteristics and course of ataxia-telangiectasia (A-T)...
- Genetic predisposition to infectious pathogens: a review of less familiar variants
Pediatric Hemato Oncology, Chaim Sheba Medical Center, Tel Hashomer, Israel
Pediatr Infect Dis J 22:457-61. 2003
..We review some of the less common variants that influence the host defense and predispose to certain infectious agents or change their outcome...
- Impact of conditioning on outcome of hematopoietic stem cell transplantation for wiskott-Aldrich syndrome
Department of Pediatric Hematology Oncology, Hadassah Hebrew University Medical Center, Jerusalem, Israel
J Pediatr Hematol Oncol 35:e234-8. 2013
..Further studies are required to determine the best conditioning regimen and optimal donor source for children with WAS. ..
- Uncommon presentation of some common pediatric diseases
Department of Pediatrics, Dana Children s Hospital, Tel Aviv Sourasky Medical Center affiliated with the Sackler Faculty of Medicine, Tel Aviv University, Israel
Adv Pediatr 50:269-304. 2003
- The nuclear-envelope protein and transcriptional repressor LAP2beta interacts with HDAC3 at the nuclear periphery, and induces histone H4 deacetylation
Sheba Cancer Research Center and Institute of Hematology, The Chaim Sheba Medical Center, Tel Hashomer 52621, Israel
J Cell Sci 118:4017-25. 2005
..Our data provide evidence for the existence of a previously unknown repressive complex, composed of an integral nuclear membrane protein and a histone modifier, at the nuclear periphery...
- Cellular and humoral aberrations in a kindred with IL-1 receptor-associated kinase 4 deficiency
J Allergy Clin Immunol 120:948-50. 2007