Raffaele Badolato

Summary

Affiliation: University of Brescia
Country: Italy

Publications

  1. pmc Type I interferon-dependent gene MxA in perinatal HIV-infected patients under antiretroviral therapy as marker for therapy failure and blood plasmacytoid dendritic cells depletion
    Raffaele Badolato
    Istituto di Medicina Molecolare Angelo Nocivelli, Department of Pediatrics, University of Brescia, Brescia 25123, Italy
    J Transl Med 6:49. 2008
  2. ncbi Hyper immunoglobulin M syndrome due to CD40 deficiency: clinical, molecular, and immunological features
    Vassilios Lougaris
    Department of Pediatrics, Istituto di Medicina Molecolare A Nocivelli, University of Brescia, Brescia, Italy
    Immunol Rev 203:48-66. 2005
  3. ncbi Innate immunity defects in Hermansky-Pudlak type 2 syndrome
    Stefania Fontana
    Istituto di Medicina Molecolare Angelo Nocivelli, Universita di Brescia, c o Spedali Civili, 25123 Brescia, Italy
    Blood 107:4857-64. 2006
  4. ncbi Virologic, immunologic, and clinical benefits from early combined antiretroviral therapy in infants with perinatal HIV-1 infection
    Elena Chiappini
    Department of Paediatrics, University of Florence, Florence, Italy
    AIDS 20:207-15. 2006
  5. ncbi Novel insights from adaptor protein 3 complex deficiency
    Raffaele Badolato
    Istituto di Medicina Molecolare Angelo Nocivelli and Clinica Pediatrica and Dipartimento di Scienze Biomediche e Biotecnologie, University of Brescia, Brescia, Italy
    J Allergy Clin Immunol 120:735-41; quiz 742-3. 2007
  6. pmc Occurrence of nodular lymphocyte-predominant hodgkin lymphoma in hermansky-pudlak type 2 syndrome is associated to natural killer and natural killer T cell defects
    Luisa Lorenzi
    Department of Molecular and Translational Medicine, Section of Pathology, University of Brescia, Brescia, Italy
    PLoS ONE 8:e80131. 2013
  7. pmc PTX3 genetic variations affect the risk of Pseudomonas aeruginosa airway colonization in cystic fibrosis patients
    M Chiarini
    Clinica Pediatrica, Istituto di Medicina Molecolare Angelo Nocivelli, Universita di Brescia, Brescia, Italy
    Genes Immun 11:665-70. 2010
  8. pmc Clinical, laboratory and molecular signs of immunodeficiency in patients with partial oculo-cutaneous albinism
    Laura Dotta
    Department of Experimental and Clinical Sciences, Institute of Molecular Medicine Angelo Nocivelli, University of Brescia, Brescia, Italy
    Orphanet J Rare Dis 8:168. 2013
  9. doi Defects of leukocyte migration in primary immunodeficiencies
    Raffaele Badolato
    Department of Pediatrics, Institute of Molecular Medicine Angelo Nocivelli, University of Brescia, Brescia, Italy
    Eur J Immunol 43:1436-40. 2013
  10. ncbi From bone marrow transplantation to cellular therapies: possible therapeutic strategies in managing autoimmune disorders
    Andrea Taddio
    Institute for Maternal and Child Health IRCCS Burlo Garofolo, Trieste, Italy
    Curr Pharm Des 18:5776-81. 2012

Detail Information

Publications49

  1. pmc Type I interferon-dependent gene MxA in perinatal HIV-infected patients under antiretroviral therapy as marker for therapy failure and blood plasmacytoid dendritic cells depletion
    Raffaele Badolato
    Istituto di Medicina Molecolare Angelo Nocivelli, Department of Pediatrics, University of Brescia, Brescia 25123, Italy
    J Transl Med 6:49. 2008
    ....
  2. ncbi Hyper immunoglobulin M syndrome due to CD40 deficiency: clinical, molecular, and immunological features
    Vassilios Lougaris
    Department of Pediatrics, Istituto di Medicina Molecolare A Nocivelli, University of Brescia, Brescia, Italy
    Immunol Rev 203:48-66. 2005
    ..In this article, we present a comprehensive overview of the clinical, genetic, and immunological features of patients with hyper IgM syndrome due to CD40 mutations...
  3. ncbi Innate immunity defects in Hermansky-Pudlak type 2 syndrome
    Stefania Fontana
    Istituto di Medicina Molecolare Angelo Nocivelli, Universita di Brescia, c o Spedali Civili, 25123 Brescia, Italy
    Blood 107:4857-64. 2006
    ..Taken together, these observations suggest that type 2 Hermansky-Pudlak syndrome is characterized by defects of innate immunity...
  4. ncbi Virologic, immunologic, and clinical benefits from early combined antiretroviral therapy in infants with perinatal HIV-1 infection
    Elena Chiappini
    Department of Paediatrics, University of Florence, Florence, Italy
    AIDS 20:207-15. 2006
    ....
  5. ncbi Novel insights from adaptor protein 3 complex deficiency
    Raffaele Badolato
    Istituto di Medicina Molecolare Angelo Nocivelli and Clinica Pediatrica and Dipartimento di Scienze Biomediche e Biotecnologie, University of Brescia, Brescia, Italy
    J Allergy Clin Immunol 120:735-41; quiz 742-3. 2007
    ..It is likely that the investigation of the physiopathology of Hermansky-Pudlak type 2 syndrome will reveal nonredundant functions of this adaptor protein in the intracellular trafficking of membrane proteins...
  6. pmc Occurrence of nodular lymphocyte-predominant hodgkin lymphoma in hermansky-pudlak type 2 syndrome is associated to natural killer and natural killer T cell defects
    Luisa Lorenzi
    Department of Molecular and Translational Medicine, Section of Pathology, University of Brescia, Brescia, Italy
    PLoS ONE 8:e80131. 2013
    ..In summary, these results suggest that a combined and profound defect of innate and adaptive effector cells might explain the susceptibility to infections and lymphoma in these HPS2 patients. ..
  7. pmc PTX3 genetic variations affect the risk of Pseudomonas aeruginosa airway colonization in cystic fibrosis patients
    M Chiarini
    Clinica Pediatrica, Istituto di Medicina Molecolare Angelo Nocivelli, Universita di Brescia, Brescia, Italy
    Genes Immun 11:665-70. 2010
    ..Moreover, a protective effect was found in association with a specific haplotype (odds ratio 0.524). Our data suggest that variations within PTX3 affect lung colonization of Pseudomonas in patients with CF...
  8. pmc Clinical, laboratory and molecular signs of immunodeficiency in patients with partial oculo-cutaneous albinism
    Laura Dotta
    Department of Experimental and Clinical Sciences, Institute of Molecular Medicine Angelo Nocivelli, University of Brescia, Brescia, Italy
    Orphanet J Rare Dis 8:168. 2013
    ..This review describes the molecular and pathogenetic mechanisms of these diseases, focusing on clinical and biochemical aspects that allow early differential diagnosis...
  9. doi Defects of leukocyte migration in primary immunodeficiencies
    Raffaele Badolato
    Department of Pediatrics, Institute of Molecular Medicine Angelo Nocivelli, University of Brescia, Brescia, Italy
    Eur J Immunol 43:1436-40. 2013
    ....
  10. ncbi From bone marrow transplantation to cellular therapies: possible therapeutic strategies in managing autoimmune disorders
    Andrea Taddio
    Institute for Maternal and Child Health IRCCS Burlo Garofolo, Trieste, Italy
    Curr Pharm Des 18:5776-81. 2012
    ..We herein present the rationale of different strategies, the preliminary data obtained in clinical trials, unsolved problems and possible next developments of novel treatment protocols of autoimmune disorders...
  11. pmc Tetralogy of fallot is an uncommon manifestation of warts, hypogammaglobulinemia, infections, and myelokathexis syndrome
    Raffaele Badolato
    Pediatric Clinic and A Nocivelli Institute of Molecular Medicine, Brescia, Italy
    J Pediatr 161:763-5. 2012
    ..This observation suggests a possible increased risk of TOF in WHIM syndrome and that birth presentation of TOF and neutropenia should lead to suspect WHIM syndrome...
  12. ncbi Congenital neutropenia: advances in diagnosis and treatment
    Raffaele Badolato
    Department of Pediatrics, Angelo Nocivelli Institute for Molecular Medicine, University of Brescia, 25123 Brescia, Italy
    Curr Opin Allergy Clin Immunol 4:513-21. 2004
    ..Moreover, leukemic transformation during treatment is not observed in all patients, but is more typical of severe congenital neutropenia and Shwachman-Diamond patients...
  13. ncbi Leukocyte circulation: one-way or round-trip? Lessons from primary immunodeficiency patients
    Raffaele Badolato
    University of Brescia, c o Spedali Civili, 25123 Brescia, Italy
    J Leukoc Biol 76:1-6. 2004
    ..This paper will be focused on the interpretation of primary immunodeficiencies as defects in leukocyte circulation between blood and primary and secondary organs...
  14. ncbi Toll-like receptor-4 genotype in children with respiratory infections
    R Badolato
    Raffaele Badolato, Department of Pediatrics, University of Brescia, c o Spedali Civili, I 25125 Brescia, Italy
    Allergy 59:1018-9. 2004
  15. doi Defect of plasmacytoid dendritic cells in warts, hypogammaglobulinemia, infections, myelokathexis (WHIM) syndrome patients
    Laura Tassone
    Department of Pediatrics, Institute of Molecular Medicine Angelo Nocivelli, Brescia, Italy
    Blood 116:4870-3. 2010
    ....
  16. doi SH2-domain mutations in STAT3 in hyper-IgE syndrome patients result in impairment of IL-10 function
    Mauro Giacomelli
    Department of Pediatrics, Institute of Molecular Medicine Angelo Nocivelli, University of Brescia, Brescia, Italy
    Eur J Immunol 41:3075-84. 2011
    ..Impairment of IL-10 signaling in patients with AD-HIES might result in an altered balance between pro-inflammatory and anti-inflammatory signals and might lead to persistent inflammation and delayed healing after infections...
  17. ncbi Role of dendritic cell-derived CXCL13 in the pathogenesis of Bartonella henselae B-rich granuloma
    William Vermi
    Department of Pathology and the Section of General Pathology and Immunology, University of Brescia, Italy
    Blood 107:454-62. 2006
    ..These findings suggest that the humoral immune response to B henselae initiates in the extrafollicular areas of infected lymph nodes and is regulated by DCs...
  18. ncbi Functional defects of dendritic cells in patients with CD40 deficiency
    Stefania Fontana
    Clinica Pediatrica, Universita di Brescia, c o Spedali Civili, 25123 Brescia, Italy
    Blood 102:4099-106. 2003
    ..These observations suggest that the severe impairment of DC maturation may contribute to the defect of T-cell-mediated immunity observed in HIGM3 patients...
  19. doi Psychosocial issues in children and adolescents with HIV infection evaluated with a World Health Organization age-specific descriptor system
    Antonietta Giannattasio
    Department of Pediatrics, University Federico II, Naples, Italy
    J Dev Behav Pediatr 32:52-5. 2011
    ..We investigated the efficacy of the ICF to describe the health status and needs of a cohort of children and adolescents with HIV seen at a reference center for pediatric AIDS in Europe...
  20. ncbi Combined decrease of defined B and T cell subsets in a group of common variable immunodeficiency patients
    Daniele Moratto
    Istituto di Medicina Molecolare Angelo Nocivelli, Clinica Pediatrica, Universita di Brescia, Spedali Civili, Piazzale Spedali Civili 1, 25123 Brescia, Italy
    Clin Immunol 121:203-14. 2006
    ..These observations suggest that a combined defect in generation of B and T subpopulations may account for the abnormal immunophenotype characterizing this subgroup of CVID patients...
  21. ncbi Altered leukocyte response to CXCL12 in patients with warts hypogammaglobulinemia, infections, myelokathexis (WHIM) syndrome
    Anna Virginia Gulino
    Clinica Pediatrica, Universita di Brescia, c o Spedali Civili, 25 123 Brescia, Italy
    Blood 104:444-52. 2004
    ....
  22. ncbi Autosomal recessive agammaglobulinemia: the third case of Igβ deficiency due to a novel non-sense mutation
    Vassilios Lougaris
    Department of Clinical and Experimental Sciences, Pediatrics Clinic and Institute for Molecular Medicine A Nocivelli, University of Brescia, Spedali Civili di Brescia, Brescia, Italy
    J Clin Immunol 34:425-7. 2014
    ..This study describes the third case worldwide of autosomal recessive agammaglobulinemia due to a novel non-sense mutation in Igβ presenting with neutropenia, ecthyma and mild respiratory infections. ..
  23. doi Activin A as a mediator of NK-dendritic cell functional interactions
    Pascal Seeger
    Department of Molecular and Translational Medicine, University of Brescia, 25123 Brescia, Italy
    J Immunol 192:1241-8. 2014
    ..In conclusion, our study reports that activin A, produced during NK-DC interactions, represents a relevant negative feedback mechanism that might function to prevent excessive immune activation by DCs. ..
  24. doi Severe impairment of IFN-γ and IFN-α responses in cells of a patient with a novel STAT1 splicing mutation
    Donatella Vairo
    Pediatric Clinic and A Nocivelli Institute of Molecular Medicine, Brescia, Italy
    Blood 118:1806-17. 2011
    ..These results suggested that exon 3 skipping of STAT1 leads to abnormal signaling in response to IFN-γ and IFN-α, which is associated with susceptibility to intracellular pathogens and viruses...
  25. pmc AIRE deficiency in thymus of 2 patients with Omenn syndrome
    Patrizia Cavadini
    1Istituto di Medicina Molecolare Angelo Nocivelli, Clinica Pediatrica, University of Brescia, Brescia, Italy
    J Clin Invest 115:728-32. 2005
    ....
  26. ncbi Defective migration of monocyte-derived dendritic cells in LAD-1 immunodeficiency
    Maurilia Fiorini
    Istituto di Medicina Molecolare Angelo Nocivelli, Universita di Brescia, Italy
    J Leukoc Biol 72:650-6. 2002
    ....
  27. pmc B cell responses to CpG correlate with CXCL16 expression levels in common variable immunodeficiency
    Vassilios Lougaris
    Pediatrics Clinic and Institute of Molecular Medicine A Nocivelli, University of Brescia, Piazzale Spedali Civili 1, 25123 Brescia, Italy
    ScientificWorldJournal 2012:960219. 2012
    ..Our data suggest that expression levels of a surface receptor, namely, CXCL16, correlate with B cell responses mediated by TLR9 in common variable immunodeficiency...
  28. ncbi Monocytes from Wiskott-Aldrich patients display reduced chemotaxis and lack of cell polarization in response to monocyte chemoattractant protein-1 and formyl-methionyl-leucyl-phenylalanine
    R Badolato
    Clinica Pediatrica, Universita di Brescia, Italy
    J Immunol 161:1026-33. 1998
    ..These results suggest that WAS protein is involved in the monocyte response to the chemokines MCP-1 and macrophage inflammatory protein-1alpha...
  29. ncbi Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy syndrome: time to review diagnostic criteria?
    F Buzi
    Pediatric Department and Institute of Molecular Medicine A Nocivelli, University of Brescia, 25123 Brescia, Italy
    J Clin Endocrinol Metab 88:3146-8. 2003
    ....
  30. ncbi Complete genomic organization of the human JAK3 gene and mutation analysis in severe combined immunodeficiency by single-strand conformation polymorphism
    R F Schumacher
    Clinica Pediatrica dell Università degli Studi di Brescia and Istituto di Medicina Molecolare Angelo Nocivelli, Italy
    Hum Genet 106:73-9. 2000
    ..In this cohort of patients, 15 independent JAK3 gene mutations have been identified, including 7 that have not been described previously. Mutation analysis information was used for genetic counseling and prenatal diagnosis...
  31. ncbi Combined immunodeficiencies due to defects in signal transduction: defects of the gammac-JAK3 signaling pathway as a model
    L D Notarangelo
    Istituto di Medicina Molecolare Angelo Nocivelli, Clinica Pediatrica Università di Brescia, Italy
    Immunobiology 202:106-19. 2000
    ....
  32. pmc Expression of inducible nitric oxide synthase in human granulomas and histiocytic reactions
    F Facchetti
    Department of Pathology, University of Brescia, Italy
    Am J Pathol 154:145-52. 1999
    ..Taken together, these results indicate that iNOS is involved in different human immune reactions characterized by histiocytic/granulomatous inflammation and associated with Th1-type cytokine secretion...
  33. ncbi Serum amyloid A is an activator of PMN antimicrobial functions: induction of degranulation, phagocytosis, and enhancement of anti-Candida activity
    R Badolato
    Clinica Pediatrica, Universita di Brescia, Italy
    J Leukoc Biol 67:381-6. 2000
    ....
  34. ncbi Prenatal molecular diagnosis of Wiskott-Aldrich syndrome by direct mutation analysis
    S Giliani
    Institute of Chemistry, Department of Paediatrics, University of Brescia, Italy
    Prenat Diagn 19:36-40. 1999
    ....
  35. doi Clinical heterogeneity and diagnostic delay of autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy syndrome
    Cinzia Mazza
    A Nocivelli Institute for Molecular Medicine, Pediatric Clinic, University of Brescia, and Laboratory of Genetic Disease of Childhood, Spedali Civili, Brescia, Italy
    Clin Immunol 139:6-11. 2011
    ..Overall, the mean diagnostic delay in our patients was 10.2 years. These results suggest that molecular analysis of AIRE should be performed in patients with relapsing mucocutaneous candidiasis for early identification of APECED...
  36. ncbi Expansion of CCR4+ activated T cells is associated with memory B cell reduction in DOCK8-deficient patients
    Sonia Caracciolo
    Department of Clinical and Experimental Medicine, Angelo Nocivelli Institute for Molecular Medicine, University of Brescia, Italy
    Clin Immunol 152:164-70. 2014
    ..These observations indicate that DOCK8-deficient patients display a distinctive immunophenotype which is characteristic of this form of HIES...
  37. ncbi Molecular and biochemical characterization of JAK3 deficiency in a patient with severe combined immunodeficiency over 20 years after bone marrow transplantation: implications for treatment
    F Bozzi
    Department of Paediatrics, University of Brescia, Italy
    Br J Haematol 102:1363-6. 1998
    ....
  38. pmc Five-year follow-up of children with perinatal HIV-1 infection receiving early highly active antiretroviral therapy
    Elena Chiappini
    Department of Pediatrics, University of Florence, Florence, Italy
    BMC Infect Dis 9:140. 2009
    ..Nevertheless, data about long-term follow-up of early treated children are lacking...
  39. doi Poor health-related quality of life and abnormal psychosocial adjustment in Italian children with perinatal HIV infection receiving highly active antiretroviral treatment
    Monica Bomba
    Cattedra di Neuropsichiatria dell Infanzia e dell Adolescenza, Universita di Brescia, Brescia, Italy
    AIDS Care 22:858-65. 2010
    ....
  40. doi Is the interruption of antiretroviral treatment during pregnancy an additional major risk factor for mother-to-child transmission of HIV type 1?
    Luisa Galli
    Department of Pediatrics, University of Florence, Italy
    Clin Infect Dis 48:1310-7. 2009
    ..However, this recommendation is poorly supported by data. We evaluated the effects of discontinuing ART during pregnancy on the rate of mother-to-child transmission...
  41. ncbi Clinical and genetic features of Warts, Hypogammaglobulinemia, Infections and Myelokathexis (WHIM) syndrome
    L Dotta
    Department of Pediatrics, Institute of Molecular Medicine Angelo Nocivelli, University of Brescia, Italy
    Curr Mol Med 11:317-25. 2011
    ..A new therapeutic strategy might include the potent inhibitor of CXCR4 function plerixafor (Mozobil), as an agent specifically targeting the molecular defect in order to attenuate the phenotypic manifestations of the syndrome...
  42. pmc Mutations of CD40 gene cause an autosomal recessive form of immunodeficiency with hyper IgM
    S Ferrari
    Istituto di Medicina Molecolare Angelo Nocivelli, Clinica Pediatrica, Universita di Brescia, Piazzale Spedali Civili 1, 25123 Brescia, Italy
    Proc Natl Acad Sci U S A 98:12614-9. 2001
    ..These findings show that mutations of the CD40 gene cause an autosomal recessive form of hyper IgM, which is immunologically and clinically undistinguishable from the X-linked form...
  43. ncbi Toll receptor-mediated regulation of NADPH oxidase in human dendritic cells
    Marisa Vulcano
    Istituto di Ricerche Farmacologiche Mario Negri, Milan, Italy
    J Immunol 173:5749-56. 2004
    ..It is likely that the selective regulation of oxygen radicals production by pathogen-activated DC may function to limit pathogen dissemination during DC trafficking to secondary lymphoid tissues...
  44. ncbi Genotyping for guiding drug choice in human immunodeficiency virus-infected children failing multiple antiretroviral treatment regimens
    Raffaele Badolato
    Pediatr Infect Dis J 24:747-9. 2005
  45. doi Decreased type I interferon receptor-soluble isoform in antiretroviral-treated HIV-positive children
    Alessandra Sottini
    Laboratorio di Biotecnologie, Department of Diagnostics, Spedali Civili di Brescia, Italy
    J Interferon Cytokine Res 28:181-9. 2008
    ....
  46. ncbi Immunological nonresponse to highly active antiretroviral therapy in HIV-infected subjects: is the bone marrow impairment causing CD4 lymphopenia?
    Raffaele Badolato
    Clin Infect Dis 46:1911-2. 2008
  47. pmc Epigenetic regulation of protein-coding and microRNA genes by the Gfi1-interacting tumor suppressor PRDM5
    Zhijun Duan
    Division of Medical Genetics, Department of Medicine, University of Washington School of Medicine, Seattle, WA 98195, USA
    Mol Cell Biol 27:6889-902. 2007
    ..In neutropenic patients, we identified PRDM5 protein sequence variants perturbing transcriptional function, suggesting a potentially important role in hematopoiesis...
  48. ncbi G-CSF treatment of severe congenital neutropenia reverses neutropenia but does not correct the underlying functional deficiency of the neutrophil in defending against microorganisms
    Marta Donini
    Department of Pathology, Section of General Pathology, University of Verona, Italy
    Blood 109:4716-23. 2007
    ..Thereby, we propose that the treatment with G-CSF is not sufficient to correct all of the functional deficiency of neutrophils, and this might account for the consistent risk of infections observed in SCN patients...
  49. pmc A robust immunoassay for anti-interferon autoantibodies that is highly specific for patients with autoimmune polyglandular syndrome type 1
    Li Zhang
    Barbara Davis Center for Childhood Diabetes, University of Colorado Health Sciences Center, Box B140 Building M20, 1775 N Ursula Street, Aurora, CO 80045 6511, USA
    Clin Immunol 125:131-7. 2007
    ..This simple high throughput competitive europium time resolved fluorescence assay had a sensitivity of > or =86% or greater and a specificity of >99.5%...