Giampaolo Merlini

Summary

Affiliation: University of Pavia
Country: Italy

Publications

  1. doi request reprint The controlling roles of Trp60 and Trp95 in beta2-microglobulin function, folding and amyloid aggregation properties
    Gennaro Esposito
    Department of Biomedical Science and Technology, University of Udine, Piazzale Kolbe 4, 33100 Udine, Italy
    J Mol Biol 378:887-97. 2008
  2. pmc Oral melphalan and dexamethasone grants extended survival with minimal toxicity in AL amyloidosis: long-term results of a risk-adapted approach
    Giovanni Palladini
    Haematologica 99:743-50. 2014
  3. pmc Effects of tafamidis on transthyretin stabilization and clinical outcomes in patients with non-Val30Met transthyretin amyloidosis
    Giampaolo Merlini
    Amyloid Research and Treatment Center, IRCCS Fondazione Policlinico San Matteo, University of Pavia, Via Golgi 19, 27100, Pavia, Italy
    J Cardiovasc Transl Res 6:1011-20. 2013
  4. ncbi request reprint Systemic light chain amyloidosis: an update for treating physicians
    Giampaolo Merlini
    Amyloidosis Research and Treatment Center, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico IRCCS Policlinico San Matteo, Pavia, Italy
    Blood 121:5124-30. 2013
  5. doi request reprint Differential diagnosis of monoclonal gammopathy of undetermined significance
    Giampaolo Merlini
    Amyloidosis Research and Treatment Center, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia, Italy
    Hematology Am Soc Hematol Educ Program 2012:595-603. 2012
  6. doi request reprint New criteria for response to treatment in immunoglobulin light chain amyloidosis based on free light chain measurement and cardiac biomarkers: impact on survival outcomes
    Giovanni Palladini
    Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo Pavia, Italy
    J Clin Oncol 30:4541-9. 2012
  7. pmc Synergy of combined doxycycline/TUDCA treatment in lowering Transthyretin deposition and associated biomarkers: studies in FAP mouse models
    Isabel Cardoso
    Molecular Neurobiology Unit, IBMC Instituto de Biologia Molecular e Celular, Rua do Campo Alegre 823, 4150 180 Porto, Portugal
    J Transl Med 8:74. 2010
  8. ncbi request reprint Lysozyme: a paradigmatic molecule for the investigation of protein structure, function and misfolding
    Giampaolo Merlini
    Biotechnology Research Laboratory, University Hospital IRCCS Policlinico San Matteo, Department of Biochemistry, University of Pavia, P le Golgi, 2, 27100 Pavia, Italy
    Clin Chim Acta 357:168-72. 2005
  9. doi request reprint Prevalence and prognostic value of conduction disturbances at the time of diagnosis of cardiac AL amyloidosis
    Michele Boldrini
    Clinica Medica II Department of Internal Medicine, Fondazione IRCCS San Matteo, University of Pavia, Italy
    Ann Noninvasive Electrocardiol 18:327-35. 2013
  10. ncbi request reprint Best use of cardiac biomarkers in patients with AL amyloidosis and renal failure
    Giovanni Palladini
    Amyloidosis Research and Treatment Center, Biotechnology Research Laboratories Department of Molecular Medicine, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico IRCCS Policlinico San Matteo Pavia, Italy
    Am J Hematol 87:465-71. 2012

Detail Information

Publications81

  1. doi request reprint The controlling roles of Trp60 and Trp95 in beta2-microglobulin function, folding and amyloid aggregation properties
    Gennaro Esposito
    Department of Biomedical Science and Technology, University of Udine, Piazzale Kolbe 4, 33100 Udine, Italy
    J Mol Biol 378:887-97. 2008
    ....
  2. pmc Oral melphalan and dexamethasone grants extended survival with minimal toxicity in AL amyloidosis: long-term results of a risk-adapted approach
    Giovanni Palladini
    Haematologica 99:743-50. 2014
    ..Future randomized trials will clarify whether bortezomib is best used in frontline combination with melphalan and dexamethasone or as rescue treatment. ..
  3. pmc Effects of tafamidis on transthyretin stabilization and clinical outcomes in patients with non-Val30Met transthyretin amyloidosis
    Giampaolo Merlini
    Amyloid Research and Treatment Center, IRCCS Fondazione Policlinico San Matteo, University of Pavia, Via Golgi 19, 27100, Pavia, Italy
    J Cardiovasc Transl Res 6:1011-20. 2013
    ..Tafamidis was well tolerated. In conclusion, our findings suggest that tafamidis 20 mg QD effectively stabilized TTR associated with several non-Val30Met variants...
  4. ncbi request reprint Systemic light chain amyloidosis: an update for treating physicians
    Giampaolo Merlini
    Amyloidosis Research and Treatment Center, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico IRCCS Policlinico San Matteo, Pavia, Italy
    Blood 121:5124-30. 2013
    ..Immune-modulatory drugs are good options for refractory/relapsed patients. Novel agents and therapeutic targets are expected to be exploited, in an integrated, more effective and less toxic treatment strategy. ..
  5. doi request reprint Differential diagnosis of monoclonal gammopathy of undetermined significance
    Giampaolo Merlini
    Amyloidosis Research and Treatment Center, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia, Italy
    Hematology Am Soc Hematol Educ Program 2012:595-603. 2012
    ..The ongoing development of novel molecular biomarkers and advanced imaging techniques will improve the identification of high-risk patients who may benefit from early therapeutic intervention through innovative clinical trials...
  6. doi request reprint New criteria for response to treatment in immunoglobulin light chain amyloidosis based on free light chain measurement and cardiac biomarkers: impact on survival outcomes
    Giovanni Palladini
    Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo Pavia, Italy
    J Clin Oncol 30:4541-9. 2012
    ..To identify the criteria for hematologic and cardiac response to treatment in immunoglobulin light chain (AL) amyloidosis based on survival analysis of a large patient population...
  7. pmc Synergy of combined doxycycline/TUDCA treatment in lowering Transthyretin deposition and associated biomarkers: studies in FAP mouse models
    Isabel Cardoso
    Molecular Neurobiology Unit, IBMC Instituto de Biologia Molecular e Celular, Rua do Campo Alegre 823, 4150 180 Porto, Portugal
    J Transl Med 8:74. 2010
    ..The observed synergistic effect of doxycycline/TUDCA in the range of human tolerable quantities, in the transgenic TTR mice models prompts their application in FAP, particularly in the early stages of disease...
  8. ncbi request reprint Lysozyme: a paradigmatic molecule for the investigation of protein structure, function and misfolding
    Giampaolo Merlini
    Biotechnology Research Laboratory, University Hospital IRCCS Policlinico San Matteo, Department of Biochemistry, University of Pavia, P le Golgi, 2, 27100 Pavia, Italy
    Clin Chim Acta 357:168-72. 2005
    ..Four amyloidogenic mutations have been identified and an apparently non-amyloidogenic polymorphism has been recently described...
  9. doi request reprint Prevalence and prognostic value of conduction disturbances at the time of diagnosis of cardiac AL amyloidosis
    Michele Boldrini
    Clinica Medica II Department of Internal Medicine, Fondazione IRCCS San Matteo, University of Pavia, Italy
    Ann Noninvasive Electrocardiol 18:327-35. 2013
    ..To evaluate the prevalence and the prognostic implications of conduction delays in a large cohort of cardiac AL patients...
  10. ncbi request reprint Best use of cardiac biomarkers in patients with AL amyloidosis and renal failure
    Giovanni Palladini
    Amyloidosis Research and Treatment Center, Biotechnology Research Laboratories Department of Molecular Medicine, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico IRCCS Policlinico San Matteo Pavia, Italy
    Am J Hematol 87:465-71. 2012
    ..Natriuretic peptides are powerful and useful markers of cardiac dysfunction and prognosis, provided that eGFR is considered in interpreting their clinical meaning. BNP should be preferred in patients with end-stage renal failure...
  11. doi request reprint Serum prealbumin: an independent marker of short-term energy intake in the presence of multiple-organ disease involvement
    Riccardo Caccialanza
    Nutrition and Dietetics Service, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
    Nutrition 29:580-2. 2013
    ....
  12. doi request reprint Midregional proadrenomedullin (MR-proADM) is a powerful predictor of early death in AL amyloidosis
    Giovanni Palladini
    Amyloidosis Research and Treatment Center, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico IRCCS Policlinico San Matteo and Department of Biochemistry, University of Pavia, Pavia, Italy
    Amyloid 18:216-21. 2011
    ..Adrenomedullin is produced by several tissues including the heart, and portends a poor prognosis in heart diseases. We investigated the ability of midregional proadrenomedullin (MR-proADM) to predict early death in AL amyloidosis...
  13. doi request reprint Salvage therapy with lenalidomide and dexamethasone in patients with advanced AL amyloidosis refractory to melphalan, bortezomib, and thalidomide
    Giovanni Palladini
    Amyloidosis Research and Treatment Center, Foundation Istituto di Ricovero e Cura a Carattere scientifico Policlinico San Matteo, Pavia, Italy
    Ann Hematol 91:89-92. 2012
    ..Salvage therapy beyond second line of treatment can improve survival in AL amyloidosis and lenalidomide plus dexamethasone is a valuable option in this setting...
  14. doi request reprint Treatment of patients with advanced cardiac AL amyloidosis with oral melphalan, dexamethasone, and thalidomide
    Giovanni Palladini
    Biotechnology Research Laboratories, Center for Amyloidosis, Department of Biochemistry, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico San Matteo and University of Pavia, Piazzale Golgi, 19, 27100 Pavia, Italy
    Ann Hematol 88:347-50. 2009
    ..Treatment with MTD is feasible in patients with advanced cardiac AL amyloidosis and effective in subjects with preserved systolic function...
  15. doi request reprint The repertoire of λ light chains causing predominant amyloid heart involvement and identification of a preferentially involved germline gene, IGLV1-44
    Vittorio Perfetti
    Amyloid Research and Treatment Center, Biotechnology Research Laboratories, Pavia, Italy
    Blood 119:144-50. 2012
    ..Study of the characteristics of IGVL1-44-LC with, and of the minority without, heart involvement might lead to identification of LC/tissue interactions...
  16. doi request reprint SIE, SIES, GITMO evidence-based guidelines on novel agents (thalidomide, bortezomib, and lenalidomide) in the treatment of multiple myeloma
    Giovanni Barosi
    Laboratory of Clinical Epidemiology and Center for the Study of Myelofibrosis, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
    Ann Hematol 91:875-88. 2012
    ..In patients who are refractory or relapsing after first-line therapy, we recommended bortezomib and pegylated liposomal doxorubicin, or lenalidomide and dexamethasone combinations (weak positive)...
  17. doi request reprint The combination of high-sensitivity cardiac troponin T (hs-cTnT) at presentation and changes in N-terminal natriuretic peptide type B (NT-proBNP) after chemotherapy best predicts survival in AL amyloidosis
    Giovanni Palladini
    Amyloidosis Research and Treatment Center and Department of Biochemistry, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico IRCCS Policlinico San Matteo and University of Pavia, Viale Golgi 19, Pavia, Italy
    Blood 116:3426-30. 2010
    ..In AL amyloidosis, hs-cTnT is the best baseline prognostic marker. Therapy should be aimed at preventing progression of cardiac biomarkers, whereas NT-proBNP response confers an additional survival benefit...
  18. ncbi request reprint The workings of the amyloid diseases
    Vittorio Bellotti
    Center for Amyloidosis, Department of Biochemistry, Biotechnology Research Laboratories, Foundation IRCCS Policlinico San Matteo, Pavia, Italy
    Ann Med 39:200-7. 2007
    ..Due to the pathogenic complexity of amyloid diseases, the integration of several therapeutic interventions involving different critical levels of the amyloidogenic cascade is envisaged...
  19. pmc A strategy for synthesis of pathogenic human immunoglobulin free light chains in E. coli
    Paola Rognoni
    Amyloidosis Research and Treatment Center and Biotechnology Research Laboratories, Fondazione IRCCS Policlinico S Matteo and Department of Molecular Medicine, University of Pavia, Pavia, Italy
    PLoS ONE 8:e76022. 2013
    ..Furthermore, as proof of pathogenicity, recombinant proteins formed amyloid fibrils in vitro. We believe that the present strategy represents a valuable tool to speed research in free LC-related disorders. ..
  20. pmc A phase II trial of cyclophosphamide, lenalidomide and dexamethasone in previously treated patients with AL amyloidosis
    Giovanni Palladini
    Amyloidosis Research and Treatment Center, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia, Italy
    Haematologica 98:433-6. 2013
    ..clinicaltrials.gov identifier: NCT00607581)...
  21. doi request reprint Proteomics in protein misfolding diseases
    Monica Stoppini
    Department of Biochemistry, University of Pavia, Pavia, Italy
    Clin Chem Lab Med 47:627-35. 2009
    ..We present the developments in the field of proteomics applied to systemic amyloidoses, and discuss the perspectives opened in the study of these diseases...
  22. ncbi request reprint Identification of amyloidogenic light chains requires the combination of serum-free light chain assay with immunofixation of serum and urine
    Giovanni Palladini
    Center for Amyloidosis, Biotechnology Research Laboratories, Department of Biochemistry, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo and University of Pavia, Pavia, Italy
    Clin Chem 55:499-504. 2009
    ....
  23. doi request reprint Prognostic value of fragmented QRS in cardiac AL amyloidosis
    Stefano Perlini
    Clinica Medica II, Department of Internal Medicine, Fondazione IRCCS San Matteo, University of Pavia, Pavia, Italy
    Int J Cardiol 167:2156-61. 2013
    ..Since cardiomyocyte damage and interstitial fibrosis are associated with cardiac amyloid deposition, aim of the present study was to analyze the prevalence and the potential prognostic value of fQRS in patients with cardiac amyloidosis...
  24. ncbi request reprint Circulating amyloidogenic free light chains and serum N-terminal natriuretic peptide type B decrease simultaneously in association with improvement of survival in AL
    Giovanni Palladini
    Biotechnology Research Laboratories IRCCS Policlinico San Matteo, Piazzale Golgi 19, 27100 Pavia, Italy
    Blood 107:3854-8. 2006
    ..Cardiac function in AL can rapidly improve due to a reduction of the circulating amyloidogenic precursor, despite the amount of cardiac amyloid deposits remaining apparently unaltered, as measured by echocardiography...
  25. ncbi request reprint Prognostic value of depressed midwall systolic function in cardiac light-chain amyloidosis
    Stefano Perlini
    aDepartment of Internal Medicine bAmyloidosis Research and Treatment Center and Department of Molecular Medicine, Fondazione IRCCS San Matteo, University of Pavia, Pavia cIntensive Cardiac Care Unit, Heart and Vessel Department dInternal Medicine Department, University of Florence, Florence eInstitute of Cardiology, S Orsola Malpighi Hospital, University of Bologna, Bologna, Italy
    J Hypertens 32:1121-31; discussion 1131. 2014
    ..In another clinical model of HFpEF, that is, pressure-overload hypertrophy, depressed midwall fractional shortening (mFS) has been shown to be a powerful prognostic factor...
  26. doi request reprint Treatment of IgM-associated AL amyloidosis with the combination of rituximab, bortezomib, and dexamethasone
    Giovanni Palladini
    Department of Biochemistry, Amyloidosis Research and Treatment Center, Fondazione IRCCS Policlinico San Matteo and University of Pavia, Pavia, Italy
    Clin Lymphoma Myeloma Leuk 11:143-5. 2011
    ..Severe adverse events (grade ≥ 3) were observed in 3 cases. Treatment of IgM-AL amyloidosis with RBDex warrants further investigation in the setting of international clinical trials...
  27. ncbi request reprint Nutritional status of outpatients with systemic immunoglobulin light-chain amyloidosis 1
    Riccardo Caccialanza
    Nutrition and Dietetics Service and the Department of Health Sciences, Section of Human Nutrition and Dietetics, University of Pavia, Pavia, Italy
    Am J Clin Nutr 83:350-4. 2006
    ..Maintenance of a good nutritional status is associated with prolonged survival in many chronic diseases. To date, the nutritional status of outpatients with immunoglobulin light-chain (AL) amyloidosis has not been evaluated...
  28. doi request reprint Nutritional status independently affects quality of life of patients with systemic immunoglobulin light-chain (AL) amyloidosis
    Riccardo Caccialanza
    Nutrition and Dietetics Service, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
    Ann Hematol 91:399-406. 2012
    ..Nutritional evaluation should be integral part of the clinical assessment of AL patients. Nutritional support intervention trials are warranted in such patients' population...
  29. ncbi request reprint A novel AbetaPP mutation exclusively associated with cerebral amyloid angiopathy
    Laura Obici
    Amyloid Center, Biotechnology Research Laboratories, IRCCS Policlinico San Matteo, Pavia, Italy
    Ann Neurol 58:639-44. 2005
    ..Pathological examination disclosed severe CAA, without parenchymal amyloid plaques or neurofibrillary tangles. This variant highlights the vascular tropism of mutated Abeta, resulting in CAA instead of the pathological hallmarks of AD...
  30. ncbi request reprint Structure, function and amyloidogenic propensity of apolipoprotein A-I
    Laura Obici
    Centro per lo Studio e la Cura delle Amiloidosi Sistemiche, Laboratorio di Biotecnologie, IRCCS Policlinico San Matteo, Pavia, Italy
    Amyloid 13:191-205. 2006
    ....
  31. ncbi request reprint Importance of the echocardiographic evaluation of right ventricular function in patients with AL amyloidosis
    Stefano Ghio
    Division of Cardiology, Fondazione IRCCS Policlinico S Matteo and University of Pavia, 27100 Pavia, Italy
    Eur J Heart Fail 9:808-13. 2007
    ..Patients with AL amyloidosis often present with signs of congestive heart failure...
  32. ncbi request reprint Prognostic validation of the international classification of immunoglobulin M gammopathies: a survival advantage for patients with immunoglobulin M monoclonal gammopathy of undetermined significance?
    Paolo G Gobbi
    Medicina Interna e Oncologia Medica and Laboratorio di Ricerche Biotecnologiche, Universita di Pavia, IRCCS Policlinico S Matteo, Piazzale Golgi no 2, 27100 Pavia, Italy
    Clin Cancer Res 11:1786-90. 2005
    ..To verify the reliability of the new criteria for the diagnosis of IgM gammopathies recently proposed by an international panel of experts (Athens, 2002)...
  33. pmc Lysine 58-cleaved beta2-microglobulin is not detectable by 2D electrophoresis in ex vivo amyloid fibrils of two patients affected by dialysis-related amyloidosis
    Sofia Giorgetti
    Department of Biochemistry, University of Pavia Laboratori di Biotecnologie, IRCCS Policlinico San Matteo, via Taramelli 3b, 27100 Pavia, Italy
    Protein Sci 16:343-9. 2007
    ..In contrast, we found no evidence for the presence of DeltaK58-beta2m...
  34. doi request reprint Clinical, radiological, and biochemical features of a bilateral buttock amyloidoma emerging after 27 years of hemodialysis
    Giovanni Montagna
    IRCCS Fondazione Salvatore Maugeri, Pavia, Italy
    Amyloid 16:115-21. 2009
    ....
  35. ncbi request reprint Advantages of the lognormal approach to determining reference change values for N-terminal propeptide B-type natriuretic peptide
    Catherine Klersy
    Department of Biometry and Clinical Epidemiology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
    Clin Chim Acta 413:544-7. 2012
    ..Currently, there is no consensus on which approach (nANOVA or lognormal) to determining RCV is better...
  36. doi request reprint Effects of the known pathogenic mutations on the aggregation pathway of the amyloidogenic peptide of apolipoprotein A-I
    Sara Raimondi
    Department of Biochemistry, University of Pavia, 27100 Pavia, Italy
    J Mol Biol 407:465-76. 2011
    ....
  37. ncbi request reprint Bone marrow amyloidosis
    Rosangela Invernizzi
    Department of Internal Medicine, University of Pavia and IRCCS Policlinico S Matteo, Pavia, Italy
    Haematologica 91:EIM01. 2006
  38. doi request reprint Benefit of doxycycline treatment on articular disability caused by dialysis related amyloidosis
    Giovanni Montagna
    Division of Nephrology and Hemodialysis, Salvatore Maugeri Foundation, IRCCS Rehabilitation Institute, Pavia, Italy
    Amyloid 20:173-8. 2013
    ....
  39. ncbi request reprint Clinical aspects of systemic amyloid diseases
    Laura Obici
    Amyloid Center, Biotechnology Research Laboratories, IRCCS Policlinico San Matteo, Pavia, Italy
    Biochim Biophys Acta 1753:11-22. 2005
    ....
  40. ncbi request reprint Liver biopsy discloses a new apolipoprotein A-I hereditary amyloidosis in several unrelated Italian families
    Laura Obici
    Biotechnology Research Laboratories, IRCCS Policlinico San Matteo, Viale Golgi 19, 27100 Pavia, Italy
    Gastroenterology 126:1416-22. 2004
    ....
  41. ncbi request reprint Serum N-terminal pro-brain natriuretic peptide is a sensitive marker of myocardial dysfunction in AL amyloidosis
    Giovanni Palladini
    Department of Internal Medicine, University Hospital IRCCS Policlinico San Matteo University of Pavia, Pavia, Italy
    Circulation 107:2440-5. 2003
    ..We investigated the prognostic value of NT-proBNP and its utility in monitoring amyloid heart dysfunction...
  42. ncbi request reprint Characterization of immunoglobulin variable regions of two human pathogenic monoclonal cryocrystalglobulins
    Valentina Navazza
    Department of Internal Medicine, Clinica Medica I, Fondazione IRCCS Policlinico San Matteo University of Pavia, P le Golgi 2, 27100 Pavia, Italy
    Mol Immunol 45:1519-24. 2008
    ..In particular, a rare replacement of a polar residue with proline is shared at the beginning of the VH complementarity-determining region 2, and this residue might be involved in intermolecular contacts...
  43. ncbi request reprint Proteomics of beta2-microglobulin amyloid fibrils
    Monica Stoppini
    Dipartimento di Biochimica, Universita degli Studi di Pavia, via Taramelli 3b, 27100 Pavia, Italy
    Biochim Biophys Acta 1753:23-33. 2005
    ..Other previously reported chemical modifications cannot be excluded, but should involve less than 1-2% of the intact molecule...
  44. ncbi request reprint Long-term results of a risk-adapted approach to melphalan conditioning in autologous peripheral blood stem cell transplantation for primary (AL) amyloidosis
    Vittorio Perfetti
    Amyloid Center, IRCCS Policlinico S Matteo P le Golgi 2, 27100 Pavia, Italy
    Haematologica 91:1635-43. 2006
    ..Refined selection of patients suitable for transplantation and personalized adaptation of the doses of melphalan might improve the outcome...
  45. ncbi request reprint Therapy and management of systemic AL (primary) amyloidosis
    Giovanni Palladini
    Biotechnology Research Laboratories, Centre for Amyloidosis, Department of Biochemistry, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico IRCCS San Matteo, Pavia, Italy
    Swiss Med Wkly 136:715-20. 2006
    ..These new tools can be combined in order to maximise the improvement of organ dysfunction and minimise toxicity, adapting the intervention to each patient...
  46. ncbi request reprint Association of melphalan and high-dose dexamethasone is effective and well tolerated in patients with AL (primary) amyloidosis who are ineligible for stem cell transplantation
    Giovanni Palladini
    Amyloid Center Biotechnology Research Laboratories, Department of Biochemistry, University Hospital, Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia, Italy
    Blood 103:2936-8. 2004
    ..M-Dex represents a feasible and effective therapeutic option for patients with advanced AL who are ineligible for ASCT...
  47. doi request reprint A novel approach for the purification and proteomic analysis of pathogenic immunoglobulin free light chains from serum
    Francesca Lavatelli
    Centro per lo Studio e la Cura delle Amiloidosi Sistemiche, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
    Biochim Biophys Acta 1814:409-19. 2011
    ..This method is a novel instrument for studying the molecular bases of FLC pathogenicity, allowing for the first time the punctual biochemical description of the circulating forms...
  48. doi request reprint Risk stratification in Waldenström macroglobulinemia
    Pierre Morel
    Amyloidosis Research and Treatment Center, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Italy
    Expert Rev Hematol 5:187-99. 2012
    ..This limitation should be taken into account when using and interpreting available prognostic information, especially survival estimates...
  49. doi request reprint Doxycycline plus tauroursodeoxycholic acid for transthyretin amyloidosis: a phase II study
    Laura Obici
    Amyloid Research and Treatment Center, Fondazione IRCCS Policlinico San Matteo, and Department of Molecular Medicine, University of Pavia, Pavia, Italy
    Amyloid 19:34-6. 2012
    ..These preliminary data indicate that the combination of Doxy-TUDCA stabilizes the disease for at least 1 year in the majority of patients with an acceptable toxicity profile...
  50. doi request reprint Diagnostic and prognostic value of low QRS voltages in cardiac AL amyloidosis
    Roberta Mussinelli
    Clinica Medica II, Department of Internal Medicine, University of Pavia, P le Golgi 19, 27100 Pavia, Italy
    Ann Noninvasive Electrocardiol 18:271-80. 2013
    ..Although considered as one of the hallmarks of the disease, its reported prevalence varies from 45% to 70%, mainly because of nonhomogeneous definitions...
  51. ncbi request reprint First report of systemic reactive (AA) amyloidosis in a patient with the hyperimmunoglobulinemia D with periodic fever syndrome
    Laura Obici
    University Hospital IRCCS Policlinico San Matteo, Pavia, Italy
    Arthritis Rheum 50:2966-9. 2004
    ..In this patient, 2 mutations in the mevalonate kinase gene were identified, one of which, the leucine-to-arginine substitution at codon 265, is novel...
  52. ncbi request reprint Conformational switching and fibrillogenesis in the amyloidogenic fragment of apolipoprotein a-I
    Alessia Andreola
    Department of Biochemistry and Centro Interdipartimentale di Biologia Applicata, University of Pavia, via Taramelli 3b, Italy
    J Biol Chem 278:2444-51. 2003
    ....
  53. pmc Insights into the regulation of immunoglobulin light chain gene rearrangements via analysis of the kappa light chain locus in lambda myeloma
    Vittorio Perfetti
    Internal Medicine and Medical Oncology, IRCCS Policlinico S Matteo University of Pavia, Pavia, Italy
    Immunology 112:420-7. 2004
    ..These findings provide insights into the regulation of light chain rearrangements and support the view that B cells may occasionally undergo sequential light chain rearrangements after the onset of somatic hypermutation...
  54. doi request reprint Diagnostic challenges of amyloidosis in Waldenström macroglobulinemia
    Giovanni Palladini
    Amyloidosis Research and Treatment Center, Department of Molecular Medicine, Fondazione IRCCS Policlinico San Matteo and University of Pavia, Pavia, Italy
    Clin Lymphoma Myeloma Leuk 13:244-6. 2013
    ..Prognostic stratification should include serum albumin concentration, which is an independent prognostic factor...
  55. ncbi request reprint Prognostic factors in symptomatic Waldenstrom's macroglobulinemia
    Giampaolo Merlini
    Research Biotechnology Laboratories, and Internal Medicine and Medical Oncology, IRCCS Policlinico San Matteo, Pavia, Italy
    Semin Oncol 30:211-5. 2003
    ..A prognostic index based on age, hemoglobin, and albumin is capable of identifying various groups of patients with different therapeutic needs...
  56. pmc Impaired osteoblastogenesis in a murine model of dominant osteogenesis imperfecta: a new target for osteogenesis imperfecta pharmacological therapy
    Roberta Gioia
    Department of Molecular Medicine, Section of Biochemistry, University of Pavia, Via Taramelli 3 B, Pavia, Italy
    Stem Cells 30:1465-76. 2012
    ..This is the first report of impaired MSC differentiation to osteoblasts in OI, and it identifies a new potential target for the pharmacological treatment of the disorder...
  57. ncbi request reprint Molecular mechanisms of amyloidosis
    Giampaolo Merlini
    Amyloid Center, Biotechnology Research Laboratory, University Hospital IRCCS Policlinico San Matteo, Pavia, Italy
    N Engl J Med 349:583-96. 2003
  58. ncbi request reprint Multicentre versus single centre approach to rare diseases: the model of systemic light chain amyloidosis
    Giovanni Palladini
    Amyloidosis Centre, IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy
    Amyloid 12:120-6. 2005
    ..We compared the AL patient population observed at a referral center, Mayo Clinic, with that recruited through the Italian network...
  59. pmc Analysis of hepatitis C virus hypervariable region 1 sequence from cryoglobulinemic patients and associated controls
    Gabriella Bianchettin
    Area Infettivologica, Dipartimento di Malattie Infettive, IRCCS Policlinico San Matteo, Via Taramelli 5, 27100 Pavia, Italy
    J Virol 81:4564-71. 2007
    ..Specific changes in HCV envelope sequence distribution are unlikely to be directly involved in the establishment of pathological B-cell monoclonal proliferation...
  60. ncbi request reprint Mechanisms of renal damage in plasma cell dyscrasias: an overview
    Giampaolo Merlini
    Amyloidosis Center, Biotechnology Research Laboratories, Fondazione, IRCCS Policlinico San Matteo and Department of Biochemistry, University of Pavia, Pavia, Italy
    Contrib Nephrol 153:66-86. 2007
    ..In patients with known plasma cell dyscrasia, the recognition of these patterns of renal injury should lead to appropriate therapeutic intervention...
  61. ncbi request reprint AA amyloidosis: basic knowledge, unmet needs and future treatments
    Laura Obici
    Amyloidosis Research and Treatment Centre, Biotechnology Research Laboratories, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
    Swiss Med Wkly 142:w13580. 2012
    ..These therapies will hopefully improve the quality of life and outcome of these patients in a near future...
  62. doi request reprint Transplantation vs. conventional-dose therapy for amyloidosis
    Giovanni Palladini
    Department of Biochemistry, University of Pavia, Pavia, Italy
    Curr Opin Oncol 23:214-20. 2011
    ..This review focuses on the role of ASCT and conventional-dose therapy in light of advances in risk stratification and patient monitoring...
  63. doi request reprint Immunoglobulin light chain amyloidosis
    Giampaolo Merlini
    Department of Molecular Medicine, University of Pavia, Foundation Scientific Institute San Matteo, Amyloidosis Research and Treatment Center, V le Golgi 19 27100, Pavia, Italy
    Expert Rev Hematol 7:143-56. 2014
    ....
  64. ncbi request reprint Management of multiple myeloma and related-disorders: guidelines from the Italian Society of Hematology (SIE), Italian Society of Experimental Hematology (SIES) and Italian Group for Bone Marrow Transplantation (GITMO)
    Giovanni Barosi
    Laboratorio di Epidemiologia Clinica, IRCCS Policlinico S Matteo, Pavia, Italy
    Haematologica 89:717-41. 2004
    ....
  65. doi request reprint Diagnostic performance of triggering receptor expressed on myeloid cells-1 and CD64 index as markers of sepsis in preterm newborns
    Iolanda Mazzucchelli
    Laboratory of Neonatal Immunology, Neonatal Intensive Care Unit, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
    Pediatr Crit Care Med 14:178-82. 2013
    ..The study aim is to assess diagnostic performance, individually and combined, of CD64 index and triggering receptor expressed on myeloid cells-1 (surface marker/soluble form), in late-onset sepsis of preterm infants...
  66. ncbi request reprint Embryonic stem and haematopoietic progenitor cells resist to Aβ oligomer toxicity and maintain the differentiation potency in culture
    Tui Neri
    Dipartimento di Biologia Animale, Centro di Ricerca Interdipartimentale di Ingegneria Tissutale, University of Pavia, Pavia
    Amyloid 17:137-45. 2010
    ..Our results support a remarkable resistance of the investigated stem cells against amyloids and hence their potential use in cell therapy of Alzheimer's disease and, possibly, other amyloid diseases...
  67. ncbi request reprint Obstructive intramural coronary amyloidosis: a distinct phenotype of cardiac amyloidosis that can cause acute heart failure
    Michele Pasotti
    Department of Cardiology, I R C C S Policlinico San Matteo, Pavia, Italy
    Eur Heart J 27:1810. 2006
  68. ncbi request reprint Dangerous small B-cell clones
    Giampaolo Merlini
    Amyloid Center, Biotechnology Research Laboratories, Foundation IRCCS Policlinico San Matteo, Piazzale Golgi 2, 27100 Pavia, Italy
    Blood 108:2520-30. 2006
    ..Although the treatment of these conditions is not optimal, significant advances have been made, improving the duration and quality of life...
  69. pmc Amyloidosis: pathogenesis and new therapeutic options
    Giampaolo Merlini
    Amyloidosis Research and Treatment Center, Foundation IRCCS Policlinico San Matteo, University of Pavia, P le Golgi, 19, 27100 Pavia, Italy
    J Clin Oncol 29:1924-33. 2011
    ..Advances in basic research have led to the identification of new drug targets and therapeutic approaches, which will be integrated with chemotherapy in the future...
  70. ncbi request reprint Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis (AL): a consensus opinion from the 10th International Symposium on Amyloid and Amyloidosis, Tours, France, 18-22 April 2004
    Morie A Gertz
    Dysproteinemia Clinic, Mayo Clinic, Rochester, Minnesota 55905, USA
    Am J Hematol 79:319-28. 2005
    ..We report criteria that centers can now use to define organ involvement and uniform response criteria for reporting outcomes in patients with light-chain AL...
  71. pmc Amyloidogenic and associated proteins in systemic amyloidosis proteome of adipose tissue
    Francesca Lavatelli
    Amyloid Treatment and Research Program, Boston, Massachusetts, USA
    Mol Cell Proteomics 7:1570-83. 2008
    ....
  72. ncbi request reprint Electron and immuno-electron microscopy of abdominal fat identifies and characterizes amyloid fibrils in suspected cardiac amyloidosis
    Eloisa Arbustini
    Amyloid 9:108-14. 2002
    ..Our results demonstrate that amyloid deposits are present in the abdominalfat of patients suspected to have cardiac amyloidosis and that immuno-electron microscopy was able to characterize the amyloid protein in all cases...
  73. ncbi request reprint Amyloid fibril protein nomenclature -- 2002
    Per Westermark
    Amyloid 9:197-200. 2002
  74. ncbi request reprint 4'-iodo-4'-deoxydoxorubicin and tetracyclines disrupt transthyretin amyloid fibrils in vitro producing noncytotoxic species: screening for TTR fibril disrupters
    Isabel Cardoso
    Amyloid Unit, Institute for Molecular and Cell Biology, University of Porto, Portugal
    FASEB J 17:803-9. 2003
    ....
  75. ncbi request reprint A primer of amyloid nomenclature
    Per Westermark
    Department of Genetics and Pathology, Uppsala University, Uppsala, Sweden
    Amyloid 14:179-83. 2007
    ..All amyloid types are today preferably named by their major fibril protein. This makes a simple and rational nomenclature for the increasing number of amyloid disorders known in humans and animals...
  76. ncbi request reprint Genetic microheterogeneity of human transthyretin detected by IEF
    Klaus Altland
    Institut fur Humangenetik, Justus Liebig Universitat, Giessen, Germany
    Electrophoresis 28:2053-64. 2007
    ..All types of amyloidogenic mutations affected the stability of TTR tetramers...
  77. ncbi request reprint Pharmaceutical strategies against amyloidosis: old and new drugs in targeting a "protein misfolding disease"
    Ersilia De Lorenzi
    Dipartimento di Chimica Farmaceutica, Universita di Pavia, Italy
    Curr Med Chem 11:1065-84. 2004
    ....
  78. ncbi request reprint Hereditary amyloidosis
    Giovanni Palladini
    N Engl J Med 347:1206-7; author reply 1206-7. 2002
  79. ncbi request reprint Ultrastructural organization of ex vivo amyloid fibrils formed by the apolipoprotein A-I Leu174Ser variant: an atomic force microscopy study
    Annalisa Relini
    National Institute for the Physics of Matter and Department of Physics, University of Genoa, Via Dodecaneso 33, I 16146, Genoa, Italy
    Biochim Biophys Acta 1690:33-41. 2004
    ..4+/-0.4 and 5.1+/-0.5 nm, measured under liquid) and is compatible with the protofilament expected diameter, suggesting that globules may represent protofilament precursors...
  80. ncbi request reprint Amyloid: toward terminology clarification. Report from the Nomenclature Committee of the International Society of Amyloidosis
    Per Westermark
    Department of Genetics and Pathology, Uppsala University, Uppsala, Sweden
    Amyloid 12:1-4. 2005
    ..Synthetic fibrils with amyloid properties are best named 'amyloid-like'. With increasing knowledge, however, the borders between different protein aggregates tend to become less sharp...
  81. doi request reprint Sjögren's syndrome and localized nodular cutaneous amyloidosis: coincidence or a distinct clinical entity?
    Jiska M Meijer
    University of Groningen, Groningen, The Netherlands
    Arthritis Rheum 58:1992-9. 2008
    ..To report 8 patients with Sjögren's syndrome (SS) and localized nodular cutaneous amyloidosis and to examine serologic and immunohistologic findings that may link the 2 diseases...