Affiliation: University of Pavia
- Myeloma kidney: advances in molecular mechanisms of acute kidney injury open novel therapeutic opportunitiesAndrew Davenport
UCL Centre for Nephrology, Royal Free Hospital, London, UK
Nephrol Dial Transplant 27:3713-8. 2012..Basic research findings have led to the design of targeted drugs that prevented the functional manifestations of acute kidney injury in animal models. There is now hope to prevent the renal damage in patients with MM...
- Doxycycline plus tauroursodeoxycholic acid for transthyretin amyloidosis: a phase II studyLaura 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...
- The Pavia approach to clinical protein analysisG Merlini
Biotechnology Research Laboratories, University Hospital IRCCS Policlinico San Matteo, Pavia, Italy
Clin Chem Lab Med 39:1025-8. 2001..This labor-intensive approach requires skills in the performance of protein analysis and in the interpretation/referral phase, as well as close communication with the attending physician...
- Mechanisms of renal damage in plasma cell dyscrasias: an overviewGiampaolo 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...
- Standardizing plasma protein measurements worldwide: a challenging enterpriseGiampaolo Merlini
Biotechnology Research Laboratories, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Department of Biochemistry, University of Pavia, Pavia, Italy
Clin Chem Lab Med 48:1567-75. 2010..Plasma protein reference materials have had a substantial impact in improving the harmonization of patient protein results that should translate into better patient care...
- Molecular mechanisms of amyloidosisGiampaolo Merlini
Amyloid Center, Biotechnology Research Laboratory, University Hospital IRCCS Policlinico San Matteo, Pavia, Italy
N Engl J Med 349:583-96. 2003
- Protein aggregationG Merlini
Biotechnology Research Laboratories, University Hospital IRCCS Policlinico San Matteo, Pavia, Italy
Clin Chem Lab Med 39:1065-75. 2001..As different molecular mechanisms are involved in the formation of the various forms of protein aggregates, the laboratory diagnostic approach remains frequently elusive...
- Dangerous small B-cell clonesGiampaolo 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...
- Amyloidosis: pathogenesis and new therapeutic optionsGiampaolo 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...
- A modified high-dose dexamethasone regimen for primary systemic (AL) amyloidosisG Palladini
Internal Medicine and Medical Oncology, and Biotechnology Research Laboratories, Policlinico San Matteo, Pavia, Italy
Br J Haematol 113:1044-6. 2001..This regimen may be considered front-line therapy when autologous stem cell transplantation is not feasible and when a rapid response is particularly important...
- Amyloid fibrils derived from the apolipoprotein A1 Leu174Ser variant contain elements of ordered helical structureP Mangione
Department of Biochemistry, University of Pavia, 27100 Pavia, Italy
Protein Sci 10:187-99. 2001..The addition of phospholipids to the monomeric form induces the formation of some helical structure, thought most likely to occur at the C-terminal end of the polypeptide...
- Serum N-terminal pro-brain natriuretic peptide is a sensitive marker of myocardial dysfunction in AL amyloidosisGiovanni 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...
- Biological features of the clone involved in primary amyloidosis (AL)V Perfetti
Department of Internal Medicine, IRCCS Policlinico S Matteo, University of Pavia, Italy
Leukemia 15:195-202. 2001..Knowledge of the pathobiology of the underlying B cell clone is of primary importance for the design and optimization of therapeutic strategies...
- Holter monitoring in AL amyloidosis: prognostic implicationsG Palladini
Department of Internal Medicine and Medical Oncology, University of Pavia, Italy
Pacing Clin Electrophysiol 24:1228-33. 2001..The presence of couplets correlated with sudden death. Holter monitoring may contribute to assessing the prognosis of patients with AL amyloidosis...
- Clotting alterations in primary systemic amyloidosisG Gamba
Istituto di Medicina Interna e Oncologia Medica, IRCCS Policlinico San Matteo, 27100 Pavia, Italy
Haematologica 85:289-92. 2000..This investigation was aimed at defining the prevalence of clotting abnormalities and their clinical manifestations in patients with AL...
- The new apolipoprotein A-I variant leu(174) --> Ser causes hereditary cardiac amyloidosis, and the amyloid fibrils are constituted by the 93-residue N-terminal polypeptideL Obici
Biotechnology Research Laboratories, Institute of Human Pathology, Division of Cardiology, IRCCS Policlinico San Matteo, Pavia, Italy
Am J Pathol 155:695-702. 1999....
- Treatment of patients with advanced cardiac AL amyloidosis with oral melphalan, dexamethasone, and thalidomideGiovanni 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...
- 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....
- Bone marrow amyloidosisRosangela Invernizzi
Department of Internal Medicine, University of Pavia and IRCCS Policlinico S Matteo, Pavia, Italy
Haematologica 91:EIM01. 2006
- Importance of the echocardiographic evaluation of right ventricular function in patients with AL amyloidosisStefano 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...
- Nutritional status of outpatients with systemic immunoglobulin light-chain amyloidosis 1Riccardo 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...
- 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)...
- [Fibril-forming proteins: the amyloidosis. New hopes for a disease that cardiologists must know]Eloisa Arbustini
Laboratorio di Diagnostica Molecolare, Area Trapiantologica di Ricerca, IRCCS Policlinico San Matteo, Dipartimento di Biochimica, Universita degli Studi, Pavia
Ital Heart J Suppl 3:590-7. 2002..The fine-needle biopsy of the periumbilical fat is the preferral procedure for amyloid detection and immunocharacterization of amyloid protein. This approach excludes, with a few exceptions, the need of endomyocardial biopsy...
- Electron and immuno-electron microscopy of abdominal fat identifies and characterizes amyloid fibrils in suspected cardiac amyloidosisEloisa 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...
- Amyloidosis: is a cure possible?G Merlini
Amyloidosis Center, Fondazione IRCCS Policlinico San Matteo University of Pavia, Pavia, Italy
Ann Oncol 19:iv63-6. 2008
- Widespread cardiovascular autonomic dysfunction in primary amyloidosis: does spontaneous hyperventilation have a compensatory role against postural hypotension?L Bernardi
Department of Internal Medicine, IRCCS S Matteo and University of Pavia, Pavia, Italy
Heart 88:615-21. 2002..To investigate the possible causes of abnormal blood pressure control in light chain related (primary, AL) amyloidosis...
- 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 amyloidosisGiovanni 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...
- Association of melphalan and high-dose dexamethasone is effective and well tolerated in patients with AL (primary) amyloidosis who are ineligible for stem cell transplantationGiovanni 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...
- Therapy and management of systemic AL (primary) amyloidosisGiovanni 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...
- Liver biopsy discloses a new apolipoprotein A-I hereditary amyloidosis in several unrelated Italian familiesLaura Obici
Biotechnology Research Laboratories, IRCCS Policlinico San Matteo, Viale Golgi 19, 27100 Pavia, Italy
Gastroenterology 126:1416-22. 2004....
- The combination of thalidomide and intermediate-dose dexamethasone is an effective but toxic treatment for patients with primary amyloidosis (AL)Giovanni Palladini
Center for Amyloidosis, Biotechnology Research Laboratories, IRCCS Policlinico San Matteo, Viale Golgi, 19 27100 Pavia, Italy
Blood 105:2949-51. 2005..The combination of thalidomide and dexamethasone is rapidly effective and may represent a valuable second-line treatment for AL...
- Utility of biochemical markers in the follow-up of heart transplant recipientsA Balduini
Biotechnology Research and Clinical Chemistry Laboratories, Department of Biochemistry, University of Pavia, IRCCS Policlinico S Matteo, Piazzale Golgi 2, 27100 Pavia, Italy
Transplant Proc 35:3075-8. 2003..Myo and CK-MBm did not show significant changes. cTnT seems to be a potentially useful addition to the EMB results, while SAA and CRP showed variations with respect to EMB grade both in central and peripheral samples...
- Translocation T(4;14)(p16.3;q32) is a recurrent genetic lesion in primary amyloidosisV Perfetti
Department of Internal Medicine, Internal Medicine and Medical Oncology, University of Pavia, Pavia, Italy
Am J Pathol 158:1599-603. 2001..In conclusion, our results demonstrate that the t(4;14)(p16.3;q32) translocation is a recurrent genetic lesion in primary amyloidosis...
- The systemic amyloidoses: clearer understanding of the molecular mechanisms offers hope for more effective therapiesG Merlini
Department of Biochemistry, Biotechnology Research Laboratory, Amyloid Center, University Hospital IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy
J Intern Med 255:159-78. 2004..The elucidation of the molecular mechanisms involved in amyloidogenesis is at the basis of the recent blossoming of new, innovative and more effective therapeutic approaches...
- Multicentre versus single centre approach to rare diseases: the model of systemic light chain amyloidosisGiovanni 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...
- First report of systemic reactive (AA) amyloidosis in a patient with the hyperimmunoglobulinemia D with periodic fever syndromeLaura 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...
- Prognostic factors in symptomatic Waldenstrom's macroglobulinemiaGiampaolo 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...
- Conformational switching and fibrillogenesis in the amyloidogenic fragment of apolipoprotein a-IAlessia Andreola
Department of Biochemistry and Centro Interdipartimentale di Biologia Applicata, University of Pavia, via Taramelli 3b, Italy
J Biol Chem 278:2444-51. 2003....
- Structure, function and amyloidogenic propensity of apolipoprotein A-ILaura 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....
- Long-term results of a risk-adapted approach to melphalan conditioning in autologous peripheral blood stem cell transplantation for primary (AL) amyloidosisVittorio 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...
- The workings of the amyloid diseasesVittorio 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...
- Clinical aspects of systemic amyloid diseasesLaura Obici
Amyloid Center, Biotechnology Research Laboratories, IRCCS Policlinico San Matteo, Pavia, Italy
Biochim Biophys Acta 1753:11-22. 2005....
- Proteomics of beta2-microglobulin amyloid fibrilsMonica 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...
- Lysine 58-cleaved beta2-microglobulin is not detectable by 2D electrophoresis in ex vivo amyloid fibrils of two patients affected by dialysis-related amyloidosisSofia 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...
- Analysis of V(lambda)-J(lambda) expression in plasma cells from primary (AL) amyloidosis and normal bone marrow identifies 3r (lambdaIII) as a new amyloid-associated germline gene segmentVittorio Perfetti
Internal Medicine and Medical Oncology, Department of Internal Medicine, University of Pavia, IRCCS Policlinico S Matteo, Pavia, Italy
Blood 100:948-53. 2002..Overusage of just 2 gene segments, 3r and 6a, can thus account for the lambda light-chain overrepresentation typical of this disorder...
- Analysis of hepatitis C virus hypervariable region 1 sequence from cryoglobulinemic patients and associated controlsGabriella 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...
- Proteomics in protein misfolding diseasesMonica 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...
- Clinical, radiological, and biochemical features of a bilateral buttock amyloidoma emerging after 27 years of hemodialysisGiovanni Montagna
IRCCS Fondazione Salvatore Maugeri, Pavia, Italy
Amyloid 16:115-21. 2009....
- Lysozyme: a paradigmatic molecule for the investigation of protein structure, function and misfoldingGiampaolo 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...
- Insights into the regulation of immunoglobulin light chain gene rearrangements via analysis of the kappa light chain locus in lambda myelomaVittorio 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...
- Doxorubicin and congo red effectiveness on prion infectivity in golden Syrian hamsterManuel Corato
Laboratory of Experimental Neurobiology, Neurological Institute IRCCS C Mondino, Pavia, Italy
Anticancer Res 29:2507-12. 2009..05). Doxorubicin and Congo Red effects do not depend upon interaction with PrP amyloid material...
- Identification of amyloidogenic light chains requires the combination of serum-free light chain assay with immunofixation of serum and urineGiovanni 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....
- Obstructive intramural coronary amyloidosis: a distinct phenotype of cardiac amyloidosis that can cause acute heart failureMichele Pasotti
Department of Cardiology, I R C C S Policlinico San Matteo, Pavia, Italy
Eur Heart J 27:1810. 2006
- Characterization of immunoglobulin variable regions of two human pathogenic monoclonal cryocrystalglobulinsValentina 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...
- Molecular and functional bases of self-antigen recognition in long-term persistent melanocyte-specific CD8+ T cells in one vitiligo patientStefania Mantovani
Experimental Immunology Laboratory, IRCCS Maugeri Foundation, Pavia, Italy
J Invest Dermatol 121:308-14. 2003..These data, together with the skin-homing potential of the clones, directly point to the in vivo pathogenic role of melanocyte-specific cytotoxic T lymphocytes in vitiligo...
- Amyloidogenic and associated proteins in systemic amyloidosis proteome of adipose tissueFrancesca Lavatelli
Amyloid Treatment and Research Program, Boston, Massachusetts, USA
Mol Cell Proteomics 7:1570-83. 2008....
- Gender-related risk of myocardial involvement in systemic amyloidosisClaudio Rapezzi
Institute of Cardiology, University of Bologna, Bologna, Italy
Amyloid 15:40-8. 2008..In conclusion, these findings raise the hypothesis that some biological characteristic associated with female gender protects against myocardial involvement in familial ATTR...
- Genetic microheterogeneity of human transthyretin detected by IEFKlaus 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...
- Eprodisate for the treatment of renal disease in AA amyloidosisLaura M Dember
Boston University School of Medicine, Boston, MA 02118, USA
N Engl J Med 356:2349-60. 2007....
- Bortezomib in the treatment of AL amyloidosis: targeted therapy?Roberto Sitia
Haematologica 92:1302-7. 2007
- Treatment with oral melphalan plus dexamethasone produces long-term remissions in AL amyloidosisGiovanni Palladini
Blood 110:787-8. 2007
- How we treat Waldenström's macroglobulinemiaMeletios A Dimopoulos
Department of Clinical Therapeutics, University of Athens School of Medicine, Greece
Haematologica 90:117-25. 2005....
- Autoantibody activity in Waldenstrom's macroglobulinemiaMarvin J Stone
Baylor Charles A Sammons Cancer Center, 3535 Worth Street, Dallas, TX 75246, USA
Clin Lymphoma 5:225-9. 2005..Study of the antigens reacting with monoclonal IgMs may provide further insight into the pathogenesis of WM...
- Diagnostic performance of amyloid A protein quantification in fat tissue of patients with clinical AA amyloidosisBouke P C Hazenberg
Department of Rheumatology and Clinical Immunology, University Medical Center Groningen, University of Groningen, 9700 RB Groningen, The Netherlands
Amyloid 14:133-40. 2007..Amyloid A protein quantification in fat tissue is a new immunochemical method for detecting AA amyloidosis, a rare but serious disease. The objective was to assess diagnostic performance in clinical AA amyloidosis...
- Hereditary amyloidosisGiovanni Palladini
N Engl J Med 347:1206-7; author reply 1206-7. 2002
- Prognostic markers and criteria to initiate therapy in Waldenstrom's macroglobulinemia: consensus panel recommendations from the Second International Workshop on Waldenstrom's MacroglobulinemiaRobert A Kyle
Mayo Clinic, Rochester, MN, USA
Semin Oncol 30:116-20. 2003..However, the panel felt that current data are inadequate to support the use of any prognostic marker to select the timing and type of therapy, and called for studies on the application of prognostic markers in WM...
- Update on recommendations for assessing response from the Third International Workshop on Waldenstrom's MacroglobulinemiaEva Kimby
Karolinska University Hospital, Stockholm, Sweden
Clin Lymphoma Myeloma 6:380-3. 2006..The new criteria should therefore help in better delineating responses to therapy in patients with WM, particularly with the wide use of nucleoside analogues and biologically based agents for this disease...
- Circulating amyloidogenic free light chains and serum N-terminal natriuretic peptide type B decrease simultaneously in association with improvement of survival in ALGiovanni 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...
- Guidelines for the analysis of Bence Jones proteinMariastella Graziani
Clinical Chemistry Laboratory, Ospedale Civile Maggiore, Verona, Italy
Clin Chem Lab Med 41:338-46. 2003..The physiology of immunoglobulins and the clinical relevance of BJP are illustrated in the two appendices to this paper...
- IX International Symposium on Amyloidosis, July 15-21, 2001, Budapest, HungaryJean D Sipe
Tissue Engineering Study Section, Center for Scientific Review, National Institutes of Health, Bethesda, MD 20892 7814, USA
Amyloid 9:52-65. 2002
- Amyloid fibril protein nomenclature -- 2002Per Westermark
Amyloid 9:197-200. 2002
- A multicenter phase II trial of 4'-iodo-4'deoxydoxorubicin (IDOX) in primary amyloidosis (AL)Morie A Gertz
Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, MN 55905, USA
Amyloid 9:24-30. 2002..4'-Iodo-4'-deoxydoxorubicin (IDOX) has been reported to bind to and lead to the catabolism of amyloid deposits. A multicenter study attempted to develop a dosing schedule to confirm those results...
- Biological variation of N-terminal pro-brain natriuretic peptide in healthy individualsGianvico Melzi d'Eril
Clin Chem 49:1554-5. 2003
- 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....
- Immunoglobulin M monoclonal gammopathies of undetermined significance and indolent Waldenstrom's macroglobulinemia recognize the same determinants of evolution into symptomatic lymphoid disorders: proposal for a common prognostic scoring systemLuca Baldini
Unità Operativa Ematologia 1, Dipartimento di Ematologia e Oncologia, Ospedale Maggiore, I R C C S, Milano, Italy
J Clin Oncol 23:4662-8. 2005....
- 4'-iodo-4'-deoxydoxorubicin and tetracyclines disrupt transthyretin amyloid fibrils in vitro producing noncytotoxic species: screening for TTR fibril disruptersIsabel Cardoso
Amyloid Unit, Institute for Molecular and Cell Biology, University of Porto, Portugal
FASEB J 17:803-9. 2003....
- Ultrastructural organization of ex vivo amyloid fibrils formed by the apolipoprotein A-I Leu174Ser variant: an atomic force microscopy studyAnnalisa 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...
- Clinical indications for plasma protein assays: transthyretin (prealbumin) in inflammation and malnutritionA Myron Johnson
Department of Pediatrics, University of North Carolina School of Medicine, Chapel Hill, NC 27410, USA
Clin Chem Lab Med 45:419-26. 2007..In addition to these caveats, assays for TTR have a relatively high level of uncertainty ("imprecision"). Clinical evaluation--history and physical examination--should remain the mainstay of nutritional assessment...
- Update on treatment recommendations from the Third International Workshop on Waldenstrom's macroglobulinemiaSteven P Treon
Bing Program for Waldenstrom s Macroglobulinemia, Dana Farber Cancer Institute LG102, 44 Binney Street, Boston, MA 02115, USA
Blood 107:3442-6. 2006....
- Renal apolipoprotein A-I amyloidosis: a rare and usually ignored cause of hereditary tubulointerstitial nephritisGina Gregorini
Division of Nephrology, Spedali Civili, Brescia, Italy
J Am Soc Nephrol 16:3680-6. 2005..Patients who present with familial tubulointerstitial nephritis associated with liver disease require a high index of suspicion for apolipoprotein A-I amyloidosis...
- Amyloid: toward terminology clarification. Report from the Nomenclature Committee of the International Society of AmyloidosisPer 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...
- 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 2004Morie 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...
- A primer of amyloid nomenclaturePer 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...
- Autologous stem cell and kidney transplantation for primary amyloidosis associated with ESRD: which should come first?Giampaolo Merlini
Am J Transplant 5:1585-6. 2005
- Beta2-microglobulin isoforms display an heterogeneous affinity for type I collagenSofia Giorgetti
Dipartimento di Biochimica, Universita degli Studi di Pavia, via Taramelli 3 b 27100 Pavia, Italy
Protein Sci 14:696-702. 2005....
- The controlling roles of Trp60 and Trp95 in beta2-microglobulin function, folding and amyloid aggregation propertiesGennaro Esposito
Department of Biomedical Science and Technology, University of Udine, Piazzale Kolbe 4, 33100 Udine, Italy
J Mol Biol 378:887-97. 2008....
- 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...