Amyloid and Amyloidosis

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ISBN: 9780849335341 | 0849335345
Cover: Hardcover
Copyright: 11/29/2004

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This authoritative volume contains 179 chapters by international experts on recent developments in our understanding of amyloid proteins, protein folding disorders, and new and proposed clinical trials in amyloidosis. Topics include detection and characterization techniques; biological functions; genetics; disorders, diagnosis, and treatments, including organ transplants and drug therapies; results from extensive clinical studies; and epidemiology. This is a valuable resource for clinicians who care for patients with systemic and localized forms of amyloidosis, and for researchers in biochemistry, neurobiology, and cell biology.

Preface

Credits

vii

SECTION 1 AMYLOID FIBRILLOGENESIS

In silico to in vitro approach for protein folding and misfolding

(3)

GILTS D., DEHOUCK Y., ROOMAN M.

Amyloidosis in vivo: from molecular interactions to therapeutic targets

(3)

KISILEVSKY R.

Biophysical characterization of misfolded oligomeric protein states during the very early stages of amyloid formation

(3)

MODLER A., GAST K.

Status of Congo red stained amyloid in polarized light

(3)

KAHR B., KAMINSKY W., CLABORN K., KURIMOTO M., JIN L.-W.

Analysis of the interactions between amyloid fibrils and lipid components

(3)

GELLERMANN G.P., NIEKRASZ E., GÜHRS K., SHTRASBURG S., PRAS M., LINKE R.P., FÄNDRICH M., DIEKMANN S., APPEL T.R.

Disaggregation experiments as a tool to detect protofibrillar intermediates

(3)

CALAMAI M., CANALE C., RELINI A., STEFANI M., CHITI F., DOBSON C.

Correlation between stability and propensity to form amyloid-like fibrils

(3)

ŽEROVNIK E., KENIG M., RABZELJ S., TURK V.

Linearly arranged OH-groups in amyloid-deposits and in tissue isolated amyloid fibrils

(3)

MAKOVITZKY J.

Tissue detection and characterization of amyloid - is proteomics the answer?

(3)

PICKEN M., THULASIRAMAN V., PICKEN RN., LOMAS L.

The conformation of amyloid β-peptide in the presence of fluorinated and alkylated nanoparticles

(5)

ROCHA S., THUNEMANN A.F., PEREIRA MC., COELHO MAN., MÖHWALD H., BREZESINSKI G.,

SECTION 2 AL AMYLOIDOSIS

Systemic equine AL amyloidosis

(2)

MURPHY C.L., CROMBIE B., WILLIAMS T.K., MACY S.D., WEISS D.T., SOLOMON A., NOUT Y.S

Radioimaging of primary (AL) amyloidosis with an amyloid-reactive monoclonal antibody

(3)

WALL J.S., PAULUS M.J., KENNEL S.J., GLEASON S., BABA J., GREGOR J., WOLFENBARGER D., WEISS D.T., SOLOMON A.

Primary amyloidosis: conformational intermediates in immunoglobulin light chain proteins upon interaction with Congo red under physiological conditions

(3)

WALSH M.T., ROSKENS V., CONNORS L., SKINNER M.

Immunoglobulin light chain variable region genes demonstrate evidence for antigen selection in primary amyloidosis

(3)

PROKAEVA T., WARD J.E., FINGAR E., KAUT M., SMITH P., O'HARA C., CONNORS L.H., SKINNER M., SELDIN D.C.

Detection and characterization of post-translational modifications in AL light chain proteins by mass spectrometry

(3)

THÉBERGE R., LIM A., JIANG Y., CONNORS L.H., EBERHARD J., SKINNER M., COSTELLO C.E.

Glycosylation studies of monoclonal immunoglobulin deposition disorders

(3)

OMTVEDT L.A., ROYLE L., HUSBY G., SLETTEN K., RADCLIFFE C., HARVEY D.J., DWEK R.A., RUDD P.M.

Structural localization of light chain amyloidogenicity

(3)

WALL J.S., MURPHY C.L., TOMAZEWSKI A., CROMBIE B., SOLOMON A.

Aggressive light chain amyloidosis cases have unstable immunoglobulin light chains

(3)

SIKKINK M., BADEN E.M., VANCE SR., RILEY K.J.-L., MOCH A.T., RAMIREZ-ALVARADO M.

Molecular classification of light chain (AL) amyloidosis by gene expression profiling

(3)

ABRAHAM R.S., MANSKE M.K., BALLMAN K.V., DISPENZIERI A., PRICE-TROSKA T.L., GERTZ M.A., FONSECA R.

Analysis of B cell clonal evolution in BM and PBL of AL and MM patients using graphical quantification of lineage trees

(3)

ABRAHAM R.S., MANSKE M.K., SOHNI A., EDELMAN H., ZUCKERMAN N., SHAHAF G., DISPENZIERI A., LACY MQ., GERTZ M.A., MEHR R.

In vitro and in vivo mouse models for AL amyloidosis

(3)

WARD J.E., BRENNER D.A., EBERHARD J., LIAO R., CONNORS L.H., O'HARA C., SELDIN D.C.

Serum cardiac troponins and N-terminal pro-brain natriuretic peptide: a staging system for primary systemic amyloidosis

(3)

DISPENZIERI A., KYLE R.A., LACY M.Q., JAFFE A.S., THERNEAU T.M., ZELDENRUST S.R., GERTZ M.A.

Amyloid-induced myocardial dysfunction depends on biochemical characteristics of the amyloidogenic protein

(3)

PALLADINI G., SARAIVA M.J., PERLINI S., OBICI L., VEZZOLI M., COELHO T., REIS H., LAVATELLI F., VADACCA G., MORATTI R., MERLINI G.

The reduction of the serum concentration of the amyloidogenic light-chain in cardiac AL results in prompt improvement of myocardial function and prolonged survival despite unaltered amount of myocardial amyloid deposits

(3)

PALLADINI G., PERLINI S., VEZZOLI M., PERFETTI V., LAVATELLI F., FERRERO I., OBICI L., CACCIALANZA R., BRADWELL A.R., MERLINI G.

Organ survival in AL amyloidosis with renal involvement

(3)

PALLADINI G., PERFETTI V., OBICI L., CACCIALANZA R., LAVATELLI F, MERLINI G.

Nutritional status of outpatients with AL systemic amyloidosis

(3)

CACCIALANZA R., PALLADINI G., CENA H., VAGIA C., ROGGI C., MERLINI G.

Congo red fluorescence circumvents pitfalls in morphometric quantitation of amyloid in tissue sections

(3)

DONINI U., LINKE R.P.

Cardiac troponins and N-terminal pro-brain natriuretic peptide predict survival in patients with primary systemic amyloidosis undergoing peripheral blood stem cell transplant

(3)

DISPENZIERI A., KYLE R.A., LACY M.Q., JAFFE A.S., THERNEAU T.M, BURRIT M.F., GERTZ MA.

BNP as a marker of cardiac involvement in amyloidosis

(2)

NORDLINGER M., MAGNANI B.J., SKINNER M., FALK R.H.

Quantitative serum free light-chain assay in the diagnostic evaluation of AL amyloidosis

(4)

AKAR H., SELDIN D.C., MAGNANI B., O'HARA C., BERK J.L., SCHOONMAKER C., CABRAL H., DEMBER L.M., SANCHORAWALA V., CONNORS L.H., SKINNER M.

AL amyloidosis-associated hypothyroidism: prevalence and relationship to nephrotic syndrome

(2)

BHATIA J.S., SANCHORAWALA V., SELDIN D.C., CASSERLY L.F., BERK J.L., FALK R., SKINNER M., DEMBER L.M.

Renal involvement in systemic amyloidosis: report of an Italian retrospective study

(3)

BERGESIO F, CICIANI A.M., PALLADINI G., BRUGNANO R., BIZZARRI D., PASQUALI S., SALVADORI M.

Biochemical characterization of an immunoglobulin lambda I light chain amyloid protein isolated from formalin-fixed paraffin-embedded tissue sections

(3)

YAZAKI M., LIEPNIEKS J.J., BENSON M.D.

Case report: unusual clinical presentation of primary AL amyloidosis

102

(3)

CHIAPPINI MG., DI GIROLAMO M., SELVAGGI G., GIANNAKAKIS K.

Serum levels of free kappa and lambda light chains in patients with systemic AL, AA, and ATTR amyloidosis

105

(2)

VAN STEIJN J., BIJZET J., DE WIT H., HAZENBERG B.P.C., VAN GAMEREN I.I., VAN DE BELT K., VELLENGA E.

Diagnostic value of free kappa and lambda light chains in fat tissue of patients with systemic AL amyloidosis

107

(2)

HAZENBERG B.P.C., BIJZET J., DE WIT H., VAN STEIJN J., VELLENGA E., VAN RIJSWIJK M.H.

Experience with a patient-adapted approach to autologous peripheral blood stem cell transplantation in AL amyloidosis

109

(3)

PERFETTI V., PALLADINI G., SIENA S., BREGNI M., DI NICOLA M., NAVAZZA V., MAGNI M., GIANNI AM., MERLINI G.

Risk-adapted intravenous melphalan with adjuvant thalidomide and dexamethasone for newly diagnosed untreated patients with systemic AL amyloidosis: interim report of a phase II trial

112

(3)

COMENZO R.L., ZHOU P., REICH L., COSTELLO S., QUINN A., FIRCANIS S., DRAKE L., tERUYA-FELDSTEIN J., FILIPPA D., FLEISHER M.

Thalidomide plus intermediate-dose dexamethasone in refractory/relapsed AL patients

115

(3)

PALLADINI G., PERFETTI V., PERLINI S., OBICI L., LAVATELLI F., INVERNIZZI R., BERGESIO F., COMOTTI B., IANNACCONE S., MERLINI G.

VAD chemotherapy followed by autologous stem cell transplantation (ASCT) for treatment of nephrotic syndrome in light chain (AL) amyloidosis

118

(3)

PERZ J.B., SCHOENLAND S.O., LINKE R.P., ZEIER M., HUNDEMER M., HO AD., GOLDSCHMIDT H.

Superior survival in primary systemic amyloidosis patients undergoing peripheral blood stem cell transplant: a case control study

121

(3)

DISPENZIERI A., KYLE R.A., LACY M.Q., THERNEAU T.M., GERTZ M.A.

Treatment of AL amyloidosis with tandem cycles of high dose melphalan and autologous stem cell transplantation

124

(3)

SANCHORAWALA V., WRIGHT D.G., QUILLEN K., KUNZ R., FINN K.T., MALEK K., FALK R.H., BERK J., DEMBER L.M., SKINNER M., SELDIN D.C.

The use of sequential living donor kidney and autologous stem cell transplantation for patients with primary (AL) amyloidosis and advanced renal failure

127

(3)

LEUNG N., GRIFFIN M.D., DISPENZIERI A., GLOOR J.M., SCHWAB T.R., TEXTOR S.C., LACY MQ., LARSON T.S., STEGALL M.D., GERTZ M.A.

Delayed resolution of nephrotic syndrome following stem cell transplant for amyloidosis (AL)

130

(3)

GERTZ M., LEUNG N., LACY M., DISPENZIERI A.

Amyloid versus non-amyloid immunoglobulin deposits

133

(3)

MERLINI G.

The role of matrix metalloproteinases (MMPs) and tissue inhibitors of metalloproteinases (TIMPs) in mesangial matrix replacement in AL-amyloidosis: An in vivo and in vitro correlative study

136

(3)

KEELING J., DEMPSEY S., JOSEPH L., TURBAT-HERRERA E.A., HERRERA GA.

Pathogenesis of renal AL-amyloidogenesis (AL-Am): an in vitro approach to understanding mechanisms involved

139

(3)

HERRERA G.A., KEELING J., TENG J.

Internalization of systemic amyloid light chains and heparan sulfate proteoglycan deposition

142

(3)

TRINKAUS-RANDALL V., STEEVES S., MONIS G., WALSH MT., CHIU J.D., EBERHARD J., CONNORS L.H., SKINNER M.

Human amyloidogenic light chains directly invoke oxidant stress and result in impaired cardiomyocyte function

145

(3)

BRENNER D.A., JAIN M., PIMENTEL DR., WANG B., CONNORS L.H., SKINNER M., APSTEIN CS., LIAO R.

Management of primary amyloidosis (AL)

148

(3)

GERTZ M.

Definition of organ involvement and treatment response in primary systemic amyloidosis (AL): A consensus opinion from the 10th International Symposium on Amyloid and Amyloidosis

151

(3)

GERTZ M.A., COMENZO R., FALK RH., FERMAND J.P., HAZENBERG B.P., HAWKINS P.N., MERLINI G., MOREAU P., RONCO P., SANCHORAWALA V., SEZER O., SOLOMON A., GRATEAU G.

Measurement of serum free light chains in AL amyloidosis

154

(3)

CARR-SMITH H.D., ABRAHAM R., MEAD GP., GOODMAN H., HAWKINS P., BRADWELL A.R.

Melphalan plus high-dose dexamethasone in AL patients who are not candidates for stem cell transplantation

157

(3)

PALLADINI G., PERFETTI V., OBICI L., LAVATELLI F., CACCIALANZA R., ADAMI F, CAVALLERO G., RUSTICHELLI R., VIRGA G., MERLINI G.

High dose melphalan and autologous stem cell transplantation (HDMJSCT) for treatment of AL amyloidosis: ten year single institutional experience

160

(3)

SELDIN D.C., SANCHORAWALA V., MALEK K., WRIGHT D.G., QUILLEN K., FINN K.T., FALK R., BERK J., DEMBER L., WIESMAN J., ANDERSON J.J., SKINNER M.

Thalidomide treatment in 80 patients with AL amyloidosis: tolerability, clonal disease response and clinical outcome

163

(1)

GOODMAN H.J.B., LACHMANN H.J., GALLIMORE R., HAWKINS P.N.

High dose therapy with autologous hematopoietic stem cell transplantation (HSCT) for patients with primary systemic amyloidosis (AL): An ABMTR Study

164

(3)

VESOLE D.H., PEREZ W., REECE D., AKASHEH M., HOROWITZ M., BREDESON C.

Prospective evaluation of the utility of the serum free light chain assay (FLC), clonal Ig VL gene identification and troponin I levels in a phase II trial of risk-adapted intravenous melphalan with adjuvant thalidomide and dexamethasone for newly diagnosed untreated patients with systemic AL amyloidosis

167

(6)

COMENZO R.L., ZHOU P., REICH L., COSTELLO S., QUINN A., FIRCANIS S., DRAKE L., HEDVAT C., TERUYA-FELDSTEIN J., FILIPPA D.A., FLEISHER M.

SECTION 3 AA AMYLOIDOSIS

Characteristic and clinical outcome of 340 patients with systemic AA amyloidosis

173

(3)

LACHMANN H.J., GOODMAN H.J.B., GALLIMORE J., GILBERTSON J.A., JOSHI J., PEPYS MB., HAWKINS P.N.

SAA1 gene analysis in Finnish patients with AA amyloidosis

176

(3)

TERAI C., KANEKO H., MORIGUCHI M., KOSEKI Y., KAJIYAMA H., KAMATANI N., TIITINEN S., KAARELA K., HURME M., MAURY C.P.J.

A prospective analysis of demography, etiology, and clinical findings of AA amyloidosis patients enrolled in the international clinical Phase II/III Fibrillex™ study

179

(3)

HAUCK W., DEMBER L.M., HAWKINS P.N., HAZENBERG B.P.C., SKINNER M., BOUWMEESTER M.-C., BRIAND R., CHICOINE E., GURBINDO C., HUGHES L., GARCEAU D., FAST GROUP

TNFRSF1A mutations in Italian patients affected by apparently sporadic periodic fever syndrome

182

(3)

OBICI L., MARCIANO S., PALLADINI G., LAVATELLI F., DONADEI S., CIGNI A., SATTA A.E., PRADERIO L., TRESOLDI M., MERLINI G.

AA-Amyloidogenesis replicated in cell culture: a serum amyloid A peptide inhibitor provides direct evidence for the role of heparan sulfate

185

(3)

ELIMOVA E., KISILEVSKY R., SZAREK WA., ANCSIN J.B.

An EM autoradiography and immunofluorescence study examining the pathway of serum amyloid A through the macrophage

188

(3)

KINKLEY S., KISILEVSKY R.

Cathepsin B generates nine different AA amyloid proteins by its carboxypeptidase activity

191

(3)

RÖCKEN C., MENARD R., BÜHLING F., RAYNES J., KÄHNE F.T.

A serum amyloid A peptide contains a low-pH heparin binding site which promotes AA-amyloidogenesis in cell culture

194

(3)

ELIMOVA E., KISILEVSKY R., ANCSIN J.B.

Mapping the surface residues on murine HDL-associated serum amyloid A reveals significant differences between the two major isoforms

197

(3)

ELIMOVA E., KISILEVSKY R., ANCSIN J.B.

Analyses of the amyloid fibrils in bovine amyloidosis in Hokkaido district, Japan

200

(3)

UEDA M., ANDO Y., HARAOKA K., MATSUI T., TAKAMUNE N., SAITO S., NAKAMURA M., TERAZAKI H., YAMASHITA T., XUGUO S., KIM J., TATEISHI T., SHOJI S., HOSHII Y., ISHIHARA T., OTAGIN M., UCHINO M.

Serum amyloid A (SAA) in mammary tissues with inflammatory processes and in mammary corpora amylacea

203

(3)

TOUSSAINT M.J.M., HOGARTH C., NGUYEN T.KA., LOEB E., BALCIUTE J., VIVANCO V., VAN EDEREN A.M., JACOBSEN S., NIEWOLD T.A., GRUYS E.,

Chemical typing of porcine systemic amyloid as AA-amyloid

206

(3)

NIEWOLD T.A., MURPHY C.L., TOUSSAINT M.J.M., GRUYS E.

Serum amyloid P component in mink

209

(3)

OMTVEDT L.A., WIEN T.N., MYRAN T., SLETTEN K., HUSBY G.

Acute-phase serum amyloid A (A-SAA) synthesized by human synovial cells (SC) induces SC migration

212

(3)

KUMON Y., ARII K., SUEHIRO T., IKEDA Y., ENZAN H., HASHIMOTO K.

Serum amyloid a (SAA) induces cytokine production in human mast cells

215

(3)

NIEMI K., BAUMANN M.H., EKLUND K.K.

The occurrence of Protein-AA specific antibodies in experimentally induced murine reactive amyloidosis

218

(3)

NYSTRÖM S., WESTERMARK G.T.

Agrin is a component of AA amyloid

221

(2)

WIEN T.N., SØRBY R., LANDSVERK T., HUSBY G.

Autofluorescence of AA amyloid

223

(2)

WIEN T.N., SØRBY R., LANDSVERK T., HUSBY G.

Fibrillar proteins occurring in the nature can induce AA amyloidosis in mice

225

(3)

LUNDMARK K., WESTERMARK G.T., OLSÉN A., WESTERMARK P.

Amyloid production by human monocyte cultures

228

(3)

KLUVE-BECKERMAN B., MAGY N., LIEPNIEKS J.J., BENSON M.D.

Experimental amyloidosis induced by Freund's adjuvant and casein in two mouse strains

231

(4)

BRADUNAITE R., POVILENAITE D., LEONAVICIENE L., VAITKIENE D., VENALIS A.

Fibrin and ubiquitin enhance the development of casein-induced experimental AA amyloidosis in C57BL/6 mice

235

(3)

LEONAVICIENE L., POVILENAITE D., BRADUNAITE R., VAITKIENE D., VENALIS A.

Lower propensity for amyloidogenesis in female mice

238

(3)

SHTRASBURG S., PRAS M., DULITZKY M., PARIENTE C., MOR A., GAL R., LIVNEH A.

In vivo influence of serum amyloid A 2.1 and its active domains on macrophage cholesterol export

241

(3)

TAM S-P., KISILEVSKY A.

Influence of murine serum amyloid A 2.1, and its active domains, on human macrophage cholesterol export in cell culture

244

(3)

TAM S.-P., KISILEVSKY R.

Systemic AA amyloidosis in the common marmoset

247

(3)

LUDLAGE E., MURPHY CL., DAVERN SM., SOLOMON A., WEISS D.T., GLENN-SMITH D., DWORKIN S., MANSFIELD K.G.

Inhibitory effect of monensin on murine AA amyloidosis

250

(3)

GONDO T., CUI D., HOSHII Y., KAWANO H., TAKAHASHI M., IWATA T., ISHIHARA T.

Amyloid-determining residues in mouse serum amyloid A

253

(3)

KLUVE-BECKERMAN B., MARKS D., WANG L., BENSON M.D., LIEPNIEKS J.J.

Hydrocortisone suppresses the second phase of amyloidogenesis in a mouse model

256

(2)

SHTRASBURG S., PRAS M., PARIENTE C., GAL R., LIVNEH A.

Targeting apolipoprotein E fragments in amyloid deposit; a study on a mouse model

258

(2)

SOMEYA T., YAMADA T., KOBAYASHI M., FUJITA S.

Scintigraphy at different time intervals after administration of ¹²³I labelled serum amyloid P component (SAP) in patients with amyloidosis

260

(3)

HAZENBERG B.P.C., JAGER P.L., PIERS D.A., LIMBURG P.C., DE HOOGE M.N., VAN RIJSWIJK M.H.

The incidence and polarization optical characteristics of pulmonary alveolar calcospherities (corpora amylacea) in rheumatoid arthritis - A retrospective clinicopathologic study of 210 autopsy patients

263

(3)

BÉLY M., APÁTHY Á.

Patient information in AA amyloidosis - what the patients want

266

(3)

HUNTER J.A., ELLISON E., FRANCKS A., SWANN R., GUPTA M., HANIF N.

Advanced glycation end products and advanced oxidation protein production in patients with AA amyloidosis

269

(3)

RYSAVÁ R., KALOUSOVÁ M., ZIMA T., DOSTÁL C., MERTA M., TESAR V.

Etanercept in the treatment of AA amyloidosis in juvenile rheumatoid arthritis - preliminary experience

272

(3)

MICHELS R., HÄFNER R., LINKE R.P.

Epidemiologic description of amyloidosis diagnosed in University Hospital of Rennes from 1995 to 1999

275

(3)

CAZALETS C., CADOR B., MAUDUIT N., DECAUX O., RAMÉE M.P., LE POGAMP P., LAURENT M., JEGO P., GROSBOIS B.

A heterozygotic Japanese patient with familial Mediterranean fever (FMF) Pyrin M694I

278

(3)

TERAZAKI H., OKUDA A., KATASE K., NAKAMURA M., UEDA M., KIM J.M., HARAOKA K., ANDO Y., SASAKI Y.

Comparison of clinical and immunohistochemical findings in patients with rheumatoid arthritis and secondary amyloidosis

281

(3)

POVILENAITE D., GRAZIENE V., BUTRIMIENE I., VENALIS A.

Renal glomerular AL and vascular AA amyloidosis in a patient with ankylosing spondylitis

284

(3)

PETTERSSON T., ANTTILA P., TÖRNROTH T.

Fat tissue analysis by Congo red method and by amyloid A protein quantification in clinical AA amyloidosis

287

(2)

HAZENBERG B.P.C., BIJZET J., LIMBURG P.C., VAN RIJSWIJK M.H., GARCEAU D., FAST GROUP

Anemia of chronic disease in patients with AA-amyloidosis: Relationship to polymorphisms of the interleukin-1 gene complex and circulating cytokine levels

289

(3)

MAURY C.P.J., LILJESTRÖM M., LAIHO K., TIITINEN S., KAARELA K., HURME M.

Influence of inflammation on lipid metabolism in the patients with rheumatoid arthritis

292

(5)

KOSEKI Y., TERAI C., KAJIYAMA H., ITO M., YAMANAKA H., HARA M., TOMATSU T., KAMATANI N.

SECTION 4 FAMILIAL AMYLOIDOSIS

Molecular and cellular aspects in transthyretin amyloidosis

297

(3)

SARAIVA M.J.

Genotype-phenotype correlations and management in transthyretin (TTR) related amyloidosis

300

(3)

ANDO Y.

The binding of diethylstilbestrol to transthyretin - a crystallographic model

303

(3)

MORAIS-DE-SÁ E., PEREIRA P.J.B., SARAIVA M.J., DAMAS A.M.

Liver transplantation in ATTR Met30 and other variants: time to shift the paradigm?

306

(3)

STANGOU AJ., HEATON N.D., RELA M., O'GRADY J., MATHIAS C.J., GOODMAN H.J.B., LACHMANN H.J., BYBEE A., ROWZCENIO D., JOSHI J., WILLIAMS R.S., PEPYS M.B., HAWKINS P.N.

Non-TTR hereditary amyloidosis

309

(3)

BENSON M.D.

Fibrinogen A α-chain amyloidosis: clinical features and outcome after hepatorenal or solitary kidney transplantation

312

(3)

STANGOU A.J., LACHMANN H.J., GOODMAN H.J.B., BYBEE A., ROWZCENIO D., TENNENT G., BRENNAN S.O., O'GRADY J.G., HEATON N.D., RELA M., PEPYS M.B., HAWKINS P.N.

A novel complex indel mutation in the fibrinogen Aα chain gene in an Asian child with systemic amyloidosis

315

(1)

BYBEE A., KANG H.G., HA I.S., PARK M.S., CHEONG H.I., CHOI Y., GILBERTSON J.A., PEPYS M.B., HAWKINS P.N.

Phenotype, genotype and outcome in hereditary apoAI amyloidosis

316

(1)

HAWKINS P.N., BYBEE A., GOODMAN H.J.B., LACHMANN H.J., ROWCZENIO D., GILBERTSON J.A., O'GRADY J., HEATON N., STANGOU A., PEPYS M.B.

Pigmented ciliary epithelium cells synthesized transthyretin in the rabbit eye

317

(3)

KAWAJI T., ANDO Y., NAKAMURA M., YAMAMOTO K., ANDO E., FUKUSHIMA M., HIRATA A., TANIHARA H.

Upregulation of the extracellular matrix remodeling genes, biglycan, neutrophil gelatinase-associated lipocalin and matrix metalloproteinase-9 in familial amyloid polyneuropathy

320

(3)

SOUSA M.M., AMARAL J.B., GUIMARÃES A., SARAIVA M.J.

How important is the role of compact denatured states on amyloid formation by transthyretin?

323

(3)

RODRIGUES J.R., BRITO R.M.M.

The X-ray crystallographic structure of the amyloidogenic variant TTR Tyr78Phe

326

(3)

NETO-DA-SILVA R., PEREIRA P.J.B., SARAIVA M.J., DAMAS A.M.

An interactive strategy for the accurate characterization of transthyretin variants using combined proteomic and genomic technologies

329

(3)

ZELDENRUST S.R., BERGEN H.R. III, NEPOMUCENO A.I., BUTZ M.L., SNOW D.S., DYCK P.J., DYCK P.J.B., KLEIN C.J., O'BRIEN J.F., THIBODEAU SN., MUDDIMAN, D.C.

Codeposition of transthyretin and immunoglobulin lambda light chain in senile cardiac (ATTR) amyloidosis

332

(2)

LIEPNIEKS J.J., BENSON M.D.

Macroscopic assessment of skin venules in patients with familial amyloidotic polyneuropathy (FAP) using near-infrared spectrophotoscopy: as a marker of autonomic dysfunction

334

(3)

OBAYASHI K., ANDO Y., MIIDA T., NAKAMURA M., YAMASHITA T., UEDA M., HARAOKA K., TERAZAKI H., OKADA M.

Incidence of transthyretin VaL122Ile amyloid mutation in African-Americans born in Indianapolis, Indiana, USA

337

(2)

BENSON M.D., YAZAKI M., YAMASHITA T., HAMIDI ASL K.

An historical case of primary amyloidosis was re-diagnosed as ATTR (V30M)

339

(3)

LINKE R.P., SCHLOTE W., GERHARD L., WINTER P., ALTLAND K.

Echocardiographic abnormalities in transthyretin (TTR) VI22I allele carriers

342

(3)

TAGOE C.E., SCHWARTZBARD A., SHAH A., BUXBAUM J.N., SALMAN L., FELICIANO E., LABAT M., MEHRA D., MALENDOWICZ S., HANNA G., PIERRE S., STEDMAN MR., ROSWELL R., FIORE L.D., BROPHY M.T., GAGNON D., JACOBSON D.R.

Familial amyloidotic polyneuropathy with severe renal involvement in association with transthyretin Gly47Glu in Dutch, British and American families

345

(3)

HAAGSMA E.B., HAWKINS P.N., BENSON M. D., LACHMANN H.J., BYBEE A., HAZENBERG B.PC.

Clinical diagnosis of familial transthyretin amyloidosis by automated data acquisition and processing with quadrupole orthogonal time-of-flight mass spectrometry

348

(3)

MCCOMB M.E., LIM A., THÉBERGE R., PROKAEVA T., CONNORS L.H., SKINNER M., COSTELLO C.E.

Liver transplantation for familial amyloidotic polyneuropathy does not prevent disease progression in a majority of patients

351

(3)

ZELDENRUST S., PERRI R., BRANDHAGEN D., ROSEN C., POTERUCHA J., GERTZ M., WEISNER R., GORES G.

Continued deposition of wild-type transthyretin in cardiac amyloid after liver transplantation

354

(3)

LIEPNIEKS J.J., YAZAKI M., BENSON M.D.

Deposition and passage of transthyretin through the blood-nerve barrier in recipients of familial amyloid polyneuropathy livers

357

(3)

SOUSA M.M., FERRÃO J., FERNANDES R., GUIMARÃES A., GERALDES J.B., PERDIGOTO R., TOMÉ L., MOTA O., NEGRÃO L., FURTADO A.L., SARAIVA M.J.

Familial amyloidosis: recent novel and rare mutations in a clinical population

360

(3)

CONNORS L.H., PROKAEVA T., AKAR H., METAYER M., SMITH P., LIM A., THÉBERGE R., COSTELLO C.E., SKINNER M.

Renal apolipoprotein A-I amyloidosis (AAPO-A-1) associated with a novel mutant Leu64Pro

363

(3)

MURPHY C.L., WANG S., WEAVER K., GERTZ M.A., WEISS D.T., SOLOMON A.

Hereditary amyloidosis in a Spanish family associated with a novel non-stop mutation in the gene for apolipoprotein AII

366

(1)

ROWCZENIO D., GILBERTSON J.A., BYBEE A., HERNÁNDEZ D., HAWKINS P.N.

Hereditary renal amyloidosis in a German family associated with fibrinogen Aα chain Glu540Val

367

(1)

BYBEE A., HOLLENBECK M., DEBUSMAN E.R., GOPAU D., GILBERTSON J.A., LACHMANN H.J., PEPYS M.B., HAWKINS P.N.

Systemic ALys amyloidosis presenting with gastrointestinal bleeding

368

(5)

RÖCKEN C., BECKER K., STIX B., RATH T., KÄHNE T., FÄNDRICH M., ROESSNER A., ALBERT F.W.

SECTION 5 CENTRAL NERVOUS SYSTEM AMYLOIDOSIS

Presenilin-independent and JLK-sensitive g-secretase pathway: a hope for Alzheimer's disease therapy?

373

(3)

CHECLER F.

Prion diseases and the prion protein

376

(3)

AUCOUTURIER P., LEVAVASSEUR E., BALLERINI C., CARNAUD C.

Exploring the folding and aggregation mechanisms of amyloid-forming peptides by computer simulations

379

(3)

SANTINI S., WEI G.-H., MOUSSEAU N., DERREUMAUX P.

cAMP-specific Pde4B mediates Aβ-induced microglial activation

382

(3)

SEBASTIANI G., MORISSETTE C., LAGACÉ C., BOULÉ M., OUELLETTE M.J., MCLAUGHLIN R.W., GERVAIS F., TREMBLAY P.

Perspectives of immunotherapy against TSE

385

(3)

CARNAUD C., GREGOIRE S., CHATELET F., BRULEY-ROSSET M., AUCOUTURIER P.

Serpinopathies and the central nervous system

388

(3)

LOMAS D.A., BELORGEY D., MALLYA M., ONDA M., KINGHORN K.J., SHARP L.K., PHILLIPS R.L., PAGE R., MIRANDA E., CROWTHER D.C.

Hereditary cerebral amyloid angiopathy associated with ATTR Tyr114Cys

391

(2)

YAMASHITA T., ANDO Y., NAKAMURA M., HARAOKA K., XUGUO S., TERAZAKI H., UEDA M., IKEDA T., SAITO S., KAWAJI T., HIRAI T., WASHIMI Y., UCHINO M.

Distribution of amyloid peptide beta 1-40 and 1-42 in cerebral amyloid angiopathy and correlation with Alzheimer disease pathology

393

(3)

ATTEMS J., LINTNER F., JELLINGER K.

Hereditary cerebral amyloid angiopathy associated with a novel amyloid beta precursor protein mutation

396

(3)

OBICI L., DE ROSA G., PALLADINI G., MARCIANO S., DONADEI S., ARBUSTINI E., VERGA L., CONCARDI M., FERRARI G., MERLINI G.

Cognitive disturbances and brain pathology in old dogs

399

(3)

D-amino-acid peptide specific for cerebral Aβamyloid in man cross-reacts with canine cerebral amyloid

402

(3)

LINKE R.P., OOS R., ROFINA J.E., WIESEHAN K., WILLBOLD D., GRUYS E.

Inhibition of β-amyloid peptide-induced microglial cell activation by glycosaminoglycans

405

(3)

MORISSETTE C., GERVAIS F., TREMBLAY P.

Morphology and growth kinetics of Alzheimer's amyloid β-peptides, Aβ and the arctic mutation Aβ (E22G): in situ atomic force microscopy study

408

(3)

HELLBERG M., NORLIN N., ANTZUTKIN ON., ALMQVIST N.

Reversing the formation of Alzheimer amyloid fibrils and blocking toxic Ca²+ influx

411

(4)

INGRAM V.M., BLANCHARD B.J., CHEN A., STAFFORD K., STOCKWELL B.

SECTION 6 LOCALIZED AMYLOIDOSIS

β Microglobulin amyloidosis

415

(3)

ARGILES A., CHARNET A., SCHMITT-BERNARD C, GARCIA - GARCIA M., MÉJEAN C., MOURAD G.

Amyloid tumours of breast, lung and rib in nonsecretory multiple myeloma

418

(3)

RICHTER S., SAUTER G., JONAS L., GAFUMBEGETE E., MAKOVITZKY J.

The spectrum of localized amyloidosis: a case series of twenty patients

421

(3)

BIEWEND M.L., MENKE D.M., CALAMIA K.T.

Localized amyloidosis: about 36 cases

424

(2)

PACCALIN M., RUBI M., HACHULLA E., CARREIRO M., TRICOT L., WECHSLER B., CABANE J., CAZALET C., ROBLOT P.

Studies on unfolded β2-microglobulin at C-terminal 92 to 99 in dialysis related amyloidosis

426

(3)

MOTOMIYA Y., HARAOKA K., SUN X., MORITA H., ANDO Y.

Systemic β2-microglobulin-related amyloidosis in a patient with chronic renal failure, receiving long term hemodialysis for 3 years - a light and electron microscopic study

429

(3)

BÉLY M., LAKATOS T., APÁTHY Á.

An unusual tumor of amyloid in the corner of the eye: a case report with polarization optical and immunohistochemical analysis

432

(3)

MAKOVITZKY J., CHOTT A.

Kappa III immunoglobulin light chain origin of localized orbital amyloidosis

435

(3)

HAMIDI ASL K., LIEPNIEKS J.J., NUNERY W.R., YAZAKI M., BENSON M.D.

Corneal dystrophy caused by a novel mutation of the TGFBI gene: a case report

438

(2)

STIX B., RÜESCHOFF J., ROESSNER A., RÖCKEN C.

Free fatty acids promote amyloid formation without participating in the amyloid like fibril

440

(3)

WESTERMARK G.T.

Medullary thyroid carcinoma-associated amyloid is comprised of calcitonin gene-related peptide II

443

(3)

MURPHY CL., WANG S., CROMBIE B., MACY S.D., WILLIAMS T.K., WEISS DT., SOLOMON A.

Studies of the amyloidogenic properties of proIAPP

446

(3)

PAULSSON J.F., WESTERMARK G.T.

Laryngeal amyloidosis: localized versus systemic disease

449

(2)

BARTELS H., DIKKERS F.G., VAN DER WAL J.E., LOKHORST H.M., HAZENBERG B.P.C.

Formation of the vascular amyloid fibril protein medin from its precursor lactadherin

451

(3)

PENG S., PERSSON A., WASSBERG E., WESTERMARK P.

Effects of medin on smooth muscle cells

454

(3)

PERSSON A., PENG S., GERWINS P., FU X., WESTERMARK P.

Transmission of mouse AApoAII amyloidosis by the amyloid fibrils; inhibitory effects on transmission by the denaturation/degradation of amyloid fibrils

457

(3)

SAWASHITA J., ZHANG H., FU X., KORENAGA T., MORI M., HIGUCHI K.

Transmission of mouse AApoAII amyloidosis from mother to pups

460

(3)

KORENAGA K., FU X., MORI M., SAWASHITA J., NAIKI H., MATSUSHITA T., HIGUCHI K.

External beam radiation for tracheobronchial amyloidosis: the Boston University experience

463

(3)

BERK J.L., GRILLONE G.A., SKINNER M., HARTFORD A.C.

Wild type transthyretin-derived senile systemic amyloidosis with limited proteolytic digestion of the monomer: clinico-pathologic studies with DNA and amyloid protein characterization

466

(2)

PICKEN M.M., LEYA F., PICKEN R.N.

Pindborg tumor-associated amyloid proteins

468

(3)

MURPHY C.L., WANG S., KESTLER D.P., MACY S.D., WILLIAMS T.K., CARLSON E.R., WEISS D.T., SOLOMON A.

Semenogelin I is the amyloidogenic protein in senile seminal vesicles

471

(3)

LINKE R.P., JOSWIG R., MURPHY C.L., WANG S., ZHOU H., GROSS U., RÖCKEN C.B., NATHRATH W.B.J., WESTERMARK P., WEISS D., SOLOMON A.

Cardiac disease and heart valve amyloid: What is the connection?

474

(5)

ENQVIST S., WASSBERG E., WILHELMSSON M., HELLMAN U., WESTERMARK P.

SECTION 7 THERAPEUTICS

Immunotherapy in AL amyloidosis

479

(3)

SOLOMON A., WEISS D.T., WALL J.S.

The amyloid-reactive monoclonal antibody 11-1F4 binds a cryptic epitope on fibrils and partially denatured immunoglobulin light chains and inhibits fibrillogenesis

482

(3)

O'NUALLAIN B., MURPHY CL.., WOLFENBARGER D.A., KENNEL S., SOLOMON A., WALL J.S.

Molecular recognition and self-assembly of amyloid fibrils: the role of aromatic interactions

485

(3)

GAZIT E.

Serum amyloid P component as a therapeutic target of amyloidosis

488

(3)

PEPYS M.B.

Glycosaminoglycans as a therapeutic target of amyloid disorders

491

(3)

HAZENBERG B.P.C.

Inhibition of IAPP toxicity and aggregation into β-sheets and amyloid by a rationally N-methylated, IAPP amyloid core-containing hexapeptide

494

(3)

KAPURNIOTU A., SCHMAUDER A., TATAREK-NOSSOL M., TENIDIS K.

Effect of a new diflunisal derivative on transthyretin binding and stabilization in serum from FAP patients

497

(3)

ALMEIDA M.R., MACEDO B., CARDOSO I., VALENCIA G., ARSEQUELL G., PLANAS A., M.J.

Suppression of transthyretin synthesis by antisense oligonucleotides

500

(3)

BENSON M.D., KLUVE-BECKERMAN B., SLOOP K.W., BODENMILLER D.M.

Targeted conversion of the transthyretin gene in vitro and in vivo

503

(2)

NAKAMURA M., ANDO Y., UEDA M., KAWAJI T., YAMASHITA T., KIM J.M., HARAOKA K , H., NAGAHARA S., SANO A., SAITOH S., UCHINO M.

Identification of new diflunisal derivatives as potent in vitro transthyretin fibril inhibitors

505

(3)

MAIRAL T., ARSEQUELL G., VALENCIA G., DOLADO I., NIETO J., PLANAS A., BARLUENGA J., BALLESTEROS A., ALMEIDA R., SARAIVA M.J.

The future of amyloidosis

508

(2)

BENSON M.D.

We eat only disperse systems: the preparation of dishes is largely based on the control of the microstructure of food, i.e. covalent and noncovalent forces between food molecules

510

(5)

THIS H. J.-M.

SECTION 8 SATELLITE SYMPOSIUM, NEUROCHEM INC.

Satellite symposium: emerging clinical practices in AA amyloidosis

515

(2)

FILLIT H.

The changing face of AA amyloidosis

517

(4)

HAZENBERG B.P.C

Uncommon conditions underlying AA amyloidosis

521

(3)

MERLINI G.

Therapeutic management of AA amyloidosis: from bench to bedside

524

(3)

SKINNER M.

Author Index

527

(8)

Subject Index

535

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Amyloid and Amyloidosis

Summary

Table of Contents

Supplemental Materials