XIth International Symposium on Amyloidosis

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  • Edition: 1st
  • Format: Hardcover
  • Copyright: 2007-07-23
  • Publisher: CRC Press

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Featuring the work of pioneering investigators, including Nobel Laureate, Dr. Stanley Prusiner, this comprehensive volume begins with a study of the mechanisms of fibril formation and cellular response and discusses emerging diagnostic and translational approaches. It covers-· Possible horizontal transmission· Charge differences of deposits in AL and NALCDD· Novel assays to detect amyloid induced apoptosis· AA and localized amyloidosis· Hereditary gelsolin amyloidosis, and other novel mutations· ALBASE as a platform analysis tool· AL amyloidosis in the elderly· Naturally occurring antibodies as a potential therapy· Clinical trials of melphalan-dexamethasone· Autologous stem cell transplantation

Table of Contents

The effect of membranes and surfaces on the fibrillation of an amyloidogenic light chain : relevance to the etiology of AL amyloidosisp. 3
Heparan sulfate promotes the refolding and oligomerization of SAA1.1 : a short sequence is implicated in this misfolding processp. 7
Mouse senile amyloidosis : possible horizontal transmission in the mouse roomp. 10
Charge differences of deposits in AL and NALCDDp. 13
Role of the C-terminal amino acids in [beta][subscript 2]-microglobulin amyloid formationp. 16
Serum amyloid A generates high-density lipoprotein from human aortic smooth muscle cellsp. 19
Amyloid light chains localize to lysosomes and cardiac fibroblasts show reduced mitochondrial functionp. 22
Cerebrospinal fluid and plasma of Alzheimer patients promote [beta]-amyloid fibril formation in vitrop. 25
A novel assay for detection of amyloid induced apoptosisp. 27
Human amyloidogenic immunoglobulin light chains alter cardiomyocyte signaling and disrupt heparan sulfate proteoglycan (HSPG) expressionp. 30
GAGs associated with fibril extracts mediate fibrillogenesis in AL amyloidosisp. 33
Decorin in AA amyloidosisp. 36
Structural evolution of immunoglobulin light chain during amyloid fibril formation : a deep UV resonance Raman spectroscopic studyp. 39
AA amyloidogenesis : basic and translational researchp. 45
Histochemical and immunohistochemical characteristics of amyloid depositsp. 50
Ultrastructural characteristics of amyloid depositsp. 53
Odontogenic feline and canine tumor-associated amyloid is formed from ameloblastinp. 56
Amyloidogenic region of odontogenic ameloblast associated protein (ODAM)p. 59
What is the best way to assess predisposition to AA amyloid in inflammatory joint disease?p. 62
Advances in non-invasive imaging of AA amyloid in micep. 65
Detection and identification of amyloid deposition associated with human unerupted tooth folliclesp. 68
Vascular AA amyloid formation modeled in cell culturep. 70
Amyloidosis in ankylosing spondylitis - unexpected findings underscoring the importance of typing of amyloid depositsp. 73
Lack of correlation between amyloid A protein levels and clinical parameters in aspirated fat tissue of familial Mediterranean fever patients with secondary amyloidosisp. 75
Chronic, slowly progressing nephropathy due to amyloidosis of CSAA (SAA4) originp. 78
Redistribution, clearance and re-induction of amyloid in reactive murine AA amyloidosisp. 81
N-formyl peptide receptor-like 1 of the neutrophils in the AA-amyloidosis tissues is less stainedp. 84
AL in the clinical setting of AA : the importance of amyloid typingp. 88
Foie gras is a dietary source of AEFp. 90
The effect of a variety of anti-inflammatory drugs on murine amyloidogenesisp. 93
Translocation of systemic amyloidosis by peripheral blood monocytesp. 96
FK506 inhibits murine AA amyloidosis without modulating serum SAA levelsp. 99
Local tumorous AA-amyloid deposition in a case of hepatic adenomatosis : immunohistochemistry and in situ hybridization employing SAA-mRNA probesp. 102
SAA[subscript 4]-related AA amyloidosisp. 105
Measurement of amyloidogenic SAA isotype, SAA1p. 108
In vitro and in vivo effects of genistein on TTR stabilization and aggregationp. 113
Labial salivary gland biopsy for diagnosis of amyloid polyneuropathy Portuguese typep. 116
Hereditary gelsolin amyloidosis in an Iranian family : the first report from the Middle Eastp. 118
A case of compound heterozygosity in a Nigerian male with identification of a new transthyretin variant Phe44Leu and Val122Ilep. 121
Suppression of hepatic transthyretin synthesis by antisense oligonucleotidesp. 124
Top-down mass spectrometry analysis of amyloidogenic proteinsp. 127
ATTR (Thr59Lys) amyloidosis with involvement of heart, peripheral and autonomic nervous system, gut and salivary glands in an Egyptian man, previously diagnosed as "familial amyloid neuropathy of Portuguese type"p. 130
Systemic amyloidosis of fibrinogen origin in the wild living stone marten (Martes foina)p. 133
Familial amyloid nephropathy of fibrinogen A[alpha] (E526V) origin. Report of the first case from Austriap. 136
Laryngeal amyloidosis in patients with apolipoprotein AI L174S and L178Pp. 139
Hereditary fibrinogen A [alpha]-chain amyloidosis : clinical features and the curative role of liver transplantationp. 141
Amyloidosis in the transgenic mice of mouse amyloidogenic apolipoprotein A-II (Apoa2[superscript c])p. 143
Urinary transthyretin : a possible biochemical marker in the early stage of renal dysfunctionp. 146
Unique phenotype of vitreous amyloidosis in different endemic areasp. 149
Composition of peripheral nerve amyloid after orthotopic liver transplant for FAPp. 152
Characterization of fibrillar transthyretin in ex vivo cardiac deposits from cases of senile systemic amyloidosisp. 154
Comparison of cardiac amyloidosis associated with wild type transthyretin and the isoleucine 122 variantp. 157
AL amyloidosis in a patient with a T60A TTR mutationp. 160
Biochemical characterization of leptomeningeal amyloid in a Val30Gly transthyretin patientp. 163
Apolipoprotein A-I (apo A-I) nature of knee joint meniscus-associated amyloidp. 166
An autopsy case of familial amyloidotic polyneuropathy ATTR Val30Met 10 years after liver transplantation : changes in the amount of amyloid deposits in the pancreasp. 169
Amyloid neuropathy in a Japanese domino liver-transplanted recipientp. 172
The natural history of hereditary apolipoprotein A-I amyloidosis according to a large series of Italian Leu75Pro patientsp. 175
Domino liver transplantation using the explanted grafts from patients with hereditary forms of amyloidosis : the King's College Hospital experiencep. 178
Cardiomyopathy in FAP Val30Met patients with and without liver transplantationp. 180
Detection of cardiac pre-amyloid deposits of transthyretin by MALDI-TOFp. 182
Effect of nitric oxide in transthyretin-related amylodiosisp. 185
Inhibition of intracellular tetramerization induces the proteasomal degradation of amyloidogenic transthyretin variantsp. 188
Structural analyses of fibrinogen A[alpha]-chain amyloid fibrilsp. 191
A transthyretin mutation (V32A) in an Israeli patient with amyloidotic neuropathy localizes to a 3D mutation clusterp. 193
Amyloid deposition in the tongue of patients with familial amyloidotic polyneuropathyp. 196
Recent novel and rare mutations in a clinic population of patients with amyloidosisp. 199
Proliferation of smooth muscle cells in the tunica intima of cerebral vessels in familial amyloidotic polyneuropathy ATTR Y114Cp. 202
Reengineering TTR amyloid inhibition properties of diflunisalp. 205
Familial amyloidosis associated with a novel mutation (D68G) in the lysozyme genep. 208
Effects of liver transplantation on hereditary cerebral amyloid angiopathy associated with ATTR Y114Cp. 211
Effect of tacrolimus and cyclosporine on transthyretin (TTR) metabolism in rats : a long term analysisp. 214
Can AL amyloidosis be cured?p. 219
Identification of novel inhibitors of light chain fibrillogenesisp. 222
Peptide phage display and epitope mapping of a fibril-related conformational epitope recognized by the 11-1F4 monoclonal antibodyp. 225
The translocation t(11;14) is frequently detected in patients with AL amyloidosisp. 228
ALBase : a platform analysis tool for the study of amyloid light chain sequence datap. 231
Risk-adapted melphalan with stem cell transplant (SCT) and adjuvant dexamethasone (dex) +/- thalidomide (thai) achieves low treatment-related mortality and high hematologic response ratesp. 234
A supervised analysis of gene-expression profiles of purified clonal plasma cells from patients with systemic light-chain amyloidosis (AL) who have high or low levels of serum free lambda light chainsp. 237
Comparison of immunoglobulin light chains (K1) : amyloidogenic vs. non-amyloidogenic proteinsp. 240
Clinicopathological analysis of thirty-two patients with AL amyloidosis : four-years experience in a single institutionp. 243
Role of light chain deamidation in the pathogenesis of AL amyloidosisp. 246
The extent and distribution of amyloid in 38 autopsies on patients with primary (AL) amyloidosisp. 249
Transplantation without growth factor engraftment kinetics following stem cell transplantation for primary systemic amyloidosisp. 252
Extent of hematologic response is important in determining outcome in transplanted patients with primary systemic amyloidosis. Importance of achieving a complete responsep. 255
A single-center experience using chemotherapy with melphalan-dexamethasone (M-dex) in patients with AL amyloidosis and advanced organ involvement not eligible for high-dose melphalan therapy (HDM)p. 258
Detection and characterization of AL light chain proteins from fibrilsp. 261
Characterization of post-translational modifications of an amyloidogenic immunoglobulin kappa light chain by mass spectrometryp. 264
Involvement of lysosomes in renal AL-amyloidogenesisp. 267
Thermal unfolding of immunoglobulin light chains is linked to aggregation : circular dichroism and light scattering studiesp. 270
Prognostic relevance of serum N-terminal pronatriuretic peptide type B and plasma BNP in patients with AL amyloidosis in dialysisp. 273
AL amyloidosis in the elderly : a review of 330 patientsp. 275
Therapeutic potential of naturally occurring fibril-reactive human antibodies for AL amyloidosisp. 278
Prolonged follow-up study of AL patients ineligible for stem cell transplantation treated with oral melphalan and dexamethasonep. 281
Left ventricular systolic function in patients with cardiac AL amyloidosisp. 283
In vitro silencing of amyloidogenic [lambda]6 light chain productionp. 286
Amyloid typing in surgical pathology : experience of a single institutionp. 289
Somatic mutations create potential N-glycosylation sites in the immunoglobulin light chain variable regions in primary amyloidosisp. 292
A trial of antioxidants to improve cardiac function in patient with AL amyloidosis and cardiac involvementp. 295
High-dose melphalan chemotherapy with autologous stem cell transplantation in 65 patients with AL amyloidosis : organ progression can be delayed in patients reaching complete remissionp. 298
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