Understanding and Ameliorating the TTR Amyloidoses

Understanding and Ameliorating the TTR Amyloidoses is …
instance of (P31):
scholarly articleQ13442814

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P356DOI10.1002/9780470572702.CH45

P50authorJeffery W. KellyQ6175781
Johan F PaulssonQ56073524
R. Luke WisemanQ57429656
Sungwook ChoiQ59662360
Joel N BuxbaumQ88718489
P2093author name stringEvan T. Powers
Steven M. Johnson
P2860cites workComparison of Lethal and Nonlethal Transthyretin Variants and Their Relationship to Amyloid DiseaseQ41670698
The most pathogenic transthyretin variant, L55P, forms amyloid fibrils under acidic conditions and protofilaments under physiological conditionsQ41696172
Fibril in senile systemic amyloidosis is derived from normal transthyretinQ24558717
Tissue damage in the amyloidoses: Transthyretin monomers and nonnative oligomers are the major cytotoxic species in tissue cultureQ24626477
Amyloid-beta protein dimers isolated directly from Alzheimer's brains impair synaptic plasticity and memoryQ24645499
Deposition of transthyretin in early stages of familial amyloidotic polyneuropathy: evidence for toxicity of nonfibrillar aggregatesQ24685634
Rational design of potent human transthyretin amyloid disease inhibitorsQ27621990
A comparative analysis of 23 structures of the amyloidogenic protein transthyretinQ27627061
An engineered transthyretin monomer that is nonamyloidogenic, unless it is partially denaturedQ27634853
Benzoxazoles as transthyretin amyloid fibril inhibitors: synthesis, evaluation, and mechanism of actionQ27641464
Synthesis and characterization of potent bivalent amyloidosis inhibitors that bind prior to transthyretin tetramerizationQ27642438
Diflunisal analogues stabilize the native state of transthyretin. Potent inhibition of amyloidogenesisQ27642917
Human-murine transthyretin heterotetramers are kinetically stable and non-amyloidogenic. A lesson in the generation of transgenic models of diseases involving oligomeric proteinsQ27649069
Biochemical and structural evaluation of highly selective 2-arylbenzoxazole-based transthyretin amyloidogenesis inhibitorsQ27649376
Structural insight into pH-induced conformational changes within the native human transthyretin tetramerQ27651370
Toward Optimization of the Linker Substructure Common to Transthyretin Amyloidogenesis Inhibitors Using Biochemical and Structural Studies †Q27652272
Structure of prealbumin: secondary, tertiary and quaternary interactions determined by Fourier refinement at 1.8 AQ27729158
Structure of a complex of two plasma proteins: transthyretin and retinol-binding proteinQ27730236
Instability, unfolding and aggregation of human lysozyme variants underlying amyloid fibrillogenesisQ27734827
Inhibiting transthyretin conformational changes that lead to amyloid fibril formationQ27765742
Adapting proteostasis for disease interventionQ28131818
A peculiar form of peripheral neuropathy; familiar atypical generalized amyloidosis with special involvement of the peripheral nervesQ28207316
Protein folding and misfoldingQ28235199
Folding proteins in fatal waysQ28235206
A primer of amyloid nomenclatureQ28241008
Autonomic dysfunction in familial amyloidotic polyneuropathy (FAP)Q28370305
The alternative conformations of amyloidogenic proteins and their multi-step assembly pathwaysQ41728317
Anion shielding of electrostatic repulsions in transthyretin modulates stability and amyloidosis: insight into the chaotrope unfolding dichotomyQ43740028
Synthesis, structure, and activity of diclofenac analogues as transthyretin amyloid fibril formation inhibitorsQ43849715
Screening transthyretin amyloid fibril inhibitors: characterization of novel multiprotein, multiligand complexes by mass spectrometryQ44022725
Pathology of early- vs late-onset TTR Met30 familial amyloid polyneuropathyQ44603656
Protofilaments, filaments, ribbons, and fibrils from peptidomimetic self-assembly:  implications for amyloid fibril formation and materials scienceQ44678447
A different amyloid formation mechanism: de novo oculoleptomeningeal amyloid deposits after liver transplantationQ44765894
Native state stabilization by NSAIDs inhibits transthyretin amyloidogenesis from the most common familial disease variantsQ44767199
Transthyretin is involved in depression-like behaviour and exploratory activityQ44792315
Transthyretin aggregation under partially denaturing conditions is a downhill polymerizationQ44925089
Neutralization of transthyretin reverses the neuroprotective effects of secreted amyloid precursor protein (APP) in APPSW mice resulting in tau phosphorylation and loss of hippocampal neurons: support for the amyloid hypothesis.Q45041133
Design, synthesis, and evaluation of oxazole transthyretin amyloidogenesis inhibitorsQ45248277
Gene therapy: lessons learned from liver transplantation for transthyretin-amyloidosisQ45879595
Kinetic stabilization of an oligomeric protein under physiological conditions demonstrated by a lack of subunit exchange: implications for transthyretin amyloidosis.Q46251178
Characterization of the transthyretin acid denaturation pathways by analytical ultracentrifugation: implications for wild-type, V30M, and L55P amyloid fibril formationQ46301078
Kinetic stabilization of the native state by protein engineering: implications for inhibition of transthyretin amyloidogenesisQ46380708
Oxidative metabolites accelerate Alzheimer's amyloidogenesis by a two-step mechanism, eliminating the requirement for nucleationQ46407641
Kinetic stabilization of an oligomeric protein by a single ligand binding eventQ46425326
Potent and selective structure-based dibenzofuran inhibitors of transthyretin amyloidogenesis: kinetic stabilization of the native stateQ46470305
Senile systemic amyloidosis affects 25% of the very aged and associates with genetic variation in alpha2-macroglobulin and tau: a population-based autopsy studyQ46645719
Transthyretin isoleucine-122 mutation in African and American blacksQ46808325
Structures of human transthyretin complexed with thyroxine at 2.0 A resolution and 3',5'-dinitro-N-acetyl-L-thyronine at 2.2 A resolutionQ47983806
Imaging distinct conformational states of amyloid-beta fibrils in Alzheimer's disease using novel luminescent probesQ48087328
The ups and downs of AbetaQ48475770
Neurodegenerative disease: amyloid pores from pathogenic mutationsQ48544171
Stopped-flow kinetics reveal multiple phases of thioflavin T binding to Alzheimer beta (1-40) amyloid fibrilsQ48683371
Intraneuronal Abeta accumulation precedes plaque formation in beta-amyloid precursor protein and presenilin-1 double-transgenic miceQ48846848
Analysis of protein aggregation kinetics.Q52143609
Genetic epidemiology of familial amyloidotic polyneuropathy (FAP)-type I in Póvoa do Varzim and Vila do Conde (north of Portugal).Q52807508
Negative cooperativity in the binding of thyroxine to human serum prealbumin. Preparation of tritium-labeled 8-anilino-1-naphthalenesulfonic acidQ52883960
Soluble oligomers of beta amyloid (1-42) inhibit long-term potentiation but not long-term depression in rat dentate gyrus.Q53245545
Progression of cardiomyopathy after liver transplantation in patients with familial amyloidotic polyneuropathy, Portuguese type.Q54160889
Familial amyloid polyneuropathyQ56031909
The gene encoding 5-lipoxygenase activating protein confers risk of myocardial infarction and strokeQ56944340
Susceptibility and modifier genes in Portuguese transthyretin V30M amyloid polyneuropathy: complexity in a single-gene diseaseQ57240374
Liver Transplantation Does Not Prevent the Development of Life-Threatening Arrhythmia in Familial Amyloidotic Polyneuropathy, Portuguese-Type (ATTR Val30Met) PatientsQ57259453
Diflunisal stabilizes familial amyloid polyneuropathy-associated transthyretin variant tetramers in serum against dissociation required for amyloidogenesisQ57424500
Synchrotron X-ray studies suggest that the core of the transthyretin amyloid fibril is a continuous β-sheet helixQ57945110
Transthyretin enhances nerve regenerationQ58154194
No Evidence Of De Novo Amyloidosis In Recipients Of Domino Liver Transplantation: 12 To 40 (Mean 24) Month Follow-UpQ58186102
Discordant symptoms in monozygotic twins with familial amyloidotic polyneuropathy (FAP) (TTR Met 30)Q58261278
In vivostabilization of mutant human transthyretin in transgenic miceQ58274802
Transthyretin: a choroid plexus-specific transport protein in human brain. The 1986 S. Weir Mitchell awardQ28609126
Alzheimer's disease: the amyloid cascade hypothesisQ29547160
Diffusible, nonfibrillar ligands derived from Abeta1-42 are potent central nervous system neurotoxinsQ29547593
Twenty years of the Alzheimer's disease amyloid hypothesis: a genetic perspectiveQ29614702
A beta oligomers - a decade of discoveryQ29615152
Opposing activities protect against age-onset proteotoxicityQ29619758
Plasma retinol-binding protein: structure and interactions with retinol, retinoids, and transthyretinQ30164014
Bisaryloxime ethers as potent inhibitors of transthyretin amyloid fibril formationQ30779129
The oxidative stress metabolite 4-hydroxynonenal promotes Alzheimer protofibril formationQ33272109
Cell division and apoptosis in the adult neural stem cell niche are differentially affected in transthyretin null miceQ33288004
The transthyretin gene is expressed in human and rodent dorsal root gangliaQ33327859
Synthesis and evaluation of inhibitors of transthyretin amyloid formation based on the non-steroidal anti-inflammatory drug, flufenamic acidQ33872413
The genetics of the amyloidosesQ33896126
Nucleated conformational conversion and the replication of conformational information by a prion determinantQ33915141
Liver transplantation for hereditary transthyretin amyloidosis.Q33927163
Support for the multigenic hypothesis of amyloidosis: the binding stoichiometry of retinol-binding protein, vitamin A, and thyroid hormone influences transthyretin amyloidogenicity in vitroQ33949040
The V122I cardiomyopathy variant of transthyretin increases the velocity of rate-limiting tetramer dissociation, resulting in accelerated amyloidosisQ33952780
Amyloid-beta aggregation: selective inhibition of aggregation in mixtures of amyloid with different chain lengthsQ34018579
Transmissibility of systemic amyloidosis by a prion-like mechanismQ34068073
Trans-suppression of misfolding in an amyloid disease.Q34092993
Prevention of transthyretin amyloid disease by changing protein misfolding energetics.Q34174171
Pathogenesis, diagnosis and treatment of systemic amyloidosisQ34190813
D18G transthyretin is monomeric, aggregation prone, and not detectable in plasma and cerebrospinal fluid: a prescription for central nervous system amyloidosis?Q34201956
Partial denaturation of transthyretin is sufficient for amyloid fibril formation in vitroQ34252457
Therapeutic approaches to protein-misfolding diseasesQ34285075
Clinical improvement and amyloid regression after liver transplantation in hereditary transthyretin amyloidosis.Q34334873
Hydroxylated polychlorinated biphenyls selectively bind transthyretin in blood and inhibit amyloidogenesis: rationalizing rodent PCB toxicityQ34378271
The acid-mediated denaturation pathway of transthyretin yields a conformational intermediate that can self-assemble into amyloidQ34380911
Alternative conformations of amyloidogenic proteins govern their behaviorQ34387876
The biological and chemical basis for tissue-selective amyloid diseaseQ34410187
Variant-sequence transthyretin (isoleucine 122) in late-onset cardiac amyloidosis in black AmericansQ34415207
Is aggregated IAPP a cause of beta-cell failure in transplanted human pancreatic islets?Q34423072
Transthyretin: a review from a structural perspective.Q34427841
Sequence-dependent denaturation energetics: A major determinant in amyloid disease diversityQ34443897
The pathway by which the tetrameric protein transthyretin dissociates.Q34469219
Native state kinetic stabilization as a strategy to ameliorate protein misfolding diseases: a focus on the transthyretin amyloidoses.Q34477195
Orally administered diflunisal stabilizes transthyretin against dissociation required for amyloidogenesisQ34581917
Familial transthyretin-type amyloid polyneuropathy in Japan: clinical and genetic heterogeneity.Q34592571
Therapeutic strategies for human amyloid diseasesQ34743288
The most infectious prion protein particles.Q34805737
Thirty years of Alzheimer's disease genetics: the implications of systematic meta-analysesQ34831241
Transthyretin in fish: state of the art.Q35054934
Alpha(1)-antitrypsin deficiency, liver disease and emphysemaQ35097639
Hereditary transthyretin amyloidosis from a Scandinavian perspective.Q35203818
A role for destabilizing amino acid replacements in light-chain amyloidosisQ35458844
DNA variation and brain region-specific expression profiles exhibit different relationships between inbred mouse strains: implications for eQTL mapping studies.Q35751646
Familial amyloidotic polyneuropathy: protein aggregation in the peripheral nervous systemQ35764307
Intraneuronal Abeta42 accumulation in human brain.Q35793325
Evaluating the binding selectivity of transthyretin amyloid fibril inhibitors in blood plasmaQ35885902
Liver transplantation in transthyretin-related familial amyloid polyneuropathyQ35887737
Alzheimer's disease-affected brain: presence of oligomeric A beta ligands (ADDLs) suggests a molecular basis for reversible memory lossQ35918370
Homing in on intracellular Abeta?Q36062369
The in vivo neuromodulatory effects of the herbal medicine ginkgo bilobaQ36227174
Mechanisms of protein fibril formation: nucleated polymerization with competing off-pathway aggregationQ36303237
Transthyretin protects Alzheimer's mice from the behavioral and biochemical effects of Abeta toxicityQ36497488
Small molecule oxidation products trigger disease-associated protein misfolding.Q36596598
Transthyretin slowly exchanges subunits under physiological conditions: A convenient chromatographic method to study subunit exchange in oligomeric proteins.Q36640504
Systematic analysis of nucleation-dependent polymerization reveals new insights into the mechanism of amyloid self-assemblyQ36736931
Folding versus aggregation: polypeptide conformations on competing pathwaysQ36858313
Inhibiting transthyretin amyloid fibril formation via protein stabilizationQ37082709
Heparin accelerates gelsolin amyloidogenesisQ37132636
Quantification of the thermodynamically linked quaternary and tertiary structural stabilities of transthyretin and its disease-associated variants: the relationship between stability and amyloidosis.Q37153865
Neuronal cell death in Alzheimer's disease correlates with apoE uptake and intracellular Abeta stabilizationQ37369949
Metabolite-initiated protein misfolding may trigger Alzheimer's diseaseQ37414917
Transthyretin mutation Leu-55-Pro significantly alters tetramer stability and increases amyloidogenicityQ38314199
A prospective evaluation of the transthyretin Ile122 allele frequency in an African-American populationQ39723184
Alzheimer's Abeta peptides containing an isostructural backbone mutation afford distinct aggregate morphologies but analogous cytotoxicity. Evidence for a common low-abundance toxic structure(s)?Q40035906
The cerebral expression of plasma protein genes in different speciesQ40525379
Deuterium-proton exchange on the native wild-type transthyretin tetramer identifies the stable core of the individual subunits and indicates mobility at the subunit interfaceQ40741466
A glimpse of a possible amyloidogenic intermediate of transthyretinQ40750422
Seeding "one-dimensional crystallization" of amyloid: a pathogenic mechanism in Alzheimer's disease and scrapie?Q40918121
For protein misassembly, it's the "I" decadeQ41106314
P304page(s)967-1003
P577publication date2010-07-02
P1476titleUnderstanding and Ameliorating the TTR Amyloidoses

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