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List of works by Jeffery W. Kelly

"Inverse Drug Discovery" Strategy To Identify Proteins That Are Targeted by Latent Electrophiles As Exemplified by Aryl Fluorosulfates

scientific article published on 21 December 2017

A Substructure Combination Strategy To Create Potent and Selective Transthyretin Kinetic Stabilizers That Prevent Amyloidogenesis and Cytotoxicity

scientific article

A biomimetic synthesis of thiazolines using hexaphenyloxodiphosphonium trifluoromethanesulfonate

scientific article published in January 2003

A chemical approach to elucidate tin mechanism of transthyretin and β-protein amyloid fibril formation

article by Jeffery W. Kelly & Peter T. Lansbury published January 1994 in Amyloid

A competition assay to identify amyloidogenesis inhibitors by monitoring the fluorescence emitted by the covalent attachment of a stilbene derivative to transthyretin ⬇️

scientific article published on December 30, 2010

A cross-strand Trp Trp pair stabilizes the hPin1 WW domain at the expense of function

scientific article published on 31 August 2007

A current pharmacologic agent versus the promise of next generation therapeutics to ameliorate protein misfolding and/or aggregation diseases

scientific article published on 06 February 2016

A designed protein binding-pocket to control excited-state intramolecular proton transfer fluorescence

scientific article published on 01 January 2019

A fluorogenic aryl fluorosulfate for intraorganellar transthyretin imaging in living cells and in Caenorhabditis elegans

scientific article published on 8 June 2015

A fundamental protein property, thermodynamic stability, revealed solely from large-scale measurements of protein function

scientific article

A general strategy for the bacterial expression of amyloidogenic peptides using BCL-XL-1/2 fusions

scientific article published on September 2009

A high-throughput cell-based Gaussia luciferase reporter assay for identifying modulators of fibulin-3 secretion

scientific article

A kinetic aggregation assay allowing selective and sensitive amyloid-β quantification in cells and tissues

scientific article published on 26 January 2011

A kinetic assessment of the C. elegans amyloid disaggregation activity enables uncoupling of disassembly and proteolysis

scientific article

A stilbene that binds selectively to transthyretin in cells and remains dark until it undergoes a chemoselective reaction to create a bright blue fluorescent conjugate

scientific article

AG10 inhibits amyloidogenesis and cellular toxicity of the familial amyloid cardiomyopathy-associated V122I transthyretin

scientific article

ALS mutants of human superoxide dismutase form fibrous aggregates via framework destabilization

scientific article

Adapting proteostasis for disease intervention

scientific article (publication date: 15 February 2008)

Adsorption of beta-hairpin peptides on the surface of water: a neutron reflection study

scientific article published in April 2003

Age-dependent cognitive dysfunction in untreated hereditary transthyretin amyloidosis

scientific article published on 5 December 2017

Aggregating knowledge about prions and amyloid

scientific article published in April 2009

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)?

scientific article published on 14 December 2007

Amide-to-E-olefin versus amide-to-ester backbone H-bond perturbations: Evaluating the O-O repulsion for extracting H-bond energies

scientific article published on December 2006

Amylin proprotein processing generates progressively more amyloidogenic peptides that initially sample the helical state

scientific article published on 19 August 2008

Amyloid Accumulation Drives Proteome-wide Alterations in Mouse Models of Alzheimer's Disease-like Pathology.

scientific article

Amyloid as a natural product

scientific article

Amyloid fibrils trigger the release of neutrophil extracellular traps (NETs), causing fibril fragmentation by NET-associated elastase

scientific article

Amyloid-β forms fibrils by nucleated conformational conversion of oligomers

scientific article published on 31 July 2011

An Adaptable Standard for Protein Export from the Endoplasmic Reticulum

article

An experimental survey of the transition between two-state and downhill protein folding scenarios

scholarly article

An ortho-Iminoquinone Compound Reacts with Lysine Inhibiting Aggregation while Remodeling Mature Amyloid Fibrils.

scientific article published on 20 April 2017

Antibody domain exchange is an immunological solution to carbohydrate cluster recognition

scientific article

Aromatic Sulfonyl Fluorides Covalently Kinetically Stabilize Transthyretin to Prevent Amyloidogenesis while Affording a Fluorescent Conjugate

scientific article

Arylfluorosulfates Inactivate Intracellular Lipid Binding Protein(s) through Chemoselective SuFEx Reaction with a Binding Site Tyr Residue

scientific article published on 18 May 2016

Attacking amyloid

scientific article

Backbone-Backbone H-Bonds Make Context-Dependent Contributions to Protein Folding Kinetics and Thermodynamics: Lessons from Amide-to-Ester Mutations.

scientific article

Benzoxazoles as transthyretin amyloid fibril inhibitors: synthesis, evaluation, and mechanism of action

scientific article

Beta-sheet folding mechanisms from perturbation energetics

scientific article

Bifunctional coumarin derivatives that inhibit transthyretin amyloidogenesis and serve as fluorescent transthyretin folding sensors

scientific article

Binding of 3,4,5,6-Tetrahydroxyazepanes to the Acid-β-glucosidase Active Site: Implications for Pharmacological Chaperone Design for Gaucher Disease

scientific article

Biochemical and structural evaluation of highly selective 2-arylbenzoxazole-based transthyretin amyloidogenesis inhibitors

scientific article

Biochemical properties of highly neuroinvasive prion strains

scientific article

Bisaryloxime ethers as potent inhibitors of transthyretin amyloid fibril formation

scientific article

Blinded potency comparison of transthyretin kinetic stabilisers by subunit exchange in human plasma

scientific article published on 18 August 2020

Broadly applicable methodology for the rapid and dosable small molecule-mediated regulation of transcription factors in human cells.

scientific article published on 21 May 2013

Ca2+ binding protects against gelsolin amyloidosis

scientific article published in October 2004

Cell based screening of inhibitors of transthyretin aggregation

scientific article published on 31 July 2006

Chaperonins resculpt folding free energy landscapes to avoid kinetic traps and accelerate protein folding

scientific article published on 05 June 2014

Chemical and Biological Approaches for Adapting Proteostasis to Ameliorate Protein Misfolding and Aggregation Diseases-Progress and Prognosis ⬇️

scientific article published on December 1, 2011

Chemical and biological approaches synergize to ameliorate protein-folding diseases.

scientific article

Chemical and/or biological therapeutic strategies to ameliorate protein misfolding diseases

scientific article published on 09 December 2010

Chemical chaperones and permissive temperatures alter localization of Gaucher disease associated glucocerebrosidase variants

scientific article published in May 2006

Chemical chaperones increase the cellular activity of N370S beta -glucosidase: a therapeutic strategy for Gaucher disease

scientific article

Chemoselective small molecules that covalently modify one lysine in a non-enzyme protein in plasma

scientific article

Comparative Characterization of a Wild Type and Transmembrane Domain-Deleted Fatty Acid Amide Hydrolase: Identification of the Transmembrane Domain as a Site for Oligomerization ⬇️

scientific article published on October 27, 1998

Comparison of Lethal and Nonlethal Transthyretin Variants and Their Relationship to Amyloid Disease ⬇️

scientific article published on October 17, 1995

Compromised mutant EFEMP1 secretion associated with macular dystrophy remedied by proteostasis network alteration

scientific article published on 26 October 2011

Conformational differences between the wild type and V30M mutant transthyretin modulate its binding to genistein: implications to tetramer stability and ligand-binding

scientific article

Context-dependent contributions of backbone hydrogen bonding to beta-sheet folding energetics

scientific article

Context-dependent effects of asparagine glycosylation on Pin WW folding kinetics and thermodynamics.

scientific article

Controlling the morphology of cross beta-sheet assemblies by rational design

scientific article published in June 2005

Correction to Context-Dependent Effects of Asparagine Glycosylation on Pin WW Folding Kinetics and Thermodynamics

scientific article published on 27 February 2012

Correction to “Mispacking of the Phe87 Side Chain Reduces the Kinetic Stability of Human Transthyretin”

scientific article published on 07 January 2019

Cys-10 mixed disulfide modifications exacerbate transthyretin familial variant amyloidogenicity: a likely explanation for variable clinical expression of amyloidosis and the lack of pathology in C10S/V30M transgenic mice?

scientific article published in June 2005

Cys10 mixed disulfides make transthyretin more amyloidogenic under mildly acidic conditions

scientific article published in July 2003

D18G transthyretin is monomeric, aggregation prone, and not detectable in plasma and cerebrospinal fluid: a prescription for central nervous system amyloidosis?

scientific article

De novo-designed enzymes as small-molecule-regulated fluorescence imaging tags and fluorescent reporters

scientific article published on 11 September 2014

Deducing the presence of proteins and proteoforms in quantitative proteomics.

scientific article published on 13 June 2018

Design, synthesis, and evaluation of oxazole transthyretin amyloidogenesis inhibitors

scientific article

Determinants for Dephosphorylation of the RNA Polymerase II C-Terminal Domain by Scp1

scientific article

Diflunisal analogues stabilize the native state of transthyretin. Potent inhibition of amyloidogenesis

scientific article

Discovery and characterization of a mammalian amyloid disaggregation activity

scientific article published on April 2010

Dissecting the structure, thermodynamic stability, and aggregation properties of the A25T transthyretin (A25T-TTR) variant involved in leptomeningeal amyloidosis: identifying protein partners that co-aggregate during A25T-TTR fibrillogenesis in [...]

scientific article

Dissociation of amyloid fibrils of alpha-synuclein and transthyretin by pressure reveals their reversible nature and the formation of water-excluded cavities

scientific article published on 4 August 2003

E-olefin dipeptide isostere incorporation into a polypeptide backbone enables hydrogen bond perturbation: probing the requirements for Alzheimer's amyloidogenesis

scientific article published in November 2005

ERdj3 is an endoplasmic reticulum degradation factor for mutant glucocerebrosidase variants linked to Gaucher's disease.

scientific article published on August 2014

Elevated levels of oxidized cholesterol metabolites in Lewy body disease brains accelerate alpha-synuclein fibrilization

scientific article published on 26 March 2006

Endoplasmic Reticulum Proteostasis Influences the Oligomeric State of an Amyloidogenic Protein Secreted from Mammalian Cells

scientific article published on 6 October 2016

Endoplasmic reticulum Ca2+ increases enhance mutant glucocerebrosidase proteostasis.

scientific article published on 9 May 2010

Energetic characteristics of the new transthyretin variant A25T may explain its atypical central nervous system pathology.

scientific article published in March 2003

Engineering a beta-sheet protein toward the folding speed limit

scientific article published in August 2005

Enhanced Aromatic Sequons Increase Oligosaccharyltransferase Glycosylation Efficiency and Glycan Homogeneity

scientific article

Erratum to “ALS Mutants of Human Superoxide Dismutase form Fibrous Aggregates via Framework Destabilization” [J. Mol. Biol. 332 (2003) 601–615]

scholarly article published in Journal of Molecular Biology

Evaluating beta-turn mimics as beta-sheet folding nucleators

scientific article

Evolving protein stability through genetic selection.

scientific article

FKBP10 depletion enhances glucocerebrosidase proteostasis in Gaucher disease fibroblasts.

scientific article published on 21 February 2013

Finally, a transgenic light chain amyloidosis mouse model ⬇️

scientific article published on December 15, 2011

Fluorescence Turn-On Folding Sensor To Monitor Proteome Stress in Live Cells.

scientific article published on 25 August 2015

Formation of gelsolin amyloid fibrils in the rough endoplasmic reticulum of skeletal muscle in the gelsolin mouse model of inclusion body myositis: comparative analysis to human sporadic inclusion body myositis

scientific article published on October 2013

Fourier transform infrared spectroscopy provides a fingerprint for the tetramer and for the aggregates of transthyretin

scientific article

Functional amyloid formation within mammalian tissue

scientific article published in January 2006

Functional amyloid--from bacteria to humans.

scientific article published on 6 April 2007

Functional amyloidogenesis and cytotoxicity-insights into biology and pathology

scientific article

Furin initiates gelsolin familial amyloidosis in the Golgi through a defect in Ca2+ stabilization ⬇️

scientific article published on November 15, 2001

Gaucher disease-associated glucocerebrosidases show mutation-dependent chemical chaperoning profiles

scientific article published in November 2005

Gelsolin amyloidosis: genetics, biochemistry, pathology and possible strategies for therapeutic intervention

scientific article published on 24 February 2012

Gelsolin domain 2 Ca2+ affinity determines susceptibility to furin proteolysis and familial amyloidosis of finnish type

scientific article

Genetic ablation of N-linked glycosylation reveals two key folding pathways for R345W fibulin-3, a secreted protein associated with retinal degeneration

scientific article

Genistein, a natural product from soy, is a potent inhibitor of transthyretin amyloidosis

scientific article

Glycosylation of the enhanced aromatic sequon is similarly stabilizing in three distinct reverse turn contexts

scientific article

Guanidine Hydrochloride-Induced Denaturation and Refolding of Transthyretin Exhibits a Marked Hysteresis: Equilibria with High Kinetic Barriers ⬇️

scientific article published on August 19, 1997

Heat-shock response transcriptional program enables high-yield and high-quality recombinant protein production in Escherichia coli.

scientific article published on 2 August 2014

Heparin accelerates gelsolin amyloidogenesis

scientific article published on February 2006

Heparin binds 8 kDa gelsolin cross-β-sheet oligomers and accelerates amyloidogenesis by hastening fibril extension

scientific article published on 15 March 2011

High-Resolution Mapping of the Folding Transition State of a WW Domain

scientific article published on 12 February 2016

Highly efficient biomimetic total synthesis and structural verification of bistratamides E and J from Lissoclinum bistratum

scientific article published on January 2004

Highly efficient enantiospecific synthesis of imidazoline-containing amino acids using bis(triphenyl)oxodiphosphonium trifluoromethanesulfonate

scientific article published on 01 May 2004

Hsp104 gives clients the individual attention they need

scientific article published on 01 November 2012

Hsp90 cochaperone Aha1 downregulation rescues misfolding of CFTR in cystic fibrosis

scientific article

Human-murine transthyretin heterotetramers are kinetically stable and non-amyloidogenic. A lesson in the generation of transgenic models of diseases involving oligomeric proteins

scientific article

Hydration and packing are crucial to amyloidogenesis as revealed by pressure studies on transthyretin variants that either protect or worsen amyloid disease

scientific article published in May 2003

Hydroxylated polychlorinated biphenyls selectively bind transthyretin in blood and inhibit amyloidogenesis: rationalizing rodent PCB toxicity

scientific article

Immunoprecipitation of amyloid fibrils by the use of an antibody that recognizes a generic epitope common to amyloid fibrils

scientific article

Improved Synthesis of the Boc and Fmoc Derivatives of 4-(2'-Aminoethyl)-6-dibenzofuranpropionic Acid: An Unnatural Amino Acid That Nucleates beta-Sheet Folding

scientific article published on 01 April 1997

Improving mass spectrometric sequencing of arginine-containing peptides by derivatization with acetylacetone ⬇️

scientific article published on December 1, 1997

Incomplete Refolding of Antibody Light Chains to Non-Native, Protease-Sensitive Conformations Leads to Aggregation: A Mechanism of Amyloidogenesis in Patients?

scientific article published on 4 December 2017

Individual and collective contributions of chaperoning and degradation to protein homeostasis in E. coli

scientific article

Influence of hPin1 WW N-terminal domain boundaries on function, protein stability, and folding

scientific article

Influence of the N-terminal domain on the aggregation properties of the prion protein

scientific article published on August 2005

Inhibiting transthyretin conformational changes that lead to amyloid fibril formation ⬇️

scientific article published on October 27, 1998

Isofagomine- and 2,5-anhydro-2,5-imino-D-glucitol-based glucocerebrosidase pharmacological chaperones for Gaucher disease intervention.

scientific article published on January 2007

Kinetic stabilization of an oligomeric protein by a single ligand binding event

scientific article published in April 2005

Kinetic stabilization of an oligomeric protein under physiological conditions demonstrated by a lack of subunit exchange: implications for transthyretin amyloidosis.

scientific article

Kinetic stabilization of the native state by protein engineering: implications for inhibition of transthyretin amyloidogenesis

scientific article

Localized structural fluctuations promote amyloidogenic conformations in transthyretin

scientific article published on 11 January 2013

Localized thermodynamic coupling between hydrogen bonding and microenvironment polarity substantially stabilizes proteins

scientific article published on 14 June 2009

Long-term effects of tafamidis for the treatment of transthyretin familial amyloid polyneuropathy

scientific article published on 22 August 2013

Lovastatin ameliorates alpha-synuclein accumulation and oxidation in transgenic mouse models of alpha-synucleinopathies

scientific article

Mechanism of Action and Clinical Application of Tafamidis in Hereditary Transthyretin Amyloidosis

scientific article

Mechanism of Action of the Cytotoxic Asmarine Alkaloids.

scientific article published on 13 April 2018

Mechanisms of transthyretin cardiomyocyte toxicity inhibition by resveratrol analogs

scientific article

Mechanisms of transthyretin inhibition of β-amyloid aggregation in vitro

scientific article published on December 2013

Medicine. The yin-yang of sirtuins

scientific article

Metabolite-initiated protein misfolding may trigger Alzheimer's disease

scholarly article

Metalloendoprotease cleavage triggers gelsolin amyloidogenesis

scientific article

Mispacking of the Phe87 Side Chain Reduces the Kinetic Stability of Human Transthyretin

scientific article published on 14 December 2018

N-PEGylation of a reverse turn is stabilizing in multiple sequence contexts, unlike N-GlcNAcylation

scientific article

N-glycosylation of enhanced aromatic sequons to increase glycoprotein stability

scientific article published on 3 February 2012

N-terminal domains elicit formation of functional Pmel17 amyloid fibrils

scientific article published on December 2009

NMR Measurements Reveal the Structural Basis of Transthyretin Destabilization by Pathogenic Mutations

scientific article published on 18 July 2018

NMR solution structure of the isolated Apo Pin1 WW domain: comparison to the x-ray crystal structures of Pin1

scientific article

Native state hydrogen exchange study of suppressor and pathogenic variants of transthyretin

scientific article published on 01 July 2002

Native state kinetic stabilization as a strategy to ameliorate protein misfolding diseases: a focus on the transthyretin amyloidoses.

scientific article

Native state stabilization by NSAIDs inhibits transthyretin amyloidogenesis from the most common familial disease variants

scientific article published in May 2004

Opposing activities protect against age-onset proteotoxicity

scientific article (publication date: 15 September 2006)

Orally administered diflunisal stabilizes transthyretin against dissociation required for amyloidogenesis

scientific article

Ordered Langmuir-Blodgett Films of Amphiphilic β-Hairpin Peptides Imaged by Atomic Force Microscopy

scientific article published on 01 January 2002

Oxidative metabolites accelerate Alzheimer's amyloidogenesis by a two-step mechanism, eliminating the requirement for nucleation

scientific article published in April 2005

Oxidized cholesterol metabolites found in human atherosclerotic lesions promote apolipoprotein C-II amyloid fibril formation

scientific article published on 13 April 2007

Partial restoration of mutant enzyme homeostasis in three distinct lysosomal storage disease cell lines by altering calcium homeostasis

scientific article

Partitioning conformational intermediates between competing refolding and aggregation pathways: insights into transthyretin amyloid disease.

scientific article published on December 2005

Pathogenic Mutations Induce Partial Structural Changes in the Native β-Sheet Structure of Transthyretin and Accelerate Aggregation.

scientific article published on 18 August 2017

Pathological and functional amyloid formation orchestrated by the secretory pathway

scientific article published on December 2003

Peptide probes detect misfolded transthyretin oligomers in plasma of hereditary amyloidosis patients.

scientific article published in September 2017

Peripheral Blood Cell Gene Expression Diagnostic for Identifying Symptomatic Transthyretin Amyloidosis Patients: Male and Female Specific Signatures.

scientific article published on 18 July 2016

Personalized medicine approach for optimizing the dose of tafamidis to potentially ameliorate wild-type transthyretin amyloidosis (cardiomyopathy).

scientific article

Pharmacologic ATF6 activating compounds are metabolically activated to selectively modify endoplasmic reticulum proteins

scientific article published on 07 August 2018

Pharmacologic ATF6 activation confers global protection in widespread disease models by reprograming cellular proteostasis

scientific article published in Nature Communications

Pharmacologic chaperoning as a strategy to treat Gaucher disease

scientific article published on October 2007

Phosphatase Inhibition Delays Translational Recovery ⬇️

scientific article published on April 1, 2011

Potent Kinetic Stabilizers That Prevent Transthyretin-Mediated Cardiomyocyte Proteotoxicity

scientific article

Potent and selective structure-based dibenzofuran inhibitors of transthyretin amyloidogenesis: kinetic stabilization of the native state

scientific article published in May 2005

Prevention of transthyretin amyloid disease by changing protein misfolding energetics.

scientific article

Probing the folding transition state structure of the villin headpiece subdomain via side chain and backbone mutagenesis.

scientific article published on June 2009

Protein energetics in maturation of the early secretory pathway.

scientific article published on 7 August 2007

Protein native-state stabilization by placing aromatic side chains in N-glycosylated reverse turns

scientific article published on February 2011

Proteins downhill all the way

scientific article published in Nature

Quantification of the thermodynamically linked quaternary and tertiary structural stabilities of transthyretin and its disease-associated variants: the relationship between stability and amyloidosis.

scientific article published on 07 June 2008

Quantification of transthyretin kinetic stability in human plasma using subunit exchange.

scientific article published on 24 March 2014

Quantitative Interactome Proteomics Reveals a Molecular Basis for ATF6-Dependent Regulation of a Destabilized Amyloidogenic Protein

R104H may suppress transthyretin amyloidogenesis by thermodynamic stabilization, but not by the kinetic mechanism characterizing T119 interallelic trans-suppression

Recombinant Human Retinol-Binding Protein Refolding, Native Disulfide Formation, and Characterization ⬇️

scientific article published on October 1, 1998

Recombinant expression, purification, and comparative characterization of torsinA and its torsion dystonia-associated variant Delta E-torsinA.

scientific article published on December 2003

Reduced IGF-1 signaling delays age-associated proteotoxicity in mice

scientific article

Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis

scientific article

Regioselective phosphoranylation and cyclodehydration of triols with diethoxytriphenylphosphorane ⬇️

scientific article published on 01 November 1986

Regulated in Development and DNA Damage Response 1 Deficiency Impairs Autophagy and Mitochondrial Biogenesis in Articular Cartilage and Increases the Severity of Experimental Osteoarthritis

scientific article published on 23 March 2017

Residues Comprising the Enhanced Aromatic Sequon Influence Protein N-glycosylation Efficiency.

scientific article

Response

scientific article published on 04 December 2020

Retention of misfolded mutant transthyretin by the chaperone BiP/GRP78 mitigates amyloidogenesis

scientific article published on 5 December 2005

Screening transthyretin amyloid fibril inhibitors: characterization of novel multiprotein, multiligand complexes by mass spectrometry

scientific article

Secretion of amyloidogenic gelsolin progressively compromises protein homeostasis leading to the intracellular aggregation of proteins

scientific article

Semen-derived amyloid fibrils drastically enhance HIV infection

scientific article

Semi-quantitative models for identifying potent and selective transthyretin amyloidogenesis inhibitors

scientific article published on 26 May 2017

Sequence determinants of thermodynamic stability in a WW domain--an all-beta-sheet protein

scientific article published on August 2009

Sequence-dependent denaturation energetics: A major determinant in amyloid disease diversity

scientific article

Site-specific modification of Alzheimer's peptides by cholesterol oxidation products enhances aggregation energetics and neurotoxicity

scholarly article

Small molecule oxidation products trigger disease-associated protein misfolding.

scientific article published on September 2006

Small molecule probes to quantify the functional fraction of a specific protein in a cell with minimal folding equilibrium shifts

scientific article

Small molecule proteostasis regulators that reprogram the ER to reduce extracellular protein aggregation.

scientific article published on 20 July 2016

Solid-State NMR Studies Reveal Native-like β-Sheet Structures in Transthyretin Amyloid.

scientific article published on 07 September 2016

Solid-phase synthesis and stereochemical assignments of tenuecyclamides A-D employing heterocyclic amino acids derived from commercially available Fmoc alpha-amino acids

scientific article published in July 2004

Stilbene Vinyl Sulfonamides as Fluorogenic Sensors of and Traceless Covalent Kinetic Stabilizers of Transthyretin That Prevent Amyloidogenesis

scientific article

Stress-independent activation of XBP1s and/or ATF6 reveals three functionally diverse ER proteostasis environments

scientific article published on 11 April 2013

Structural and Energetic Basis of Carbohydrate–Aromatic Packing Interactions in Proteins

scientific article

Structural biology: form and function instructions

scientific article published on 01 September 2005

Structural insight into pH-induced conformational changes within the native human transthyretin tetramer

scientific article

Structure-based design of kinetic stabilizers that ameliorate the transthyretin amyloidoses

scientific article

Structure-function-folding relationship in a WW domain

scientific article

Sulfated glycosaminoglycans accelerate transthyretin amyloidogenesis by quaternary structural conversion

scientific article

Support for the multigenic hypothesis of amyloidosis: The binding stoichiometry of retinol-binding protein, vitamin A, and thyroid hormone influences transthyretin amyloidogenicity in vitro ⬇️

scientific article published on October 30, 2001

Surface adsorption considerations when working with amyloid fibrils in multiwell plates and Eppendorf tubes

scientific article published on 30 September 2013

Synthesis and Conformational Preferences of a Potential beta-Sheet Nucleator Based on the 9,9-Dimethylxanthene Skeleton

scientific article published on 01 October 1996

Synthesis and Hydrogen Bonding Capabilities of Biphenyl-Based Amino Acids Designed To Nucleate beta-Sheet Structure

scientific article published on 01 May 1996

Synthesis and characterization of potent bivalent amyloidosis inhibitors that bind prior to transthyretin tetramerization

scientific article

Synthesis of Sulfotyrosine-Containing Peptides by Incorporating Fluorosulfated Tyrosine Using an Fmoc-Based Solid-Phase Strategy.

scientific article published on 22 December 2015

Synthesis of a negatively charged dibenzofuran-based beta-turn mimetic and its incorporation into the WW miniprotein-enhanced solubility without a loss of thermodynamic stability

scientific article published in October 2002

Synthesis of all nineteen appropriately protected chiral alpha-hydroxy acid equivalents of the alpha-amino acids for Boc solid-phase depsi-peptide synthesis

scientific article published in February 2004

Synthesis, structure, and activity of diclofenac analogues as transthyretin amyloid fibril formation inhibitors

scientific article published in January 2002

Tafamidis for transthyretin familial amyloid polyneuropathy: a randomized, controlled trial

scientific article

Tafamidis, a potent and selective transthyretin kinetic stabilizer that inhibits the amyloid cascade

scientific article

Targeting protein aggregation for the treatment of degenerative diseases

scientific article

Targeting unfolded protein response signaling pathways to ameliorate protein misfolding diseases

scientific article published on 04 May 2013

Temporal requirements of insulin/IGF-1 signaling for proteotoxicity protection

scientific article published on 11 December 2009

The 8 and 5 kDa fragments of plasma gelsolin form amyloid fibrils by a nucleated polymerization mechanism, while the 68 kDa fragment is not amyloidogenic

scientific article

The Dependence of Carbohydrate-Aromatic Interaction Strengths on the Structure of the Carbohydrate

scientific article published on June 2016

The Kinetic Stability of a Full-Length Antibody Light Chain Dimer Determines whether Endoproteolysis Can Release Amyloidogenic Variable Domains

scientific article

The Role of Protein Thermodynamics and Primary Structure in Fibrillogenesis of Variable Domains from Immunoglobulin Light Chains

scientific article published on 14 August 2019

The V122I cardiomyopathy variant of transthyretin increases the velocity of rate-limiting tetramer dissociation, resulting in accelerated amyloidosis ⬇️

scientific article published on December 18, 2001

The alternative conformations of amyloidogenic proteins and their multi-step assembly pathways ⬇️

scientific article published on February 1, 1998

The biological and chemical basis for tissue-selective amyloid disease

scientific article

The core trisaccharide of an N-linked glycoprotein intrinsically accelerates folding and enhances stability

scholarly article

The effect of backbone cyclization on the thermodynamics of beta-sheet unfolding: stability optimization of the PIN WW domain

scientific article published in May 2002

The endoplasmic reticulum HSP40 co-chaperone ERdj3/DNAJB11 assembles and functions as a tetramer.

scientific article

The environmental dependency of protein folding best explains prion and amyloid diseases ⬇️

scientific article published on February 3, 1998

The integration of cell and chemical biology in protein folding

scientific article published in May 2006

The intrinsic and extrinsic effects of N-linked glycans on glycoproteostasis

scientific article

The oxidative stress metabolite 4-hydroxynonenal promotes Alzheimer protofibril formation

scientific article

The pathway by which the tetrameric protein transthyretin dissociates.

scientific article

The transthyretin amyloidoses: from delineating the molecular mechanism of aggregation linked to pathology to a regulatory-agency-approved drug

scientific article published on 05 January 2012

The two shapes of the Tau protein

The unfolded protein response regulator ATF6 promotes mesodermal differentiation.

scientific article published on 13 February 2018

Therapeutic strategies for human amyloid diseases

scientific article

Tissue damage in the amyloidoses: Transthyretin monomers and nonnative oligomers are the major cytotoxic species in tissue culture

scientific article

Toward Optimization of the Linker Substructure Common to Transthyretin Amyloidogenesis Inhibitors Using Biochemical and Structural Studies †

scientific article

Toward Optimization of the Second Aryl Substructure Common to Transthyretin Amyloidogenesis Inhibitors Using Biochemical and Structural Studies †

scientific article

Toward assessing the position-dependent contributions of backbone hydrogen bonding to beta-sheet folding thermodynamics employing amide-to-ester perturbations

scientific article published on 01 December 2004

Toward quantification of protein backbone-backbone hydrogen bonding energies: An energetic analysis of an amide-to-ester mutation in an alpha-helix within a protein

scientific article published on 23 April 2008

Toward the molecular mechanism(s) by which EGCG treatment remodels mature amyloid fibrils

scientific article published on 7 May 2013

Translation efficiency is maintained at elevated temperature in E. coli

scientific article

Translational attenuation differentially alters the fate of disease-associated fibulin proteins

scientific article

Transthyretin Quaternary and Tertiary Structural Changes Facilitate Misassembly into Amyloid ⬇️

scientific article published on January 1, 1997

Transthyretin aggregation under partially denaturing conditions is a downhill polymerization

scientific article

Treating the Periphery to Ameliorate Neurodegenerative Diseases ⬇️

scientific article published on June 10, 2011

Tuning the free-energy landscape of a WW domain by temperature, mutation, and truncation

scientific article

Understanding and Ameliorating the TTR Amyloidoses

Understanding the mechanism of beta-sheet folding from a chemical and biological perspective

scientific article

Unfolded protein response activation reduces secretion and extracellular aggregation of amyloidogenic immunoglobulin light chain

scientific article

Unfolded protein response-induced ERdj3 secretion links ER stress to extracellular proteostasis.

scientific article

Using Cooperatively Folded Peptides To Measure Interaction Energies and Conformational Propensities

scientific article published on 19 July 2017

Using sulfuramidimidoyl fluorides that undergo sulfur(VI) fluoride exchange for inverse drug discovery

scientific article published on 31 August 2020

X-ray structure of human acid-beta-glucosidase covalently bound to conduritol-B-epoxide. Implications for Gaucher disease

scientific article published on 6 April 2005

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