Hydration and Packing are Crucial to Amyloidogenesis as Revealed by Pressure Studies on Transthyretin Variants that Either Protect or Worsen Amyloid Disease

Amyloid fibrillogenesis: themes and variations

2000 • 1,082 citations

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

1998 • 996 citations

Structure of prealbumin: Secondary, tertiary and quaternary interactions determined by Fourier refinement at 1.8 Å

1978 • 784 citations

Fibril in senile systemic amyloidosis is derived from normal transthyretin.

1990 • 683 citations

Protein-solvent preferential interactions, protein hydration, and the modulation of biochemical reactions by solvent components

2002 • 664 citations

The pressure dependence of hydrophobic interactions is consistent with the observed pressure denaturation of proteins

1998 • 577 citations

The Acid-Mediated Denaturation Pathway of Transthyretin Yields a Conformational Intermediate That Can Self-Assemble into Amyloid

1996 • 546 citations

Pressure Stability of Proteins

1993 • 515 citations

Amyloid fibers are water-filled nanotubes

2002 • 509 citations

Amyloid fibril protein in familial amyloidotic polyneuropathy, Portuguese type. Definition of molecular abnormality in transthyretin (prealbumin).

1984 • 427 citations

Pressure provides new insights into protein folding, dynamics and structure

2001 • 382 citations

Trans -Suppression of Misfolding in an Amyloid Disease

2001 • 283 citations

Structure of human plasma prealbumin at 2.5 A resolution

1974 • 272 citations

Manipulating the Amyloid-β Aggregation Pathway with Chemical Chaperones

1999 • 264 citations

A comparative analysis of 23 structures of the amyloidogenic protein transthyretin

2000 • 228 citations

Reaction volume of protonic ionization for buffering agents. Prediction of pressure dependence of pH and pOH

1987 • 216 citations

Transthyretin mutation Leu-55-Pro significantly alters tetramer stability and increases amyloidogenicity

1993 • 201 citations

Characterization of the Transthyretin Acid Denaturation Pathways by Analytical Ultracentrifugation: Implications for Wild-Type, V30M, and L55P Amyloid Fibril Formation

1998 • 199 citations

The preaggregated state of an amyloidogenic protein: Hydrostatic pressure converts native transthyretin into the amyloidogenic state

2000 • 196 citations

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

1995 • 195 citations

Comparative Fourier Transform Infrared Spectroscopy Study of Cold-, Pressure-, and Heat-Induced Unfolding and Aggregation of Myoglobin

2002 • 186 citations

The Tetrameric Protein Transthyretin Dissociates to a Non-native Monomer in Solution

1999 • 185 citations

High pressure fosters protein refolding from aggregates at high concentrations

1999 • 178 citations

The Most Pathogenic Transthyretin Variant, L55P, Forms Amyloid Fibrils under Acidic Conditions and Protofilaments under Physiological Conditions

1999 • 172 citations

High-Pressure NMR Spectroscopy of Proteins and Membranes

1994 • 167 citations

For Protein Misassembly, It's the “I” Decade

1996 • 153 citations

A glimpse of a possible amyloidogenic intermediate of transthyretin.

2000 • 139 citations

From Computer Simulations to Human Disease

1999 • 134 citations

Familial amyloidotic polyneuropathy

1989 • 124 citations

Pressure-induced protein-folding/unfolding kinetics

1999 • 114 citations

The role of conformational flexibility in prion propagation and maintenance for Sup35p.

2001 • 104 citations

Arc Repressor will not Denature Under Pressure in the Absence of Water

1994 • 100 citations

Transthyretin slowly exchanges subunits under physiological conditions: A convenient chromatographic method to study subunit exchange in oligomeric proteins

2001 • 96 citations

High Hydrostatic Pressure Can Reverse Aggregation of Protein Folding Intermediates and Facilitate Acquisition of Native Structure

1998 • 96 citations

Hydrostatic pressure rescues native protein from aggregates

1999 • 92 citations

Transthyretin stability as a key factor in amyloidogenesis: X-ray analysis at atomic resolution

2001 • 90 citations

The β-Slip

2000 • 89 citations

Review: TTR Amyloidosis—Structural Features Leading to Protein Aggregation and Their Implications on Therapeutic Strategies

2000 • 83 citations

Anomalous Pressure Dissociation of Large Protein Aggregates

1989 • 79 citations

Familial amyloid polyneuropathy: new developments in genetics and treatment.

1996 • 78 citations

Identification of a Subunit Interface in Transthyretin Amyloid Fibrils: Evidence for Self-Assembly from Oligomeric Building Blocks

2001 • 74 citations

Familial amyloid polyneuropathy

1996 • 70 citations

Comparative Stability and Clearance of [Met30]Transthyretin and [Met119]Transthyretin

1997 • 70 citations

Capture of a Dimeric Intermediate during Transthyretin Amyloid Formation

2001 • 53 citations

Decreased Thermodynamic Stability as a Crucial Factor for Familial Amyloidotic Polyneuropathy

2002 • 47 citations

Native State Hydrogen Exchange Study of Suppressor and Pathogenic Variants of Transthyretin

2002 • 40 citations

Designing transthyretin mutants affecting tetrameric structure: implications in amyloidogenicity

2000 • 39 citations

Thyroxine interactions with transthyretin: a comparison of 10 different naturally occurring human transthyretin variants.

1993 • 33 citations

Polymorphism of Rhesus Monkey Serum Prealbumin

1973 • 26 citations

Designing transthyretin mutants affecting tetrameric structure: implications in amyloidogenicity

2000 • 11 citations

Thyroxine interactions with transthyretin: a comparison of 10 different naturally occurring human transthyretin variants

1993 • 10 citations