Unfolding the role of protein misfolding in neurodegenerative diseases

A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes

1993 • 7,941 citations

Mutation in the α-Synuclein Gene Identified in Families with Parkinson's Disease

1997 • 7,814 citations

Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis

1993 • 6,762 citations

Alzheimer's disease: Initial report of the purification and characterization of a novel cerebrovascular amyloid protein

1984 • 5,166 citations

Prions

1998 • 5,102 citations

Segregation of a missense mutation in the amyloid precursor protein gene with familial Alzheimer's disease

1991 • 4,533 citations

Naturally secreted oligomers of amyloid β protein potently inhibit hippocampal long-term potentiation in vivo

2002 • 4,135 citations

Physical basis of cognitive alterations in alzheimer's disease: Synapse loss is the major correlate of cognitive impairment

1991 • 4,120 citations

Motor Neuron Degeneration in Mice that Express a Human Cu,Zn Superoxide Dismutase Mutation

1994 • 3,959 citations

Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB)

1996 • 3,940 citations

Diffusible, nonfibrillar ligands derived from Aβ _1–42 are potent central nervous system neurotoxins

1998 • 3,404 citations

Immunization with amyloid-β attenuates Alzheimer-disease-like pathology in the PDAPP mouse

1999 • 3,112 citations

A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. The Huntington's Disease Collaborative Research Group.

1993 • 3,080 citations

Exon 1 of the HD Gene with an Expanded CAG Repeat Is Sufficient to Cause a Progressive Neurological Phenotype in Transgenic Mice

1996 • 3,012 citations

Aggregation of Huntingtin in Neuronal Intranuclear Inclusions and Dystrophic Neurites in Brain

1997 • 2,762 citations

Reactive microglia are positive for HLA‐DR in the substantia nigra of Parkinson's and Alzheimer's disease brains

1988 • 2,754 citations

Alzheimer-type neuropathology in transgenic mice overexpressing V717F β-amyloid precursor protein

1995 • 2,456 citations

Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseases

2002 • 2,424 citations

Conversion of alpha-helices into beta-sheets features in the formation of the scrapie prion proteins.

1993 • 2,237 citations

Formation of Neuronal Intranuclear Inclusions Underlies the Neurological Dysfunction in Mice Transgenic for the HD Mutation

1997 • 2,184 citations

Hydrogen peroxide mediates amyloid β protein toxicity

1994 • 2,065 citations

Protein misfolding, evolution and disease

1999 • 1,948 citations

Dopaminergic Loss and Inclusion Body Formation in α-Synuclein Mice: Implications for Neurodegenerative Disorders

2000 • 1,729 citations

Microtubule-associated protein tau. A component of Alzheimer paired helical filaments.

1986 • 1,709 citations

Common core structure of amyloid fibrils by synchrotron X-ray diffraction 1 1Edited by F. E. Cohen

1997 • 1,648 citations

Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein

1992 • 1,632 citations

Mice Lacking α-Synuclein Display Functional Deficits in the Nigrostriatal Dopamine System

2000 • 1,628 citations

Huntingtin Acts in the Nucleus to Induce Apoptosis but Death Does Not Correlate with the Formation of Intranuclear Inclusions

1998 • 1,592 citations

Apoptosis in neurodegenerative disorders

2000 • 1,542 citations

Aβ peptide vaccination prevents memory loss in an animal model of Alzheimer's disease

2000 • 1,498 citations

Accelerated in vitro fibril formation by a mutant α-synuclein linked to early-onset Parkinson disease

1998 • 1,460 citations

Neuropathology of Parkinsonʼs Disease

1996 • 1,457 citations

Aβ peptide immunization reduces behavioural impairment and plaques in a model of Alzheimer's disease

2000 • 1,433 citations

Molecular cloning of cDNA encoding an unrecognized component of amyloid in Alzheimer disease.

1993 • 1,426 citations

Treatment with a Copper-Zinc Chelator Markedly and Rapidly Inhibits β-Amyloid Accumulation in Alzheimer's Disease Transgenic Mice

2001 • 1,418 citations

Arthritis and anti-inflammatory agents as possible protective factors for Alzheimer's disease

1996 • 1,371 citations

Glutamine Repeats and Neurodegeneration

2000 • 1,309 citations

The inflammatory response system of brain: implications for therapy of Alzheimer and other neurodegenerative diseases

1995 • 1,290 citations

Huntingtin-Encoded Polyglutamine Expansions Form Amyloid-like Protein Aggregates In Vitro and In Vivo

1997 • 1,283 citations

Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury

1996 • 1,261 citations

Identification of a Protein That Purifies with the Scrapie Prion

1982 • 1,260 citations

Aggregation and Motor Neuron Toxicity of an ALS-Linked SOD1 Mutant Independent from Wild-Type SOD1

1998 • 1,207 citations

Clinical, pathological, and neurochemical changes in dementia: A subgroup with preserved mental status and numerous neocortical plaques

1988 • 1,186 citations

Inflammation in Neurodegenerative Disease—A Double-Edged Sword

2002 • 1,167 citations

Amyloid β-Protein Fibrillogenesis

1997 • 1,103 citations

Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases.

1994 • 1,065 citations

Reversal of Neuropathology and Motor Dysfunction in a Conditional Model of Huntington's Disease

2000 • 1,063 citations

Amyloid β-Protein Fibrillogenesis

1999 • 1,041 citations

Apoptosis is induced by beta-amyloid in cultured central nervous system neurons.

1993 • 1,035 citations

Ataxin-1 Nuclear Localization and Aggregation

1998 • 959 citations

Neurotoxicity of a prion protein fragment

1993 • 937 citations

Protofibrillar intermediates of amyloid beta-protein induce acute electrophysiological changes and progressive neurotoxicity in cortical neurons.

1999 • 917 citations

Relationship of microglia and astrocytes to amyloid deposits of Alzheimer disease

1989 • 904 citations

Alzheimer disease amyloid beta protein forms calcium channels in bilayer membranes: blockade by tromethamine and aluminum.

1993 • 901 citations

Nuclear and Neuropil Aggregates in Huntington’s Disease: Relationship to Neuropathology

1999 • 879 citations

β-sheet breaker peptides inhibit fibrillogenesis in a rat brain model of amyloidosis: Implications for Alzheimer's therapy

1998 • 839 citations

Linkage of a prion protein missense variant to Gerstmann–Sträussler syndrome

1989 • 834 citations

Protofibrillar Intermediates of Amyloid β-Protein Induce Acute Electrophysiological Changes and Progressive Neurotoxicity in Cortical Neurons

1999 • 797 citations

Amyloid fibrils from muscle myoglobin

2001 • 797 citations

Conformational disease

1997 • 794 citations

Fibrils Formed in Vitro from α-Synuclein and Two Mutant Forms Linked to Parkinson's Disease are Typical Amyloid

2000 • 767 citations

Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1

1998 • 730 citations

Metals and neuroscience

2000 • 724 citations

Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils: Implications for Huntington’s disease pathology

1999 • 716 citations

Fiber diffraction of synthetic α-synuclein filaments shows amyloid-like cross-β conformation

2000 • 714 citations

α-Synuclein Promotes Mitochondrial Deficit and Oxidative Stress

2000 • 694 citations

α-Synuclein Fibrillogenesis Is Nucleation-dependent

1999 • 686 citations

Early Phenotypic Changes in Transgenic Mice That Overexpress Different Mutants of Amyloid Precursor Protein in Brain

1999 • 664 citations

Electron Microscopic Observations on a Fibrous Component in Amyloid of Diverse Origins

1959 • 629 citations

Solution conformations and aggregational properties of synthetic amyloid β-peptides of Alzheimer's disease

1992 • 618 citations

Water-soluble Aβ(N-40, N-42) Oligomers in Normal and Alzheimer Disease Brains

1996 • 603 citations

Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice

2000 • 553 citations

Spontaneous Neurodegeneration in Transgenic Mice with Mutant Prion Protein

1990 • 531 citations

Immunologic reactions in amyotrophic lateral sclerosis brain and spinal cord tissue.

1992 • 531 citations

Targeted pharmacological depletion of serum amyloid P component for treatment of human amyloidosis

2002 • 527 citations

Early and Progressive Accumulation of Reactive Microglia in the Huntington Disease Brain

2001 • 495 citations

Assembly of Aβ Amyloid Protofibrils: An in Vitro Model for a Possible Early Event in Alzheimer's Disease

1999 • 460 citations

Is there a cause-and-effect relationship between α-synuclein fibrillization and Parkinson’s disease?

2000 • 455 citations

Amyloid-β immunotherapy for Alzheimer's disease: the end of the beginning

2002 • 437 citations

Prominent neurodegeneration and increased plaque formation in complement-inhibited Alzheimer's mice

2002 • 434 citations

Substitutions of hydrophobic amino acids reduce the amyloidogenicity of Alzheimer's disease βA4 peptides

1992 • 406 citations

Molecular Basis of the Neurodegenerative Disorders

1999 • 393 citations

Post-natal knockout of prion protein alters hippocampal CA1 properties, but does not result in neurodegeneration

2002 • 381 citations

Loss of normal huntingtin function: new developments in Huntington's disease research

2001 • 376 citations

Prion Protein Protects Human Neurons against Bax-mediated Apoptosis

2001 • 374 citations

A Critical Role for Amino-Terminal Glutamine/Asparagine Repeats in the Formation and Propagation of a Yeast Prion

1998 • 369 citations

Cellular prion protein transduces neuroprotective signals

2002 • 366 citations

Arresting amyloidosis in vivo using small-molecule anionic sulphonates or sulphates: implications for Alzheimer's disease

1995 • 358 citations

Aggregates from mutant and wild‐type α‐synuclein proteins and NAC peptide induce apoptotic cell death in human neuroblastoma cells by formation of β‐sheet and amyloid‐like filaments

1998 • 357 citations

Evidence for Seeding of β-Amyloid by Intracerebral Infusion of Alzheimer Brain Extracts in β-Amyloid Precursor Protein-Transgenic Mice

2000 • 356 citations

Amyloid-like Features of Polyglutamine Aggregates and Their Assembly Kinetics

2002 • 352 citations

A Long CAG Repeat in the Mouse Sca1 Locus Replicates SCA1 Features and Reveals the Impact of Protein Solubility on Selective Neurodegeneration

2002 • 343 citations

Inhibiting transthyretin amyloid fibril formation via protein stabilization

1996 • 338 citations

Receptor-dependent cell stress and amyloid accumulation in systemic amyloidosis

2000 • 337 citations

Genetic Classification of Primary Neurodegenerative Disease

1998 • 337 citations

Protein misfolding and disease; protein refolding and therapy

2001 • 335 citations

The α-Helical to β-Strand Transition in the Amino-terminal Fragment of the Amyloid β-Peptide Modulates Amyloid Formation

1995 • 319 citations

Prolines and Aamyloidogenicity in Fragments of the Alzheimer's Peptide .beta./A4

1995 • 306 citations

Chemical chaperones interfere with the formation of scrapie prion protein.

1996 • 297 citations

Polyglutamine protein aggregates are dynamic

2002 • 297 citations

Microtubule-Associated Protein Tau

1998 • 287 citations

Trans -Suppression of Misfolding in an Amyloid Disease

2001 • 283 citations

Structural and kinetic features of amyloid β-protein fibrillogenesis

1998 • 278 citations

Reversion of prion protein conformational changes by synthetic b-sheet breaker peptides

2000 • 275 citations

Transmissibility of systemic amyloidosis by a prion-like mechanism

2002 • 272 citations

The Lack of Accumulation of Senile Plaques or Amyloid Burden in Alzheimerʼs Disease Suggests a Dynamic Balance Between Amyloid Deposition and Resolution

1993 • 269 citations

Chemokines/chemokine receptors in the central nervous system and Alzheimer's disease

1999 • 263 citations

Therapeutic strategies for human amyloid diseases

2002 • 259 citations

The C‐Terminus of the β Protein is Critical in Amyloidogenesis^a

1993 • 250 citations

Prions as Protein-Based Genetic Elements

2002 • 249 citations

Synthetic peptides homologous to prion protein residues 106-147 form amyloid-like fibrils in vitro.

1993 • 241 citations

Anti‐amyloidogenic activity of tetracyclines: studies in vitro

2001 • 230 citations

In vitro formation of amyloid fibrils from two synthetic peptides of different lengths homologous to alzheimer's disease β-protein

1986 • 226 citations

Decreased Neuronal and Increased Oligodendroglial Densities in Huntington's Disease Caudate Nucleus

1991 • 225 citations

Aging-associated Changes in Human Brain

1997 • 218 citations

Nicotine Inhibits Amyloid Formation by the β-Peptide

1996 • 218 citations

Reduction of amyloid load and cerebral damage in transgenic mouse model of Alzheimer's disease by treatment with a β‐sheet breaker peptide

2002 • 216 citations

Immunocytochemical Co-localization of the Proteasome in Ubiquitinated Structures in Neurodegenerative Diseases and the Elderly

1997 • 214 citations

Wild-type but Not Parkinson's Disease-related Ala-53 → Thr Mutant α-Synuclein Protects Neuronal Cells from Apoptotic Stimuli

2000 • 211 citations

Pathology of amyotrophic lateral sclerosis.

1982 • 209 citations

Amyloid β and Amylin Fibrils Induce Increases in Proinflammatory Cytokine and Chemokine Production by THP‐1 Cells and Murine Microglia

2000 • 205 citations

Interaction of the anthracycline 4'-iodo-4'-deoxydoxorubicin with amyloid fibrils: inhibition of amyloidogenesis.

1995 • 202 citations

A cellular model that recapitulates major pathogenic steps of Huntington's disease

1998 • 192 citations

Channel Formation by a Neurotoxic Prion Protein Fragment

1997 • 191 citations

Immunization Delays the Onset of Prion Disease in Mice

2002 • 190 citations

Contribution of somal Lewy bodies to neuronal death

1997 • 178 citations

The cellular prion protein (PrP) selectively binds to Bcl-2 in the yeast two-hybrid system

1995 • 172 citations

Neuronal Apoptosis in Creutzfeldt-Jakob Disease

1999 • 161 citations

Molecular Structure of a Fibrillar Alzheimer's Aβ Fragment

2000 • 155 citations

Dominant-negative inhibition of prion replication in transgenic mice

2002 • 153 citations

Superoxide Dismutase 1 Subunits with Mutations Linked to Familial Amyotrophic Lateral Sclerosis Do Not Affect Wild-type Subunit Function

1995 • 149 citations

Cellular prion protein: on the road for functions

2002 • 140 citations

Amyloid fibril formation by a helical cytochrome

2001 • 124 citations

Microglial cells respond to amyloidogenic PrP peptide by the production of inflammatory cytokines

1999 • 117 citations

Protein engineering as a strategy to avoid formation of amyloid fibrils

2000 • 114 citations

Protein Misfolding and Disease

2003 • 113 citations

Transmission of Mouse Senile Amyloidosis

2001 • 109 citations

α-Helix Structure in Alzheimer's Disease Aggregates of Tau-Protein

2002 • 108 citations

Neurofibrillary degeneration and neuronal loss in alzheimer's disease

1989 • 106 citations

Reactive microgIia in Creutzfeldt‐Jakob disease

1995 • 98 citations

Soluble multimeric Alzheimer β(1–40) pre-amyloid complexes in dilute solution

1995 • 96 citations

[20] Analysis of circular dichroism spectra

1992 • 91 citations

Wild-type but not Alzheimer-mutant amyloid precursor protein confers resistance against p53-mediated apoptosis

1999 • 89 citations

Interventional strategies against prion diseases

2001 • 82 citations

Small is not beautiful: antagonizing functions for the prion protein PrPC and its homologue Dpl

2002 • 80 citations

Mice Deficient for the Amyloid Precursor Protein Gene

1996 • 74 citations

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

1997 • 70 citations

Compound heterozygotes of transthyretin Met30 and transthyretin Met119 are protected from the devastating effects of familial amyloid polyneuropathy

1996 • 62 citations

The Challenge of Inhibiting Aβ Polymerization

2002 • 41 citations

Neuron numbers in Alzheimer's disease: Cell-specific pathology

1987 • 9 citations