MOLECULAR PATHOPHYSIOLOGY OF PARKINSON'S DISEASE

Staging of brain pathology related to sporadic Parkinson’s disease

2002 • 9,557 citations

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

1997 • 7,814 citations

Mutations in the parkin gene cause autosomal recessive juvenile parkinsonism

1998 • 5,063 citations

Parkinson's Disease

2003 • 5,061 citations

Chronic Parkinsonism in Humans Due to a Product of Meperidine-Analog Synthesis

1983 • 4,822 citations

α-Synuclein Locus Triplication Causes Parkinson's Disease

2003 • 4,187 citations

The Ubiquitin-Proteasome Proteolytic Pathway: Destruction for the Sake of Construction

2002 • 4,069 citations

Chronic systemic pesticide exposure reproduces features of Parkinson's disease

2000 • 3,559 citations

Hereditary Early-Onset Parkinson's Disease Caused by Mutations in PINK1

2004 • 3,278 citations

α-Synuclein in filamentous inclusions of Lewy bodies from Parkinson’s disease and dementia with Lewy bodies

1998 • 2,898 citations

Mutations in LRRK2 Cause Autosomal-Dominant Parkinsonism with Pleomorphic Pathology

2004 • 2,880 citations

Mutations in the DJ-1 Gene Associated with Autosomal Recessive Early-Onset Parkinsonism

2003 • 2,691 citations

The new mutation, E46K, of α‐synuclein causes parkinson and Lewy body dementia

2003 • 2,591 citations

Cloning of the Gene Containing Mutations that Cause PARK8-Linked Parkinson's Disease

2004 • 2,358 citations

Impairment of the Ubiquitin-Proteasome System by Protein Aggregation

2001 • 2,167 citations

Mitochondrial Complex I Deficiency in Parkinson's Disease

1990 • 2,147 citations

A Drosophila model of Parkinson's disease

2000 • 2,062 citations

Oxidative stress in Parkinson's disease

2003 • 2,001 citations

Familial Parkinson disease gene product, parkin, is a ubiquitin-protein ligase

2000 • 1,975 citations

Parkinson's Disease

1998 • 1,953 citations

Aggresomes, inclusion bodies and protein aggregation

2000 • 1,881 citations

Impaired Degradation of Mutant α-Synuclein by Chaperone-Mediated Autophagy

2004 • 1,870 citations

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

2000 • 1,729 citations

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

2000 • 1,628 citations

Oxidative Damage Linked to Neurodegeneration by Selective α-Synuclein Nitration in Synucleinopathy Lesions

2000 • 1,526 citations

Stabilization of α-Synuclein Secondary Structure upon Binding to Synthetic Membranes

1998 • 1,507 citations

Acceleration of oligomerization, not fibrillization, is a shared property of both α-synuclein mutations linked to early-onset Parkinson's disease: Implications for pathogenesis and therapy

2000 • 1,497 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

Association between Early-Onset Parkinson's Disease and Mutations in theParkinGene

2000 • 1,398 citations

α-Synuclein Is Degraded by Both Autophagy and the Proteasome

2003 • 1,354 citations

Amyloid pores from pathogenic mutations

2002 • 1,260 citations

Chaperone Suppression of α-Synuclein Toxicity in a Drosophila Model for Parkinson's Disease

2002 • 1,220 citations

Mitochondrial pathology and apoptotic muscle degeneration in Drosophila parkin mutants

2003 • 1,156 citations

Parkinson's Disease

1998 • 1,150 citations

Kinetic Stabilization of the α-Synuclein Protofibril by a Dopamine-α-Synuclein Adduct

2001 • 1,116 citations

The Parkinson's disease protein DJ-1 is neuroprotective due to cysteine-sulfinic acid-driven mitochondrial localization

2004 • 1,091 citations

An Unfolded Putative Transmembrane Polypeptide, which Can Lead to Endoplasmic Reticulum Stress, Is a Substrate of Parkin

2001 • 1,048 citations

Ubiquitination of a New Form of α-Synuclein by Parkin from Human Brain: Implications for Parkinson's Disease

2001 • 1,036 citations

Parkin functions as an E2-dependent ubiquitin– protein ligase and promotes the degradation of the synaptic vesicle-associated protein, CDCrel-1

2000 • 975 citations

Mitochondrial Dysfunction and Oxidative Damage in parkin-deficient Mice

2004 • 959 citations

Parkin-deficient Mice Exhibit Nigrostriatal Deficits but Not Loss of Dopaminergic Neurons

2003 • 884 citations

The Neuron-Specific Protein PGP 9.5 Is a Ubiquitin Carboxyl-Terminal Hydrolase

1989 • 864 citations

Synucleins are developmentally expressed, and alpha-synuclein regulates the size of the presynaptic vesicular pool in primary hippocampal neurons.

2000 • 830 citations

Initiation and Synergistic Fibrillization of Tau and Alpha-Synuclein

2003 • 830 citations

DJ‐1 has a role in antioxidative stress to prevent cell death

2004 • 828 citations

Environmental Risk Factors and Parkinson's Disease: Selective Degeneration of Nigral Dopaminergic Neurons Caused by the Herbicide Paraquat

2002 • 804 citations

Yeast Cells Provide Insight into Alpha-Synuclein Biology and Pathobiology

2003 • 790 citations

The UCH-L1 Gene Encodes Two Opposing Enzymatic Activities that Affect α-Synuclein Degradation and Parkinson's Disease Susceptibility

2002 • 768 citations

Human α-synuclein-harboring familial Parkinson's disease-linked Ala-53 → Thr mutation causes neurodegenerative disease with α-synuclein aggregation in transgenic mice

2002 • 760 citations

Mapping of a Gene for Parkinson's Disease to Chromosome 4q21-q23

1996 • 759 citations

The synucleins: a family of proteins involved in synaptic function, plasticity, neurodegeneration and disease

1998 • 756 citations

S -Nitrosylation of Parkin Regulates Ubiquitination and Compromises Parkin's Protective Function

2004 • 750 citations

Parkin ubiquitinates the α-synuclein–interacting protein, synphilin-1: implications for Lewy-body formation in Parkinson disease

2001 • 738 citations

Molecular pathways to neurodegeneration

2004 • 732 citations

Synucleins Are Developmentally Expressed, and α-Synuclein Regulates the Size of the Presynaptic Vesicular Pool in Primary Hippocampal Neurons

2000 • 724 citations

Dopamine-dependent neurotoxicity of α-synuclein: A mechanism for selective neurodegeneration in Parkinson disease

2002 • 722 citations

A Manual of Diseases of the Nervous System.

1892 • 704 citations

Subcutaneous Rotenone Exposure Causes Highly Selective Dopaminergic Degeneration and α-Synuclein Aggregation

2002 • 695 citations

α-Synuclein Promotes Mitochondrial Deficit and Oxidative Stress

2000 • 694 citations

The Herbicide Paraquat Causes Up-regulation and Aggregation of α-Synuclein in Mice

2002 • 644 citations

A new locus for Parkinson's disease (PARK8) maps to chromosome 12p11.2–q13.1

2002 • 644 citations

Oxidative stress and nitration in neurodegeneration: Cause, effect, or association?

2003 • 634 citations

Origin and functional consequences of the complex I defect in Parkinson's disease

1996 • 629 citations

Subcellular Localization of Wild-Type and Parkinson's Disease-Associated Mutant α-Synuclein in Human and Transgenic Mouse Brain

2000 • 629 citations

Oxidative stress and nitration in neurodegeneration: Cause, effect, or association?

2003 • 626 citations

AnIn VitroModel of Parkinson's Disease: Linking Mitochondrial Impairment to Altered α-Synuclein Metabolism and Oxidative Damage

2002 • 575 citations

β-Amyloid peptides enhance α-synuclein accumulation and neuronal deficits in a transgenic mouse model linking Alzheimer's disease and Parkinson's disease

2001 • 573 citations

Resistance of α-synuclein null mice to the parkinsonian neurotoxin MPTP

2002 • 569 citations

Altered Proteasomal Function in Sporadic Parkinson's Disease

2002 • 565 citations

Parkin Protects against the Toxicity Associated with Mutant α-Synuclein

2002 • 552 citations

Mitochondrial Polymorphisms Significantly Reduce the Risk of Parkinson Disease

2003 • 543 citations

The Formation of Highly Soluble Oligomers of α-Synuclein Is Regulated by Fatty Acids and Enhanced in Parkinson's Disease

2003 • 541 citations

Systemic exposure to proteasome inhibitors causes a progressive model of Parkinson's disease

2004 • 531 citations

Intragenic deletion in the gene encoding ubiquitin carboxy-terminal hydrolase in gad mice

1999 • 505 citations

Parkin gene inactivation alters behaviour and dopamine neurotransmission in the mouse

2003 • 500 citations

Lipid Rafts Mediate the Synaptic Localization of α-Synuclein

2004 • 493 citations

Inducible expression of mutant alpha-synuclein decreases proteasome activity and increases sensitivity to mitochondria-dependent apoptosis

2001 • 490 citations

Synphilin-1 associates with α-synuclein and promotes the formation of cytosolic inclusions

1999 • 481 citations

PINK1 mutations are associated with sporadic early‐onset parkinsonism

2004 • 480 citations

Lewy bodies and parkinsonism in families with parkin mutations

2001 • 479 citations

Mutations in NR4A2 associated with familial Parkinson disease

2002 • 478 citations

A Protein-Chameleon: Conformational Plasticity of α-Synuclein, a Disordered Protein Involved in Neurodegenerative Disorders

2003 • 474 citations

Localization of a Novel Locus for Autosomal Recessive Early-Onset Parkinsonism, PARK6, on Human Chromosome 1p35-p36

2001 • 473 citations

CHIP Is Associated with Parkin, a Gene Responsible for Familial Parkinson's Disease, and Enhances Its Ubiquitin Ligase Activity

2002 • 470 citations

The Nigrostriatal Dopaminergic System as a Preferential Target of Repeated Exposures to Combined Paraquat and Maneb: Implications for Parkinson's Disease

2000 • 462 citations

A susceptibility locus for Parkinson's disease maps to chromosome 2p13

1998 • 461 citations

The expression of DJ-1 (PARK7) in normal human CNS and idiopathic Parkinson's disease

2004 • 454 citations

Direct binding and functional coupling of α-synuclein to the dopamine transporters accelerate dopamine-induced apoptosis

2001 • 445 citations

Lipid Droplet Binding and Oligomerization Properties of the Parkinson's Disease Protein α-Synuclein

2002 • 429 citations

Regulation of Phospholipase D2: Selective Inhibition of Mammalian Phospholipase D Isoenzymes by α- and β-Synucleins

1998 • 427 citations

Drosophila parkinmutants have decreased mass and cell size and increased sensitivity to oxygen radical stress

2004 • 425 citations

Aggregated and Monomeric α-Synuclein Bind to the S6′ Proteasomal Protein and Inhibit Proteasomal Function

2003 • 407 citations

Degradation of α-Synuclein by Proteasome

1999 • 406 citations

β-Synuclein Inhibits α-Synuclein Aggregation

2001 • 401 citations

Phosphorylated α-Synuclein Is Ubiquitinated in α-Synucleinopathy Lesions

2002 • 400 citations

Aggresomes Formed by α-Synuclein and Synphilin-1 Are Cytoprotective

2004 • 398 citations

Parkin prevents mitochondrial swelling and cytochrome c release in mitochondria-dependent cell death

2003 • 388 citations

Parkin Is a Component of an SCF-like Ubiquitin Ligase Complex and Protects Postmitotic Neurons from Kainate Excitotoxicity

2003 • 385 citations

Localization of a gene for an autosomal recessive form of juvenile Parkinsonism to chromosome 6q25.2-27.

1997 • 372 citations

PARK7, a Novel Locus for Autosomal Recessive Early-Onset Parkinsonism, on Chromosome 1p36

2001 • 371 citations

Proteasomal Inhibition by α-Synuclein Filaments and Oligomers

2004 • 368 citations

Pathologic and biochemical studies of juvenile parkinsonism linked to chromosome 6q

1998 • 366 citations

Parkin Suppresses Dopaminergic Neuron-Selective Neurotoxicity Induced by Pael-R in Drosophila

2003 • 359 citations

Down regulation of DJ-1 enhances cell death by oxidative stress, ER stress, and proteasome inhibition

2003 • 358 citations

Parkinson's disease in 65 pairs of twins and in a set of quadruplets

1983 • 353 citations

Ubiquitin carboxyl‐terminal hydrolase (PGP 9.5) is selectively present in ubiquitinated inclusion bodies characteristic of human neurodegenerative diseases

1990 • 351 citations

α‐Synuclein metabolism and aggregation is linked to ubiquitin‐independent degradation by the proteasome

2001 • 350 citations

Ubiquitin carboxy-terminal hydrolase L1 binds to and stabilizes monoubiquitin in neuron

2003 • 348 citations

Effect of Mutant α-Synuclein on Dopamine Homeostasis in a New Human Mesencephalic Cell Line

2002 • 339 citations

Loss of locus coeruleus neurons and reduced startle in parkin null mice

2004 • 336 citations

Misfolded proteinase K–resistant hyperphosphorylated α-synuclein in aged transgenic mice with locomotor deterioration and in human α-synucleinopathies

2002 • 325 citations

Lewy-body formation is an aggresome-related process: a hypothesis

2004 • 305 citations

The 1.1-Å resolution crystal structure of DJ-1, the protein mutated in autosomal recessive early onset Parkinson's disease

2003 • 300 citations

Enhanced substantia nigra mitochondrial pathology in human α-synuclein transgenic mice after treatment with MPTP111-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine.

2003 • 291 citations

Complete Genomic Screen in Parkinson Disease

2001 • 289 citations

Parkin binds to alpha/beta tubulin and increases their ubiquitination and degradation.

2003 • 282 citations

Misfolded proteinase K–resistant hyperphosphorylated α-synuclein in aged transgenic mice with locomotor deterioration and in human α-synucleinopathies

2002 • 277 citations

Familial Aggregation of Parkinson's Disease in Iceland

2000 • 274 citations

Golgi Fragmentation Occurs in the Cells with Prefibrillar α-Synuclein Aggregates and Precedes the Formation of Fibrillar Inclusion

2002 • 273 citations

Familial Parkinson's Disease-associated L166P Mutation Disrupts DJ-1 Protein Folding and Function

2004 • 269 citations

Functional Consequences of α-Synuclein Tyrosine Nitration

2004 • 266 citations

Autosomal dominant parkinsonism associated with variable synuclein and tau pathology

2004 • 262 citations

Synphilin-1 is present in Lewy bodies in Parkinson's disease

2000 • 261 citations

Oxidized forms of peroxiredoxins and DJ-1 on two-dimensional gels increased in response to sublethal levels of paraquat

2001 • 258 citations

UCHL1 is a Parkinson's disease susceptibility gene

2004 • 257 citations

A susceptibility gene for late‐onset idiopathic Parkinson's disease

2002 • 254 citations

Crystal Structure of Human DJ-1, a Protein Associated with Early Onset Parkinson's Disease

2003 • 253 citations

Risk of Parkinson's disease among first-degree relatives

1996 • 252 citations

Parkin Binds to α/β Tubulin and Increases their Ubiquitination and Degradation

2003 • 247 citations

Association of DJ-1 and parkin mediated by pathogenic DJ-1 mutations and oxidative stress

2004 • 245 citations

Parkin protects human dopaminergic neuroblastoma cells against dopamine-induced apoptosis

2004 • 241 citations

Parkin binds the Rpn10 subunit of 26S proteasomes through its ubiquitin‐like domain

2003 • 240 citations

Crystal Structures of Human DJ-1 and Escherichia coli Hsp31, Which Share an Evolutionarily Conserved Domain

2003 • 239 citations

The p38 subunit of the aminoacyl-tRNA synthetase complex is a Parkin substrate: linking protein biosynthesis and neurodegeneration

2003 • 239 citations

Selective Insolubility of α-Synuclein in Human Lewy Body Diseases Is Recapitulated in a Transgenic Mouse Model

2001 • 238 citations

L166P Mutant DJ-1, Causative for Recessive Parkinson's Disease, Is Degraded through the Ubiquitin-Proteasome System

2003 • 234 citations

Are Ubiquitination Pathways Central to Parkinson's Disease?

2003 • 232 citations

Effect of the overexpression of wild‐type or mutant α‐synuclein on cell susceptibility to insult

2001 • 227 citations

Novel PINK1 mutations in early-onset parkinsonism

2005 • 226 citations

Environment, Mitochondria, and Parkinson's Disease

2002 • 224 citations

The role of the ubiquitin-proteasomal pathway in Parkinson's disease and other neurodegenerative disorders

2001 • 221 citations

A missense mutation (L166P) in DJ‐1, linked to familial Parkinson's disease, confers reduced protein stability and impairs homo‐oligomerization

2003 • 220 citations

Selective loss of 20S proteasome α-subunits in the substantia nigra pars compacta in Parkinson's disease

2002 • 219 citations

The PARK8 Locus in Autosomal Dominant Parkinsonism: Confirmation of Linkage and Further Delineation of the Disease-Containing Interval

2004 • 218 citations

The autosomal recessive juvenile Parkinson disease gene product, parkin, interacts with and ubiquitinates synaptotagmin XI

2003 • 217 citations

Case-control study of the ubiquitin carboxy-terminal hydrolase L1 gene in Parkinson’s disease

1999 • 214 citations

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

1997 • 214 citations

Rare genetic mutations shed light on the pathogenesis of Parkinson disease

2003 • 212 citations

α‐Synuclein promoter confers susceptibility to Parkinson's disease

2004 • 209 citations

Characterization of the Precursor Protein of the Non-Aβ Component of Senile Plaques (NACP) in the Human Central Nervous System

1996 • 202 citations

Proteasome Inhibition Alters Neural Mitochondrial Homeostasis and Mitochondria Turnover

2004 • 201 citations

Expression of mutant alpha-synuclein causes increased susceptibility to dopamine toxicity

2000 • 198 citations

Parkin genetics: one model for Parkinson's disease

2004 • 192 citations

Rare genetic mutations shed light on the pathogenesis of Parkinson disease

2003 • 183 citations

Stabilization of α-Synuclein Protein with Aging and Familial Parkinson's Disease-Linked A53T Mutation

2004 • 171 citations

β-Synuclein gene alterations in dementia with Lewy bodies

2004 • 168 citations

Significant Linkage of Parkinson Disease to Chromosome 2q36-37

2003 • 156 citations

Reduced anti-oxidative stress activities of DJ-1 mutants found in Parkinson’s disease patients

2004 • 156 citations

Distinct cleavage patterns of normal and pathologic forms of α‐synuclein by calpain I in vitro

2003 • 153 citations

Identification and functional characterization of a novel R621C mutation in the synphilin-1 gene in Parkinson's disease

2003 • 149 citations

Pathological properties of the Parkinson?s disease-associated protein DJ-1 in a-synucleinopathies and tauopathies: relevance for multiple system atrophy and Pick?s disease

2004 • 149 citations

Matrilineal inheritance of complex I dysfunction in a multigenerational Parkinson's disease family

1998 • 146 citations

DJ‐1 colocalizes with tau inclusions: A link between parkinsonism and dementia

2003 • 142 citations

Pael‐R is accumulated in Lewy bodies of Parkinson's disease

2004 • 140 citations

Linking DJ-1 to neurodegeneration offers novel insights for understanding the pathogenesis of Parkinson?s disease

2004 • 137 citations

Parkinson's disease in a nationwide twin cohort

1988 • 135 citations

Parkin-associated Parkinson’s disease

2004 • 133 citations

Dopamine-dependent neurodegeneration in rats induced by viral vector-mediated overexpression of the parkin target protein, CDCrel-1

2003 • 129 citations

Genetics of Parkinson's disease and biochemical studies of implicated gene products

2002 • 119 citations

Low frequency of pathogenic mutations in the ubiquitin carboxyterminal hydrolase gene in familial Parkinsonʼs disease

1999 • 119 citations

Genes and parkinsonism

2003 • 114 citations

Parkin

2003 • 112 citations

PARK6-linked parkinsonism occurs in several European families.

2002 • 112 citations

The 1.6-Å crystal structure of the class of chaperones represented by Escherichia coli Hsp31 reveals a putative catalytic triad

2003 • 107 citations

Park6‐linked parkinsonism occurs in several european families

2001 • 106 citations

Maternal inheritance in Parkinson's disease

1997 • 95 citations

Genetics of parkin-linked disease

2004 • 93 citations

Sensitization of Neuronal Cells to Oxidative Stress with Mutated Human α‐Synuclein

2000 • 89 citations

The role of the ubiquitin-proteasomal pathway in Parkinson's disease and other neurodegenerative disorders

2001 • 85 citations

An electron microscopic study of MPTP-induced inclusion bodies in an old monkey

1988 • 84 citations

Genetics of Parkinson's disease and biochemical studies of implicated gene products: Commentary

2002 • 67 citations

Role for the Ubiquitin-Proteasome System in Parkinson's Disease and Other Neurodegenerative Brain Amyloidoses

2003 • 59 citations

α-Synuclein Is Required for the Fibrillar Nature of Ubiquitinated Inclusions Induced by Proteasomal Inhibition in Primary Neurons

2004 • 51 citations

Biochemical Analysis of Cybrids Expressing Mitochondrial DNA from Contursi Kindred Parkinson's Subjects

2001 • 44 citations

The gene responsible for PARK6 Parkinson's disease, PINK1, does not influence common forms of parkinsonism

2004 • 43 citations

A PEDIGREE OF PARALYSIS AGITANS

1926 • 40 citations

Polymorphism in the human DJ-1 gene is not associated with sporadic dementia with Lewy bodies or Parkinson's disease

2003 • 38 citations

Linkage exclusion in French families with probable Parkinson's disease

2000 • 33 citations