The ubiquitin protein catabolic disorders

THE UBIQUITIN SYSTEM

1998 • 8,360 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

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

1997 • 2,762 citations

The HPV-16 E6 and E6-AP complex functions as a ubiquitin-protein ligase in the ubiquitination of p53

1993 • 2,329 citations

Aggresomes: A Cellular Response to Misfolded Proteins

1998 • 2,116 citations

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

2000 • 1,975 citations

Recognition of the polyubiquitin proteolytic signal

2000 • 1,651 citations

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

1998 • 1,592 citations

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

1998 • 1,460 citations

Amyloid, the presenilins and Alzheimer's disease

1997 • 1,370 citations

mdm2 expression is induced by wild type p53 activity.

1993 • 1,286 citations

Degradation of CFTR by the ubiquitin-proteasome pathway

1995 • 1,285 citations

UBE3A/E6-AP mutations cause Angelman syndrome

1997 • 1,270 citations

RING fingers mediate ubiquitin-conjugating enzyme (E2)-dependent ubiquitination

1999 • 1,134 citations

De novo truncating mutations in E6-AP ubiquitin-protein ligase gene (UBE3A) in Angelman syndrome

1997 • 847 citations

Impaired Proteasome Function in Alzheimer's Disease

2000 • 795 citations

Parkin Suppresses Unfolded Protein Stress-induced Cell Death through Its E3 Ubiquitin-protein Ligase Activity

2000 • 757 citations

Proteasomal function is impaired in substantia nigra in Parkinson's disease

2001 • 631 citations

Frameshift Mutants of β Amyloid Precursor Protein and Ubiquitin-B in Alzheimer's and Down Patients

1998 • 558 citations

Ubiquitin is a common factor in intermediate filament inclusion bodies of diverse type in man, including those of Parkinson's disease, Pick's disease, and Alzheimer's disease, as well as Rosenthal fibres in cerebellar astrocytomas, cytoplasmic bodies in muscle, and mallory bodies in alcoholic liver disease

1988 • 541 citations

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

1999 • 505 citations

Presenilins are required for γ-secretase cleavage of β-APP and transmembrane cleavage of Notch-1

2000 • 407 citations

Degradation of α-Synuclein by Proteasome

1999 • 406 citations

The von Hippel-Lindau tumor suppressor protein is a component of an E3 ubiquitin-protein ligase activity

1999 • 377 citations

Huntingtin Is Ubiquitinated and Interacts with a Specific Ubiquitin-conjugating Enzyme

1996 • 365 citations

Effects of heat shock, heat shock protein 40 (HDJ-2), and proteasome inhibition on protein aggregation in cellular models of Huntington's disease

2000 • 360 citations

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

1990 • 351 citations

Inhibition of the ubiquitin-proteasome system in Alzheimer's disease

2000 • 330 citations

Ubiquitin is conjugated with amino-terminally processed tau in paired helical filaments

1993 • 312 citations

In vivo disassembly of free polyubiquitin chains by yeast Ubp14 modulates rates of protein degradation by the proteasome

1997 • 235 citations

Siah-1 N-Terminal RING Domain Is Required for Proteolysis Function, and C-Terminal Sequences Regulate Oligomerization and Binding to Target Proteins

1999 • 234 citations

Motor Neurone Disease-inclusion Dementia

1996 • 224 citations

Role of I Bα Ubiquitination in Signal-induced Activation of NF- B in Vivo

1996 • 216 citations

Defective ubiquitination of cerebral proteins in Alzheimer's disease

2000 • 216 citations

αB crystallin expression in nonlenticular tissues and selective presence in ubiquitinated inclusion bodies in human disease

1992 • 206 citations

Lessons from Keratin 18 Knockout Mice: Formation of Novel Keratin Filaments, Secondary Loss of Keratin 7 and Accumulation of Liver-specific Keratin 8-Positive Aggregates

1998 • 205 citations

Expression of ?-synuclein, parkin, and ubiquitin carboxy-terminal hydrolase L1 mRNA in human brain: Genes associated with familial Parkinson's disease

2000 • 195 citations

Ubiquitin in Neurodegenerative Diseases

1993 • 171 citations

Proteasomal Degradation and N-terminal Protease Resistance of the Codon 145 Mutant Prion Protein

1999 • 165 citations

Protein precipitation: a common etiology in neurodegenerative disorders?

1998 • 158 citations

Tau gene mutations in frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17).

2000 • 120 citations

α-Synuclein and the Parkinson's disease-related mutant Ala53Thr-α-synuclein do not undergo proteasomal degradation in HEK293 and neuronal cells

2000 • 108 citations

The stabilization mechanism of mutant-type p53 by impaired ubiquitination: the loss of wild-type p53 function and the hsp90 association

1999 • 101 citations

Tau gene mutations in frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17)

2000 • 48 citations

The latent membrane protein‐1 in Epstein‐Barr virus‐transformed lymphoblastoid cells is found with ubiquitin‐protein conjugates and heat‐shock protein 70 in lysosomes oriented around the microtubule organizing centre

1991 • 46 citations

Autosomal recessive juvenile parkinsonism: A key to understanding nigral degeneration in sporadic Parkinson's disease

2000 • 33 citations

Neurofibrillary tangles in progressive supranuclear palsy brains exhibit immunoreactivity to frameshift mutant ubiquitin-B protein

2000 • 31 citations

C-Terminal Maturation Fragments of Presenilin 1 and 2 Control Secretion of APPα and Aβ by Human Cells and Are Degraded by Proteasome

1999 • 29 citations

Molecular misreading in non‐neuronal cells

2000 • 27 citations

Molecular misreading in non-neuronal cells

2000 • 13 citations

Does an inhibition of the ubiquitin/26S proteasome pathway of protein degradation underlie the pathogenesis of non-familial Alzheimer’s disease?

2001 • 10 citations