The ubiquitin system: pathogenesis of human diseases and drug targeting

CDK inhibitors: positive and negative regulators of G1-phase progression

1999 • 6,003 citations

Targeting of HIF-α to the von Hippel-Lindau Ubiquitylation Complex by O ₂ -Regulated Prolyl Hydroxylation

2001 • 5,255 citations

Mutations in the parkin gene cause autosomal recessive juvenile parkinsonism

1998 • 5,063 citations

The tumour suppressor protein VHL targets hypoxia-inducible factors for oxygen-dependent proteolysis

1999 • 4,973 citations

Phosphorylation Meets Ubiquitination: The Control of NF-κB Activity

2000 • 4,644 citations

HIFα Targeted for VHL-Mediated Destruction by Proline Hydroxylation: Implications for O ₂ Sensing

2001 • 4,537 citations

The E6 oncoprotein encoded by human papillomavirus types 16 and 18 promotes the degradation of p53

1990 • 3,896 citations

Identification of Ubiquitin Ligases Required for Skeletal Muscle Atrophy

2001 • 3,292 citations

A Phase 2 Study of Bortezomib in Relapsed, Refractory Myeloma

2003 • 2,642 citations

Akt/mTOR pathway is a crucial regulator of skeletal muscle hypertrophy and can prevent muscle atrophy in vivo

2001 • 2,539 citations

A Conserved Family of Prolyl-4-Hydroxylases That Modify HIF

2001 • 2,481 citations

Ischemia- and cytokine-induced mobilization of bone marrow-derived endothelial progenitor cells for neovascularization

1999 • 2,425 citations

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

1993 • 2,329 citations

Impairment of the Ubiquitin-Proteasome System by Protein Aggregation

2001 • 2,167 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

Atrogin-1, a muscle-specific F-box protein highly expressed during muscle atrophy

2001 • 1,714 citations

Caught up in a Wnt storm: Wnt signaling in cancer

2003 • 1,473 citations

Ubiquitin-Dependent Sorting into the Multivesicular Body Pathway Requires the Function of a Conserved Endosomal Protein Sorting Complex, ESCRT-I

2001 • 1,373 citations

UBE3A/E6-AP mutations cause Angelman syndrome

1997 • 1,270 citations

RING Finger Proteins

2000 • 1,216 citations

Stabilization of β-Catenin by Genetic Defects in Melanoma Cell Lines

1997 • 1,215 citations

Interaction of the Fanconi Anemia Proteins and BRCA1 in a Common Pathway

2001 • 1,206 citations

Orchestrating the unfolded protein response in health and disease

2002 • 1,163 citations

Mechanisms of Muscle Wasting — The Role of the Ubiquitin–Proteasome Pathway

1996 • 1,149 citations

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

1999 • 1,134 citations

Complementation Cloning of NEMO, a Component of the IκB Kinase Complex Essential for NF-κB Activation

1998 • 1,070 citations

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

2001 • 1,048 citations

The Tyrosine Kinase Negative Regulator c-Cbl as a RING-Type, E2-Dependent Ubiquitin-Protein Ligase

1999 • 1,045 citations

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

2001 • 1,036 citations

A Novel Ubiquitination Factor, E4, Is Involved in Multiubiquitin Chain Assembly

1999 • 1,031 citations

The tumour suppressor CYLD negatively regulates NF-κB signalling by deubiquitination

2003 • 983 citations

Cellular Defenses against Unfolded Proteins

2001 • 979 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

Orchestrating the unfolded protein response in health and disease

2002 • 966 citations

Ubiquitin-dependent c-Jun degradation in vivo is mediated by the δ domain

1994 • 930 citations

DEGRADATION OF CELL PROTEINS AND THE GENERATION OF MHC CLASS I-PRESENTED PEPTIDES

1999 • 898 citations

c-Cbl/Sli-1 regulates endocytic sorting and ubiquitination of the epidermal growth factor receptor

1998 • 865 citations

Inhibition of antigen processing by the internal repeat region of the Epstein–Barr virus nuclear antigen-1

1995 • 820 citations

Hakai, a c-Cbl-like protein, ubiquitinates and induces endocytosis of the E-cadherin complex

2002 • 817 citations

X-linked anhidrotic ectodermal dysplasia with immunodeficiency is caused by impaired NF-κB signaling

2001 • 806 citations

Rbx1, a Component of the VHL Tumor Suppressor Complex and SCF Ubiquitin Ligase

1999 • 771 citations

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

2002 • 768 citations

The p53–Mdm2 module and the ubiquitin system

2002 • 759 citations

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

2001 • 738 citations

Muscle Protein Breakdown and the Critical Role of the Ubiquitin-Proteasome Pathway in Normal and Disease States

1999 • 734 citations

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

2002 • 722 citations

Phosphorylation of Nedd4-2 by Sgk1 regulates epithelial Na+ channel cell surface expression

2001 • 699 citations

Axin-mediated CKI phosphorylation of β-catenin at Ser 45: a molecular switch for the Wnt pathway

2002 • 698 citations

Identification of the familial cylindromatosis tumour-suppressor gene

2000 • 654 citations

Identification of the receptor component of the IκBα–ubiquitin ligase

1998 • 648 citations

The RING Heterodimer BRCA1-BARD1 Is a Ubiquitin Ligase Inactivated by a Breast Cancer-derived Mutation

2001 • 623 citations

Antigen processing by the proteasome

2001 • 620 citations

Formation of high molecular weight complexes of mutant Cu,Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis

2000 • 604 citations

Cbl–CIN85–endophilin complex mediates ligand-induced downregulation of EGF receptors

2002 • 571 citations

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

1998 • 558 citations

Parkin Protects against the Toxicity Associated with Mutant α-Synuclein

2002 • 552 citations

An F-box protein, FWD1, mediates ubiquitin-dependent proteolysis of β-catenin

1999 • 525 citations

Mutation of the E6-AP Ubiquitin Ligase Reduces Nuclear Inclusion Frequency While Accelerating Polyglutamine-Induced Pathology in SCA1 Mice

1999 • 507 citations

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

1999 • 505 citations

Polyglutamine Pathogenesis

2002 • 505 citations

Skp2 is oncogenic and overexpressed in human cancers

2001 • 498 citations

Imprinted expression of the murine Angelman syndrome gene, Ube3a, in hippocampal and Purkinje neurons

1997 • 495 citations

What do we really know about the ubiquitin-proteasome pathway in muscle atrophy?

2001 • 412 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

Biosynthesis and Degradation of CFTR

1999 • 406 citations

Inactivation of the HR6B Ubiquitin-Conjugating DNA Repair Enzyme in Mice Causes Male Sterility Associated with Chromatin Modification

1996 • 402 citations

Ubiquitin, cellular inclusions and their role in neurodegeneration

1998 • 396 citations

The yeast DOA4 gene encodes a deubiquitinating enzyme related to a product of the human tre-2 oncogene

1993 • 379 citations

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

1999 • 377 citations

Deregulated degradation of the cdk inhibitor p27 and malignant transformation

2002 • 372 citations

The septin CDCrel-1 binds syntaxin and inhibits exocytosis

1999 • 371 citations

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

1996 • 365 citations

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

2003 • 359 citations

Regulation of microtubule stability by the von Hippel-Lindau tumour suppressor protein pVHL

2002 • 330 citations

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

2000 • 330 citations

The itchy locus encodes a novel ubiquitin protein ligase that is disrupted in a18H mice

1998 • 328 citations

Recognition and Ubiquitination of Notch by Itch, a Hect-type E3 Ubiquitin Ligase

2000 • 321 citations

An Essential Role of N-Terminal Arginylation in Cardiovascular Development

2002 • 313 citations

The Cbl protooncoprotein stimulates CSF-1 receptor multiubiquitination and endocytosis, and attenuates macrophage proliferation

1999 • 291 citations

The NF-kappaB signalling pathway in human diseases: from incontinentia pigmenti to ectodermal dysplasias and immune-deficiency syndromes

2002 • 288 citations

Activation of the E3 ligase function of the BRCA1/BARD1 complex by polyubiquitin chains

2002 • 274 citations

Part II: α-synuclein and its molecular pathophysiological role in neurodegenerative disease

2003 • 263 citations

Synaptic defects in ataxia mice result from a mutation in Usp14, encoding a ubiquitin-specific protease

2002 • 261 citations

The BRCA1/BARD1 heterodimer, a tumor suppressor complex with ubiquitin E3 ligase activity

2002 • 260 citations

Potential for proteasome inhibition in the treatment of cancer

2003 • 244 citations

Inhibition of NF-kappa B cellular function via specific targeting of the Ikappa B-ubiquitin ligase

1997 • 238 citations

Induction of apoptosis in human papillomaviruspositive cancer cells by peptide aptamers targeting the viral E6 oncoprotein

2000 • 238 citations

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

2001 • 227 citations

Mutant ubiquitin found in neurodegenerative disorders is a ubiquitin fusion degradation substrate that blocks proteasomal degradation

2002 • 220 citations

Autoubiquitination of the BRCA1·BARD1 RING Ubiquitin Ligase

2002 • 218 citations

Endoproteolytic Cleavage and Proteasomal Degradation of Presenilin 2 in Transfected Cells

1997 • 213 citations

The von Hippel–Lindau tumor suppressor protein

2001 • 208 citations

The von Hippel–Lindau tumor suppressor, hypoxia-inducible factor-1 (HIF-1) degradation, and cancer pathogenesis

2002 • 202 citations

Expression of Alzheimer’s Disease-associated Presenilin-1 Is Controlled by Proteolytic Degradation and Complex Formation

1998 • 199 citations

Proteasome Inhibitor PS519 Reduces Infarction and Attenuates Leukocyte Infiltration in a Rat Model of Focal Cerebral Ischemia

2000 • 183 citations

Activity and Regulation of the Centrosome-associated Proteasome

2000 • 172 citations

Human papillomavirus-induced carcinogenesis and the ubiquitin–proteasome system

2002 • 163 citations

Rates of ubiquitin conjugation increase when muscles atrophy, largely through activation of the N-end rule pathway

1998 • 155 citations

The Pathway of Us11-Dependent Degradation of Mhc Class I Heavy Chains Involves a Ubiquitin-Conjugated Intermediate

1999 • 150 citations

Amplification pattern of 12q13-q15 genes (MDM2, CDK4, GLI) in urinary bladder cancer

2002 • 146 citations

Polyglutamines Placed into Context

2003 • 138 citations

The Drosophila bendless gene encodes a neural protein related to ubiquitin-conjugating enzymes

1993 • 134 citations

BRCA1-Independent Ubiquitination of FANCD2

2003 • 132 citations

Understanding p27^kip1Deregulation in Cancer: Downregulation or Mislocalizaiton?

2002 • 121 citations

Proteasome inhibition: A novel mechanism to combat asthma☆☆☆

1999 • 108 citations

Altered Activity, Social Behavior, and Spatial Memory in Mice Lacking the NTAN1p Amidase and the Asparagine Branch of the N-End Rule Pathway

2000 • 105 citations

Interleukin-6 Induces G1Arrest through Induction of p27Kip1, a Cyclin-Dependent Kinase Inhibitor, and Neuron-like Morphology in LNCaP Prostate Tumor Cells

1999 • 102 citations

Elevated expression of Unph, a proto-oncogene at 3p21.3, in human lung tumors

1995 • 92 citations

From fruit fly to bedside

2003 • 90 citations

Treatment of Established Relapsing Experimental Autoimmune Encephalomyelitis with the Proteasome Inhibitor PS-5191

2000 • 85 citations

Triplet repeat expansion in neuromuscular disease

2000 • 83 citations

Parkin is associated with actin filaments in neuronal and nonneural cells

2000 • 79 citations

Angelman syndrome: a review of clinical and genetic aspects

1999 • 78 citations

Ariadne-1: A Vital Drosophila Gene Is Required in Development and Defines a New Conserved Family of RING-Finger Proteins

2000 • 78 citations

The role of ubiquitylation in signaling by growth factors: implications to cancer

2002 • 75 citations

Regulation of the Fanconi anemia pathway by monoubiquitination

2002 • 75 citations

Pharmacological treatment of the ion transport defect in cystic fibrosis

2001 • 71 citations

SEL‐10 interacts with presenilin 1, facilitates its ubiquitination, and alters A‐beta peptide production

2002 • 68 citations

Regulation of the epithelial sodium channel (ENaC) by accessory proteins

2000 • 57 citations

First Proteasome Inhibitor Approved for Multiple Myeloma

2003 • 55 citations

Death and NF-κB in T Cell Activation: Life at the Edge

2003 • 51 citations

The Role of the Ubiquitin–Proteasome Pathway in the Formation of Mallory Bodies

2002 • 51 citations

Genetic analysis of the role of theDrosophila fat facets gene in the Ubiquitin pathway

1999 • 38 citations

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

2000 • 31 citations

Synergistic antitumor effects of a selective proteasome inhibitor and TNF in mice.

2000 • 31 citations

The ubiquitin/proteasome system in Epstein–Barr virus latency and associated malignancies

2002 • 26 citations

Characterization of the ubiquitin-specific protease activity of the mouse/human Unp/Unph oncoprotein

2000 • 25 citations

p27Kip1 is the key mediator of phenylacetate induced cell cycle arrest in human prostate cancer cells.

2000 • 18 citations

NF-κB Defects in Humans: The NEMO/Incontinentia Pigmenti Connection

2000 • 18 citations