Cellular prion protein neuroprotective function: implications in prion diseases

Novel Proteinaceous Infectious Particles Cause Scrapie

1982 • 4,972 citations

Mice devoid of PrP are resistant to scrapie

1993 • 2,058 citations

BH1 and BH2 domains of Bcl-2 are required for inhibition of apoptosis and heterodimerization with Bax

1994 • 1,303 citations

Conversion of Bcl-2 to a Bax-like Death Effector by Caspases

1997 • 1,116 citations

Breaking the mitochondrial barrier

2001 • 947 citations

Scrapie and cellular PrP isoforms are encoded by the same chromosomal gene

1986 • 829 citations

Signal Transduction Through Prion Protein

2000 • 786 citations

BAX Is Required for Neuronal Death after Trophic Factor Deprivation and during Development

1996 • 745 citations

Depleting Neuronal PrP in Prion Infection Prevents Disease and Reverses Spongiosis

2003 • 728 citations

A Transmembrane Form of the Prion Protein in Neurodegenerative Disease

1998 • 724 citations

Fatal Familial Insomnia, a Prion Disease with a Mutation at Codon 178 of the Prion Protein Gene

1992 • 588 citations

129/Ola mice carrying a null mutation in PrP that abolishes mRNA production are developmentally normal

1994 • 563 citations

Extracellular-signal-regulated kinase signalling in neurons

1999 • 554 citations

Expression of Amino-Terminally Truncated PrP in the Mouse Leading to Ataxia and Specific Cerebellar Lesions

1998 • 529 citations

Ataxia in prion protein (PrP)-deficient mice is associated with upregulation of the novel PrP-like protein doppel

1999 • 523 citations

Normal prion protein has an activity like that of superoxide dismutase

1999 • 520 citations

Modulation of cell death by Bcl-x _L through caspase interaction

1998 • 505 citations

Loss of cerebellar Purkinje cells in aged mice homozygous for a disrupted PrP gene

1996 • 483 citations

Neurotoxicity and Neurodegeneration When PrP Accumulates in the Cytosol

2002 • 477 citations

Ablation of the prion protein (PrP) gene in mice prevents scrapie and facilitates production of anti-PrP antibodies.

1993 • 461 citations

Prion Protein-Deficient Cells Show Altered Response to Oxidative Stress Due to Decreased SOD-1 Activity

1997 • 447 citations

bcl-2 protein expression is widespread in the developing nervous system and retained in the adult PNS

1994 • 432 citations

Stress-inducible protein 1 is a cell surface ligand for cellular prion that triggers neuroprotection

2002 • 427 citations

Scrapie prion proteins are synthesized in neurons.

1986 • 422 citations

The 37-kDa/67-kDa laminin receptor acts as the cell-surface receptor for the cellular prion protein

2001 • 422 citations

Prion Protein Protects Human Neurons against Bax-mediated Apoptosis

2001 • 374 citations

Binding of neural cell adhesion molecules (N-CAMs) to the cellular prion protein

2001 • 371 citations

Cellular prion protein transduces neuroprotective signals

2002 • 366 citations

Cross-Linking Cellular Prion Protein Triggers Neuronal Apoptosis in Vivo

2004 • 360 citations

Cellular prion protein binds laminin and mediates neuritogenesis

2000 • 322 citations

Signal transduction mediated by Bid, a pro-death Bcl-2 family proteins, connects the death receptor and mitochondria apoptosis pathways

2000 • 305 citations

Transmissible and genetic prion diseases share a common pathway of neurodegeneration

1999 • 302 citations

Wild-type PrP and a mutant associated with prion disease are subject to retrograde transport and proteasome degradation

2001 • 291 citations

No propagation of prions in mice devoid of PrP

1994 • 282 citations

Vascular variant of prion protein cerebral amyloidosis with tau-positive neurofibrillary tangles: the phenotype of the stop codon 145 mutation in PRNP.

1996 • 275 citations

Onset of ataxia and Purkinje cell loss in PrP null mice inversely correlated with Dpl level in brain

2001 • 252 citations

Proteasomes and ubiquitin are involved in the turnover of the wild-type prion protein

2001 • 247 citations

Imbalance of antioxidant defense in mice lacking cellular prion protein

2001 • 245 citations

PrPC Directly Interacts with Proteins Involved in Signaling Pathways

2001 • 224 citations

Lack of prion protein expression results in a neuronal phenotype sensitive to stress

2001 • 223 citations

Mutant PrP Is Delayed in Its Exit from the Endoplasmic Reticulum, but Neither Wild-type nor Mutant PrP Undergoes Retrotranslocation Prior to Proteasomal Degradation

2003 • 222 citations

A mouse prion protein transgene rescues mice deficient for the prion protein gene from purkinje cell degeneration and demyelination.

1999 • 208 citations

Cellular prion protein function in copper homeostasis and redox signalling at the synapse

2003 • 206 citations

Prion Protein-Deficient Neurons Reveal Lower Glutathione Reductase Activity and Increased Susceptibility to Hydrogen Peroxide Toxicity

1999 • 191 citations

An Amber Mutation of Prion Protein in Gerstmann-Sträussler Syndrome with Mutant PrP Plaques

1993 • 191 citations

NADPH oxidase and extracellular regulated kinases 1/2 are targets of prion protein signaling in neuronal and nonneuronal cells

2003 • 190 citations

Increased Sensitivity to Seizures in Mice Lacking Cellular Prion Protein

1999 • 184 citations

Prion protein as trans-interacting partner for neurons is involved in neurite outgrowth and neuronal survival

2003 • 181 citations

Neuroprotective functions of prion protein

2003 • 177 citations

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

1995 • 172 citations

Cytosolic Prion Protein Is Not Toxic and Protects against Bax-mediated Cell Death in Human Primary Neurons

2003 • 172 citations

Increased levels of oxidative stress markers detected in the brains of mice devoid of prion protein

2001 • 171 citations

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

1999 • 165 citations

PrP knock-out and PrP transgenic mice in prion research

2003 • 157 citations

Conformational control of Bax localization and apoptotic activity by Pro168

2004 • 142 citations

Soluble Dimeric Prion Protein Binds PrPSc In Vivo and Antagonizes Prion Disease

2003 • 135 citations

Mutant Prion Proteins Are Partially Retained in the Endoplasmic Reticulum

2001 • 127 citations

The cellular prion protein (PrPC) prevents apoptotic neuronal cell death and mitochondrial dysfunction induced by serum deprivation

2004 • 127 citations

Prion Protein Prevents Human Breast Carcinoma Cell Line from Tumor Necrosis Factor α-Induced Cell Death

2004 • 116 citations

No Superoxide Dismutase Activity of Cellular Prion Protein in vivo

2003 • 116 citations

Familial spongiform encephalopathy associated with a novel prion protein gene mutation

1997 • 108 citations

Differential Contribution of Superoxide Dismutase Activity by Prion Protein in Vivo

2000 • 107 citations

Analysis of interaction sites in homo- and heteromeric complexes containing Bcl-2 family members and the cellular prion protein

1996 • 107 citations

Regulation of Protein Topology by trans-Acting Factors at the Endoplasmic Reticulum

1998 • 104 citations

Intercellular Transfer of the Cellular Prion Protein

2002 • 101 citations

Cellular Phenotyping of Secretory and Nuclear Prion Proteins Associated with Inherited Prion Diseases

2002 • 100 citations

Cellular Prion Protein Sensitizes Neurons to Apoptotic Stimuli through Mdm2-regulated and p53-dependent Caspase 3-like Activation

2003 • 98 citations

Cellular prion protein status in sheep: tissue-specific biochemical signatures

2001 • 95 citations

Impairment of superoxide dismutase activation by N-terminally truncated prion protein (PrP) in PrP-deficient neuronal cell line

2003 • 82 citations

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

2002 • 80 citations

Overexpression of PrP^c triggers caspase 3 activation: potentiation by proteasome inhibitors and blockade by anti‐PrP antibodies

2002 • 73 citations

Primary Cultured Neurons Devoid of Cellular Prion Display Lower Responsiveness to Staurosporine through the Control of p53 at Both Transcriptional and Post-transcriptional Levels

2003 • 66 citations

Induction of HO-1 and NOS in Doppel-Expressing Mice Devoid of PrP: Implications for Doppel Function

2001 • 64 citations

Prions prevent brain damage after experimental brain injury: a preliminary report

2003 • 48 citations

Deletion of N-terminal Residues 23–88 from Prion Protein (PrP) Abrogates the Potential to Rescue PrP-deficient Mice from PrP-like Protein/Doppel-induced Neurodegeneration

2003 • 48 citations

Transgene-driven expression of the Doppel protein in Purkinje cells causes Purkinje cell degeneration and motor impairment

2004 • 46 citations

Analysis of the Interactions Between HIV-1 and the Cellular Prion Protein in a Human Cell Line

2004 • 46 citations

Disruption of Doppel prevents neurodegeneration in mice with extensive Prnp deletions

2004 • 44 citations

Post-translational Import of the Prion Protein into the Endoplasmic Reticulum Interferes with Cell Viability

2003 • 44 citations

Neuron-Specific mRNA Complexity Responses during Hippocampal Apoptosis after Traumatic Brain Injury

2004 • 44 citations

Glycosyl Phosphatidylinositol Anchor

1998 • 38 citations

Heat shock modulates prion protein expression in human NT-2 cells

2000 • 34 citations

Prion protein interactions with nucleic acid: possible models for prion disease and prion function.

2003 • 29 citations

Transfection of prion protein gene suppresses coxsackievirus B3 replication in prion protein gene-deficient cells

2003 • 25 citations

A human prion protein peptide (PrP59–91) protects against copper neurotoxicity

2003 • 24 citations

Unraveling the Controversy of Human Prion Protein Diseases

1998 • 4 citations