Protofibrils, pores, fibrils, and neurodegeneration: separating the responsible protein aggregates from the innocent bystanders.
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[1] S. Prusiner,et al. Scrapie prions aggregate to form amyloid-like birefringent rods , 1983, Cell.
[2] H. Gelderblom,et al. Scrapie infectivity, fibrils and low molecular weight protein , 1983, Nature.
[3] L. Kurland,et al. Fine Structural Study of Neurofibrillary Changes in a Family with Amyotrophic Lateral Sclerosis , 1984, Journal of neuropathology and experimental neurology.
[4] Ruedi Aebersold,et al. A cellular gene encodes scrapie PrP 27-30 protein , 1985, Cell.
[5] G. Multhaup,et al. The major polypeptide of scrapie‐associated fibrils (SAF) has the same size, charge distribution and N‐terminal protein sequence as predicted for the normal brain protein (PrP). , 1986, The EMBO journal.
[6] S. Prusiner,et al. Properties of scrapie prion protein liposomes. , 1988, The Journal of biological chemistry.
[7] G. Multhaup,et al. Molecular pathology of scrapie-associated fibril protein (PrP) in mouse brain affected by the ME7 strain of scrapie. , 1988, European Journal of Biochemistry.
[8] L. Hood,et al. Purification and properties of the cellular and scrapie hamster prion proteins. , 1988, European journal of biochemistry.
[9] P. McBride,et al. Precise targeting of the pathology of the sialoglycoprotein, PrP, and vacuolar degeneration in mouse scrapie , 1989, Neuroscience Letters.
[10] P. Liberski,et al. Conservation of infectivity in purified fibrillary extracts of scrapie-infected hamster brain after sequential enzymatic digestion or polyacrylamide gel electrophoresis. , 1990, Proceedings of the National Academy of Sciences of the United States of America.
[11] D. Kirschner,et al. Neurotrophic and neurotoxic effects of amyloid beta protein: reversal by tachykinin neuropeptides. , 1990, Science.
[12] D. Zopf,et al. Molecular mass, biochemical composition, and physicochemical behavior of the infectious form of the scrapie precursor protein monomer. , 1990, Proceedings of the National Academy of Sciences of the United States of America.
[13] G. J. Raymond,et al. The scrapie-associated form of PrP is made from a cell surface precursor that is both protease- and phospholipase-sensitive. , 1991, The Journal of biological chemistry.
[14] G. J. Raymond,et al. N-terminal truncation of the scrapie-associated form of PrP by lysosomal protease(s): implications regarding the site of conversion of PrP to the protease-resistant state , 1991, Journal of virology.
[15] S. Prusiner,et al. Scrapie prion rod formation in vitro requires both detergent extraction and limited proteolysis , 1991, Journal of virology.
[16] S. Prusiner,et al. Evidence for synthesis of scrapie prion proteins in the endocytic pathway. , 1992, The Journal of biological chemistry.
[17] J. Haines,et al. Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis , 1993, Nature.
[18] A. Aguzzi,et al. Mice devoid of PrP are resistant to scrapie , 1993, Cell.
[19] P. Lansbury,et al. The carboxy terminus of the beta amyloid protein is critical for the seeding of amyloid formation: implications for the pathogenesis of Alzheimer's disease. , 1993, Biochemistry.
[20] R. Bowler,et al. Three scrapie prion isolates exhibit different accumulation patterns of the prion protein scrapie isoform. , 1993, Proceedings of the National Academy of Sciences of the United States of America.
[21] P. Lansbury,et al. Seeding “one-dimensional crystallization” of amyloid: A pathogenic mechanism in Alzheimer's disease and scrapie? , 1993, Cell.
[22] E. Rojas,et al. Giant multilevel cation channels formed by Alzheimer disease amyloid beta-protein [A beta P-(1-40)] in bilayer membranes. , 1993, Proceedings of the National Academy of Sciences of the United States of America.
[23] H. Ushijima,et al. Cytoprotective effect of NMDA receptor antagonists on prion protein (PrionSc)-induced toxicity in rat cortical cell cultures. , 1993, European journal of pharmacology.
[24] P. Lansbury,et al. Cell-free formation of protease-resistant prion protein , 1994, Nature.
[25] J. Hope. The Nature of the Scrapie Agent: The Evolution of the Virino , 1994, Annals of the New York Academy of Sciences.
[26] B. Yankner,et al. Beta-amyloid neurotoxicity requires fibril formation and is inhibited by congo red. , 1994, Proceedings of the National Academy of Sciences of the United States of America.
[27] S. Prusiner,et al. Degeneration of skeletal muscle, peripheral nerves, and the central nervous system in transgenic mice overexpressing wild-type prion proteins , 1994, Cell.
[28] T. Umahara,et al. Cu Zn superoxide dismutase-like immunoreactivity in Lewy body-like inclusions of sporadic amyotrophic lateral sclerosis , 1994, Neuroscience Letters.
[29] S. Prusiner,et al. Serial transmission in rodents of neurodegeneration from transgenic mice expressing mutant prion protein. , 1994, Proceedings of the National Academy of Sciences of the United States of America.
[30] D. Borchelt,et al. Superoxide dismutase is an abundant component in cell bodies, dendrites, and axons of motor neurons and in a subset of other neurons. , 1995, Proceedings of the National Academy of Sciences of the United States of America.
[31] David F. Clayton,et al. Characterization of a novel protein regulated during the critical period for song learning in the zebra finch , 1995, Neuron.
[32] P. Andersen,et al. Amyotrophic lateral sclerosis associated with homozygosity for an Asp90Ala mutation in CuZn-superoxide dismutase , 1995, Nature Genetics.
[33] W. Hung,et al. Intense Superoxide Dismutase‐1 Immunoreactivity in Intracytoplasmic Hyaline Inclusions of Familial Amyotrophic Lateral Sclerosis with Posterior Column Involvement , 1996, Journal of neuropathology and experimental neurology.
[34] S. Younkin,et al. Correlative Memory Deficits, Aβ Elevation, and Amyloid Plaques in Transgenic Mice , 1996, Science.
[35] S. Prusiner,et al. Disruption of prion rods generates 10-nm spherical particles having high alpha-helical content and lacking scrapie infectivity , 1996, Journal of virology.
[36] R. Kastelein,et al. Mouse T Cell Membrane Proteins Rt61 and Rt62 Are Arginine/Protein Mono(ADPribosyl)transferases and Share Secondary Structure Motifs with ADP-ribosylating Bacterial Toxins (*) , 1996, The Journal of Biological Chemistry.
[37] E. Rojas,et al. Zn2+ interaction with Alzheimer amyloid beta protein calcium channels. , 1996, Proceedings of the National Academy of Sciences of the United States of America.
[38] D. Selkoe,et al. Sequence of Deposition of Heterogeneous Amyloid β-Peptides and APO E in Down Syndrome: Implications for Initial Events in Amyloid Plaque Formation , 1996, Neurobiology of Disease.
[39] W. Robberecht,et al. D90A heterozygosity in the SOD1 gene is associated with familial and apparently sporadic amyotrophic lateral sclerosis , 1996, Neurology.
[40] J. Hardy,et al. Increased amyloid-β42(43) in brains of mice expressing mutant presenilin 1 , 1996, Nature.
[41] N. Shibata,et al. Sporadic amyotrophic lateral sclerosis with dementia and Cu/Zn superoxide dismutase-positive Lewy body-like inclusions. , 1996, Clinical neuropathology.
[42] A. Aguzzi,et al. Normal host prion protein necessary for scrapie-induced neurotoxicity , 1996, Nature.
[43] M. Eigen,et al. Prionics or the kinetic basis of prion diseases. , 1996, Biophysical chemistry.
[44] F. Cohen,et al. High-level expression and characterization of a purified 142-residue polypeptide of the prion protein. , 1996, Biochemistry.
[45] R. Petersen,et al. Effect of the D178N Mutation and the Codon 129 Polymorphism on the Metabolism of the Prion Protein (*) , 1996, The Journal of Biological Chemistry.
[46] Guy A. Rouleau,et al. SOD1 mutation is assosiated with accumulation of neurofilaments in amyotrophic lateral scelaries , 1996 .
[47] F. Cohen,et al. Physical studies of conformational plasticity in a recombinant prion protein. , 1997, Biochemistry.
[48] Robert L. Nussbaum,et al. Mutation in the α-Synuclein Gene Identified in Families with Parkinson's Disease , 1997 .
[49] B. Kagan,et al. Channel Formation by a Neurotoxic Prion Protein Fragment* , 1997, The Journal of Biological Chemistry.
[50] J. Rossier,et al. Transmission of the BSE Agent to Mice in the Absence of Detectable Abnormal Prion Protein , 1997, Science.
[51] Peter T. Lansbury,et al. Observation of metastable Aβ amyloid protofibrils by atomic force microscopy , 1997 .
[52] P. Lansbury,et al. Atomic force microscopic imaging of seeded fibril formation and fibril branching by the Alzheimer's disease amyloid-beta protein. , 1997, Chemistry & biology.
[53] Shu G. Chen,et al. Prion Protein Aggregation Reverted by Low Temperature in Transfected Cells Carrying a Prion Protein Gene Mutation* , 1997, The Journal of Biological Chemistry.
[54] D. Figlewicz,et al. Aggregation of Mutant Cu/Zn Superoxide Dismutase Proteins in a Culture Model of ALS , 1997, Journal of neuropathology and experimental neurology.
[55] G. J. Raymond,et al. In Situ Formation of Protease-resistant Prion Protein in Transmissible Spongiform Encephalopathy-infected Brain Slices* , 1997, The Journal of Biological Chemistry.
[56] P. Lansbury,et al. Models of amyloid seeding in Alzheimer's disease and scrapie: mechanistic truths and physiological consequences of the time-dependent solubility of amyloid proteins. , 1997, Annual review of biochemistry.
[57] D. Walsh,et al. Amyloid beta-protein fibrillogenesis. Detection of a protofibrillar intermediate. , 1997, The Journal of biological chemistry.
[58] Robert H. Brown,et al. Amyotrophic lateral sclerosis. Insights from genetics. , 1997, Archives of neurology.
[59] P. Andersen,et al. Phenotypic heterogeneity in motor neuron disease patients with CuZn-superoxide dismutase mutations in Scandinavia. , 1997, Brain : a journal of neurology.
[60] M. L. Schmidt,et al. α-Synuclein in Lewy bodies , 1997, Nature.
[61] Hans Lehrach,et al. Huntingtin-Encoded Polyglutamine Expansions Form Amyloid-like Protein Aggregates In Vitro and In Vivo , 1997, Cell.
[62] L. Bruijn,et al. Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1. , 1998, Science.
[63] R. Kopito,et al. Aggresomes: A Cellular Response to Misfolded Proteins , 1998, The Journal of cell biology.
[64] P. Lansbury,et al. A detergent-insoluble membrance compartment contains Aβ in vivo , 1998, Nature Medicine.
[65] A. Paetau,et al. A variant of Alzheimer's disease with spastic paraparesis and unusual plaques due to deletion of exon 9 of presenilin 1 , 1998, Nature Medicine.
[66] M. Goedert,et al. Binding of α-Synuclein to Brain Vesicles Is Abolished by Familial Parkinson’s Disease Mutation* , 1998, The Journal of Biological Chemistry.
[67] Peter T. Lansbury,et al. Accelerated in vitro fibril formation by a mutant α-synuclein linked to early-onset Parkinson disease , 1998, Nature Medicine.
[68] C. Hayward,et al. Homozygosity for Asn86Ser mutation in the CuZn-superoxide dismutase gene produces a severe clinical phenotype in a juvenile onset case of familial amyotrophic lateral sclerosis. , 1998, Journal of medical genetics.
[69] T. Morgan,et al. Diffusible, nonfibrillar ligands derived from Abeta1-42 are potent central nervous system neurotoxins. , 1998, Proceedings of the National Academy of Sciences of the United States of America.
[70] B. Caughey,et al. The importance of the disulfide bond in prion protein conversion , 1998, Neuroreport.
[71] A. Giese,et al. Role of Microglia in Neuronal Cell Death in Prion Disease , 1998, Brain pathology.
[72] D. Cleveland. From Charcot to SOD1 Mechanisms of Selective Motor Neuron Death in ALS , 1999, Neuron.
[73] J. Collinge,et al. Protease-resistant prion protein produced in vitro lacks detectable infectivity. , 1999, The Journal of general virology.
[74] C. Dobson. Protein misfolding, evolution and disease. , 1999, Trends in biochemical sciences.
[75] C. Soto,et al. Cell-lysate conversion of prion protein into its protease-resistant isoform suggests the participation of a cellular chaperone. , 1999, Biochemical and biophysical research communications.
[76] Dominic M. Walsh,et al. Protofibrillar Intermediates of Amyloid β-Protein Induce Acute Electrophysiological Changes and Progressive Neurotoxicity in Cortical Neurons , 1999, The Journal of Neuroscience.
[77] J Collinge,et al. Reversible conversion of monomeric human prion protein between native and fibrilogenic conformations. , 1999, Science.
[78] R. Nicoll,et al. Plaque-independent disruption of neural circuits in Alzheimer's disease mouse models. , 1999, Proceedings of the National Academy of Sciences of the United States of America.
[79] Peter T. Lansbury,et al. Assembly of Aβ Amyloid Protofibrils: An in Vitro Model for a Possible Early Event in Alzheimer's Disease† , 1999 .
[80] Veerle Baekelandt,et al. Early Phenotypic Changes in Transgenic Mice That Overexpress Different Mutants of Amyloid Precursor Protein in Brain* , 1999, The Journal of Biological Chemistry.
[81] V. Daggett,et al. PrPc glycoform heterogeneity as a function of brain region: implications for selective targeting of neurons by prion strains. , 1999, Journal of neuropathology and experimental neurology.
[82] P. Lansbury. Evolution of amyloid: what normal protein folding may tell us about fibrillogenesis and disease. , 1999, Proceedings of the National Academy of Sciences of the United States of America.
[83] D. Selkoe,et al. Amyloid beta-protein fibrillogenesis. Structure and biological activity of protofibrillar intermediates. , 1999, The Journal of biological chemistry.
[84] R. Glockshuber,et al. Influence of amino acid substitutions related to inherited human prion diseases on the thermodynamic stability of the cellular prion protein. , 1999, Biochemistry.
[85] B. Caughey,et al. Specific binding of normal prion protein to the scrapie form via a localized domain initiates its conversion to the protease‐resistant state , 1999, The EMBO journal.
[86] M. Citron,et al. Both Familial Parkinson’s Disease Mutations Accelerate α-Synuclein Aggregation* , 1999, The Journal of Biological Chemistry.
[87] G A Johnson,et al. Detection of neuritic plaques in Alzheimer's disease by magnetic resonance microscopy. , 1999, Proceedings of the National Academy of Sciences of the United States of America.
[88] S. Prusiner,et al. Transmissible and genetic prion diseases share a common pathway of neurodegeneration , 1999, Nature.
[89] M. Citron,et al. alpha-synuclein fibrillogenesis is nucleation-dependent. Implications for the pathogenesis of Parkinson's disease. , 1999, The Journal of biological chemistry.
[90] H. Wiśniewski,et al. Plasma amyloid β‐peptide 1–42 and incipient Alzheimer's disease , 1999 .
[91] P. Lansbury,et al. Inhibition of fibrillization and accumulation of prefibrillar oligomers in mixtures of human and mouse alpha-synuclein. , 2000, Biochemistry.
[92] A. Goldberg,et al. PAN, the proteasome-activating nucleotidase from archaebacteria, is a protein-unfolding molecular chaperone , 2000, Nature Cell Biology.
[93] T. Wisniewski,et al. Reversion of prion protein conformational changes by synthetic b-sheet breaker peptides , 2000, The Lancet.
[94] D. Westaway,et al. Copper(II)-induced Conformational Changes and Protease Resistance in Recombinant and Cellular PrP , 2000, The Journal of Biological Chemistry.
[95] P. Lansbury,et al. Acceleration of oligomerization, not fibrillization, is a shared property of both alpha-synuclein mutations linked to early-onset Parkinson's disease: implications for pathogenesis and therapy. , 2000, Proceedings of the National Academy of Sciences of the United States of America.
[96] D. Riesner,et al. Neurotoxicity but not infectivity of prion proteins can be induced reversibly in vitro. , 2000, Archives of virology. Supplementum.
[97] A. Paetau,et al. Variant Alzheimer's disease with spastic paraparesis and cotton wool plaques is caused by PS‐1 mutations that lead to exceptionally high amyloid‐β concentrations , 2000, Annals of neurology.
[98] F E Cohen,et al. A synthetic peptide initiates Gerstmann-Sträussler-Scheinker (GSS) disease in transgenic mice. , 2000, Journal of molecular biology.
[99] A. Johnston,et al. Synapse loss associated with abnormal PrP precedes neuronal degeneration in the scrapie‐infected murine hippocampus , 2000, Neuropathology and applied neurobiology.
[100] Kenji Nakashima,et al. New consensus research on neuropathological aspects of familial amyotrophic lateral sclerosis with superoxide dismutase 1 (SOD1) gene mutations: Inclusions containing SOD1 in neurons and astrocytes , 2000, Amyotrophic lateral sclerosis and other motor neuron disorders : official publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases.
[101] M. Gurney,et al. Formation of high molecular weight complexes of mutant Cu, Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis. , 2000, Proceedings of the National Academy of Sciences of the United States of America.
[102] Kang Hu,et al. High-Level Neuronal Expression of Aβ1–42 in Wild-Type Human Amyloid Protein Precursor Transgenic Mice: Synaptotoxicity without Plaque Formation , 2000, The Journal of Neuroscience.
[103] D. F. Andrews,et al. A one-hit model of cell death in inherited neuronal degenerations , 2000, Nature.
[104] D. Cleveland,et al. Oxidation versus aggregation — how do SOD1 mutants cause ALS? , 2000, Nature Medicine.
[105] D. Selkoe,et al. A de novo designed helix-turn-helix peptide forms nontoxic amyloid fibrils , 2000, Nature Structural Biology.
[106] P N Leigh,et al. Recent advances in amyotrophic lateral sclerosis , 2000, Current opinion in neurology.
[107] J. Julien,et al. The neuronal Golgi apparatus is fragmented in transgenic mice expressing a mutant human SOD1, but not in mice expressing the human NF-H gene , 2000, Journal of the Neurological Sciences.
[108] L. Mucke,et al. Dopaminergic loss and inclusion body formation in alpha-synuclein mice: implications for neurodegenerative disorders. , 2000, Science.
[109] W. Bender,et al. A Drosophila model of Parkinson's disease , 2000, Nature.
[110] B. Ghetti,et al. Accumulation of protease-resistant prion protein (PrP) and apoptosis of cerebellar granule cells in transgenic mice expressing a PrP insertional mutation. , 2000, Proceedings of the National Academy of Sciences of the United States of America.
[111] J. Bureš,et al. Specific spatial learning deficits become severe with age in β-amyloid precursor protein transgenic mice that harbor diffuse β-amyloid deposits but do not form plaques , 2001, Proceedings of the National Academy of Sciences of the United States of America.
[112] R. S. Stewart,et al. Most Pathogenic Mutations Do Not Alter the Membrane Topology of the Prion Protein* , 2001, The Journal of Biological Chemistry.
[113] S. Younkin,et al. The 'Arctic' APP mutation (E693G) causes Alzheimer's disease by enhanced Aβ protofibril formation , 2001, Nature Neuroscience.
[114] M. Baldwin. Mass spectrometric analysis of prion proteins. , 2001, Advances in protein chemistry.
[115] J. Julien,et al. Amyotrophic Lateral Sclerosis Unfolding the Toxicity of the Misfolded , 2001, Cell.
[116] R. Kopito,et al. Impairment of the ubiquitin-proteasome system by protein aggregation. , 2001, Science.
[117] John Q. Trojanowski,et al. Chaperone Suppression of α-Synuclein Toxicity in a Drosophila Model for Parkinson's Disease , 2001, Science.
[118] W. Surewicz,et al. On the Mechanism of α-Helix to β-Sheet Transition in the Recombinant Prion Protein† , 2001 .
[119] Makoto Hashimoto,et al. β-Synuclein Inhibits α-Synuclein Aggregation A Possible Role as an Anti-Parkinsonian Factor , 2001, Neuron.
[120] M. Kirkitadze,et al. Identification and characterization of key kinetic intermediates in amyloid beta-protein fibrillogenesis. , 2001, Journal of molecular biology.
[121] C. Haass,et al. Protofibrils, the unifying toxic molecule of neurodegenerative disorders? , 2001, Nature Neuroscience.
[122] D. Dickson,et al. Analysis of tauopathies with transgenic mice. , 2001, Trends in molecular medicine.
[123] Rushana Azimova,et al. The channel hypothesis of Huntington’s disease , 2001, Brain Research Bulletin.
[124] B. Austen,et al. Non-fibrillar oligomeric species of the amyloid ABri peptide, implicated in familial British dementia, are more potent at inducing apoptotic cell death than protofibrils or mature fibrils. , 2001, Journal of Molecular Biology.
[125] M. F. Perutz,et al. Cause of neural death in neurodegenerative diseases attributable to expansion of glutamine repeats , 2001, Nature.
[126] V. Uversky,et al. Effect of familial Parkinson's disease point mutations A30P and A53T on the structural properties, aggregation, and fibrillation of human alpha-synuclein. , 2001, Biochemistry.
[127] G. Rouleau,et al. Compound heterozygous D90A and D96N SOD1 mutations in a recessive amyotrophic lateral sclerosis family , 2001, Annals of neurology.
[128] G. Forloni,et al. Studies on peptide fragments of prion proteins. , 2001, Advances in protein chemistry.
[129] Nobutaka Hattori,et al. Ubiquitination of a New Form of α-Synuclein by Parkin from Human Brain: Implications for Parkinson's Disease , 2001, Science.
[130] K Wüthrich,et al. Three-dimensional structures of prion proteins. , 2001, Advances in protein chemistry.
[131] H. Scheraga,et al. A role for intermolecular disulfide bonds in prion diseases? , 2001, Proceedings of the National Academy of Sciences of the United States of America.
[132] Witold K. Surewicz,et al. Crystal structure of the human prion protein reveals a mechanism for oligomerization , 2002, Nature Structural Biology.
[133] B. Caughey,et al. Reversibility of Scrapie-associated Prion Protein Aggregation* , 2001, The Journal of Biological Chemistry.
[134] D. Harris,et al. Copper Converts the Cellular Prion Protein into a Protease-resistant Species That Is Distinct from the Scrapie Isoform* , 2001, The Journal of Biological Chemistry.
[135] Y. Cordeiro,et al. DNA Converts Cellular Prion Protein into the β-Sheet Conformation and Inhibits Prion Peptide Aggregation* , 2001, The Journal of Biological Chemistry.
[136] A. Chong,et al. Onset of accumulation of PrPres in murine ME7 scrapie in relation to pathological and PrP immunohistochemical changes. , 2001, Journal of comparative pathology.
[137] D. Harris. Biosynthesis and cellular processing of the prion protein. , 2001, Advances in protein chemistry.
[138] S. Minotti,et al. Mutant Cu/Zn-Superoxide Dismutase Proteins Have Altered Solubility and Interact with Heat Shock/Stress Proteins in Models of Amyotrophic Lateral Sclerosis* , 2001, The Journal of Biological Chemistry.
[139] S. Prusiner,et al. Copper-catalyzed oxidation of the recombinant SHa(29–231) prion protein , 2001, Proceedings of the National Academy of Sciences of the United States of America.
[140] B. Permanne,et al. Sensitive detection of pathological prion protein by cyclic amplification of protein misfolding , 2001, Nature.
[141] J. Hardy,et al. A Pathogenic Presenilin-1 Deletion Causes Abberrant Aβ42 Production in the Absence of Congophilic Amyloid Plaques* , 2001, The Journal of Biological Chemistry.
[142] D. Small,et al. Alzheimer's disease and Aβ toxicity: from top to bottom , 2001, Nature Reviews Neuroscience.
[143] T. Morgan,et al. Vaccination with soluble Aβ oligomers generates toxicity‐neutralizing antibodies , 2001, Journal of neurochemistry.
[144] R. Glockshuber. Folding dynamics and energetics of recombinant prion proteins. , 2001, Advances in protein chemistry.
[145] G. J. Raymond,et al. Interactions and conversions of prion protein isoforms. , 2001, Advances in protein chemistry.
[146] R. Lal,et al. Amyloid β protein forms ion channels: implications for Alzheimer's disease pathophysiology , 2001, FASEB journal : official publication of the Federation of American Societies for Experimental Biology.
[147] B. Caughey,et al. Interactions between prion protein isoforms: the kiss of death? , 2001, Trends in biochemical sciences.
[148] G. J. Raymond,et al. Conformational change, aggregation and fibril formation induced by detergent treatments of cellular prion protein , 2001, Journal of neurochemistry.
[149] P. Lansbury,et al. Vesicle permeabilization by protofibrillar alpha-synuclein: implications for the pathogenesis and treatment of Parkinson's disease. , 2001, Biochemistry.
[150] Peter T. Lansbury,et al. Kinetic Stabilization of the α-Synuclein Protofibril by a Dopamine-α-Synuclein Adduct , 2001, Science.
[151] P. Lansbury,et al. Neurodegenerative disease: Amyloid pores from pathogenic mutations , 2002, Nature.
[152] B. Chesebro,et al. Conversion of raft associated prion protein to the protease‐resistant state requires insertion of PrP‐res (PrPSc) into contiguous membranes , 2002, The EMBO journal.
[153] M. Beal,et al. Mutated Human SOD1 Causes Dysfunction of Oxidative Phosphorylation in Mitochondria of Transgenic Mice* , 2002, The Journal of Biological Chemistry.
[154] Smita Patel,et al. Golgi Fragmentation Occurs in the Cells with Prefibrillar α-Synuclein Aggregates and Precedes the Formation of Fibrillar Inclusion* , 2002, The Journal of Biological Chemistry.
[155] A. Chakrabartty,et al. Oxidation-induced Misfolding and Aggregation of Superoxide Dismutase and Its Implications for Amyotrophic Lateral Sclerosis* , 2002, The Journal of Biological Chemistry.
[156] S. Lindquist,et al. Neurotoxicity and Neurodegeneration When PrP Accumulates in the Cytosol , 2002, Science.
[157] I. Vorberg,et al. Molecular Basis of Scrapie Strain Glycoform Variation* , 2002, The Journal of Biological Chemistry.
[158] N. Arispe,et al. Plasma membrane cholesterol controls the cytotoxicity of Alzheimer's disease AβP (1–40) and (1–42) peptides , 2002, FASEB journal : official publication of the Federation of American Societies for Experimental Biology.
[159] H. Cai,et al. Genetically engineered mouse models of neurodegenerative diseases , 2002, Nature Neuroscience.
[160] Seung-Jae Lee,et al. Characterization of cytoplasmic alpha-synuclein aggregates. Fibril formation is tightly linked to the inclusion-forming process in cells. , 2002, The Journal of biological chemistry.
[161] W. K. Cullen,et al. Naturally secreted oligomers of amyloid β protein potently inhibit hippocampal long-term potentiation in vivo , 2002, Nature.
[162] Robert Burke,et al. Resistance of α-synuclein null mice to the parkinsonian neurotoxin MPTP , 2002, Proceedings of the National Academy of Sciences of the United States of America.
[163] Christopher A Ross,et al. Polyglutamine Pathogenesis Emergence of Unifying Mechanisms for Huntington's Disease and Related Disorders , 2002, Neuron.
[164] C. Dobson,et al. Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseases , 2002, Nature.
[165] Robert H. Brown,et al. Familial Amyotrophic Lateral Sclerosis-associated Mutations Decrease the Thermal Stability of Distinctly Metallated Species of Human Copper/Zinc Superoxide Dismutase* , 2002, The Journal of Biological Chemistry.
[166] Bruce A. Yankner,et al. Dopamine-dependent neurotoxicity of α-synuclein: A mechanism for selective neurodegeneration in Parkinson disease , 2002, Nature Medicine.
[167] David A Agard,et al. Structural studies of the scrapie prion protein by electron crystallography , 2002, Proceedings of the National Academy of Sciences of the United States of America.
[168] T. Pinheiro,et al. Binding of prion protein to lipid membranes and implications for prion conversion. , 2002, Journal of molecular biology.
[169] Peter T Lansbury,et al. Genetics of Parkinson's disease and biochemical studies of implicated gene products. , 2002, Current opinion in cell biology.
[170] Patrik Brundin,et al. Impaired dopamine storage resulting from alpha-synuclein mutations may contribute to the pathogenesis of Parkinson's disease. , 2002, Human molecular genetics.
[171] Christopher A. Ross,et al. Huntingtin Spheroids and Protofibrils as Precursors in Polyglutamine Fibrilization* , 2002, The Journal of Biological Chemistry.
[172] J. Hoh,et al. Growth of β-amyloid(1-40) protofibrils by monomer elongation and lateral association. Characterization of distinct products by light scattering and atomic force microscopy , 2002 .
[173] Neena Singh,et al. Prion Peptide 106–126 Modulates the Aggregation of Cellular Prion Protein and Induces the Synthesis of Potentially Neurotoxic Transmembrane PrP* , 2002, The Journal of Biological Chemistry.
[174] Christina A. Wilson,et al. Presenilins are not required for Aβ42 production in the early secretory pathway , 2002, Nature Neuroscience.
[175] F. Hartl,et al. Molecular Chaperones in the Cytosol: from Nascent Chain to Folded Protein , 2002, Science.
[176] Rustam Azimov,et al. The channel hypothesis of Alzheimer’s disease: current status , 2002, Peptides.
[177] J. Hardy,et al. The Amyloid Hypothesis of Alzheimer ’ s Disease : Progress and Problems on the Road to Therapeutics , 2009 .
[178] P. Lansbury,et al. Alpha-synuclein, especially the Parkinson's disease-associated mutants, forms pore-like annular and tubular protofibrils. , 2002, Journal of molecular biology.
[179] D. Borchelt,et al. Fibrillar Inclusions and Motor Neuron Degeneration in Transgenic Mice Expressing Superoxide Dismutase 1 with a Disrupted Copper-Binding Site , 2002, Neurobiology of Disease.
[180] F. Cohen,et al. Pathway Complexity of Prion Protein Assembly into Amyloid* , 2002, The Journal of Biological Chemistry.
[181] P. Lansbury,et al. Annular alpha-synuclein protofibrils are produced when spherical protofibrils are incubated in solution or bound to brain-derived membranes. , 2002, Biochemistry.
[182] Sebastian Doniach,et al. Biophysical Properties of the Synucleins and Their Propensities to Fibrillate , 2002, The Journal of Biological Chemistry.
[183] P. Lansbury,et al. Vesicle permeabilization by protofibrillar alpha-synuclein is sensitive to Parkinson's disease-linked mutations and occurs by a pore-like mechanism. , 2002, Biochemistry.
[184] H. E. Stanley,et al. Neurotoxic effects of thioflavin S-positive amyloid deposits in transgenic mice and Alzheimer's disease , 2002, Proceedings of the National Academy of Sciences of the United States of America.
[185] J. Trojanowski,et al. Neuronal α-Synucleinopathy with Severe Movement Disorder in Mice Expressing A53T Human α-Synuclein , 2002, Neuron.
[186] L. K. Baker,et al. Oligomeric and Fibrillar Species of Amyloid-β Peptides Differentially Affect Neuronal Viability* , 2002, The Journal of Biological Chemistry.
[187] A. LeBlanc,et al. Selective cytotoxicity of intracellular amyloid β peptide1–42 through p53 and Bax in cultured primary human neurons , 2002, The Journal of cell biology.
[188] J. Schaack,et al. Overexpression of human α-synuclein causes dopamine neuron death in primary human mesencephalic culture , 2002, Brain Research.
[189] H. Scheraga,et al. Intramolecular Versus Intermolecular Disulfide Bonds in Prion Proteins* , 2002, The Journal of Biological Chemistry.
[190] Robert H. Brown,et al. Decreased Metallation and Activity in Subsets of Mutant Superoxide Dismutases Associated with Familial Amyotrophic Lateral Sclerosis* 210 , 2002, The Journal of Biological Chemistry.
[191] P. Aebischer,et al. α-Synucleinopathy and selective dopaminergic neuron loss in a rat lentiviral-based model of Parkinson's disease , 2002, Proceedings of the National Academy of Sciences of the United States of America.
[192] P. Lansbury,et al. The UCH-L1 Gene Encodes Two Opposing Enzymatic Activities that Affect α-Synuclein Degradation and Parkinson's Disease Susceptibility , 2002, Cell.
[193] K. Fischbeck,et al. Toxic Proteins in Neurodegenerative Disease , 2002, Science.
[194] Nancy A. Jenkins,et al. Human α-synuclein-harboring familial Parkinson's disease-linked Ala-53 → Thr mutation causes neurodegenerative disease with α-synuclein aggregation in transgenic mice , 2002, Proceedings of the National Academy of Sciences of the United States of America.
[195] S. Lindquist,et al. Conversion of PrP to a Self-Perpetuating PrPSc-like Conformation in the Cytosol , 2002, Science.
[196] H. Wiśniewski,et al. Abnormal fibrils from scrapie-infected brain , 2004, Acta Neuropathologica.