Prion-like propagation of protein aggregation and related therapeutic strategies.

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Published in Neurotherapeutics on July 01, 2013

Authors

Sarah K Kaufman1, Marc I Diamond

Author Affiliations

1: Department of Neurology, Washington University in St Louis, 660 South Euclid Avenue, St Louis, MO 63110, USA.

Associated clinical trials:

Safety, Tolerability, and Activity Study of ISIS SOD1Rx to Treat Familial Amyotrophic Lateral Sclerosis (ALS) Caused by SOD1 Gene Mutations (SOD-1) | NCT01041222

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Neuropathological stageing of Alzheimer-related changes. Acta Neuropathol (1991) 49.03

Mutation in the alpha-synuclein gene identified in families with Parkinson's disease. Science (1997) 28.30

Prions. Proc Natl Acad Sci U S A (1998) 27.80

Staging of brain pathology related to sporadic Parkinson's disease. Neurobiol Aging (2002) 22.40

alpha-Synuclein locus triplication causes Parkinson's disease. Science (2003) 20.20

Dissociable intrinsic connectivity networks for salience processing and executive control. J Neurosci (2007) 19.58

Segregation of a missense mutation in the amyloid precursor protein gene with familial Alzheimer's disease. Nature (1991) 16.77

Intrinsic functional architecture in the anaesthetized monkey brain. Nature (2007) 14.47

TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis. Science (2008) 14.09

Cloning of a gene bearing missense mutations in early-onset familial Alzheimer's disease. Nature (1995) 13.80

Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis. Science (2009) 13.45

Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6. Science (2009) 12.99

Immunization with amyloid-beta attenuates Alzheimer-disease-like pathology in the PDAPP mouse. Nature (1999) 12.95

Ala30Pro mutation in the gene encoding alpha-synuclein in Parkinson's disease. Nat Genet (1998) 12.92

Mice devoid of PrP are resistant to scrapie. Cell (1993) 11.46

Conversion of alpha-helices into beta-sheets features in the formation of the scrapie prion proteins. Proc Natl Acad Sci U S A (1993) 10.36

Neurodegenerative diseases target large-scale human brain networks. Neuron (2009) 9.69

The new mutation, E46K, of alpha-synuclein causes Parkinson and Lewy body dementia. Ann Neurol (2004) 9.62

Neurodegenerative tauopathies. Annu Rev Neurosci (2001) 9.53

Transplantation of embryonic dopamine neurons for severe Parkinson's disease. N Engl J Med (2001) 9.51

Resting-state functional connectivity reflects structural connectivity in the default mode network. Cereb Cortex (2008) 9.38

Eight prion strains have PrP(Sc) molecules with different conformations. Nat Med (1998) 9.01

Peripherally administered antibodies against amyloid beta-peptide enter the central nervous system and reduce pathology in a mouse model of Alzheimer disease. Nat Med (2000) 8.89

Candidate gene for the chromosome 1 familial Alzheimer's disease locus. Science (1995) 8.31

The promises and pitfalls of RNA-interference-based therapeutics. Nature (2009) 7.53

Transmission and spreading of tauopathy in transgenic mouse brain. Nat Cell Biol (2009) 7.50

A beta peptide vaccination prevents memory loss in an animal model of Alzheimer's disease. Nature (2001) 7.35

Synthetic mammalian prions. Science (2004) 7.35

Lewy bodies in grafted neurons in subjects with Parkinson's disease suggest host-to-graft disease propagation. Nat Med (2008) 6.90

Inclusion formation and neuronal cell death through neuron-to-neuron transmission of alpha-synuclein. Proc Natl Acad Sci U S A (2009) 6.87

Lewy body-like pathology in long-term embryonic nigral transplants in Parkinson's disease. Nat Med (2008) 6.86

Staging of Alzheimer's disease-related neurofibrillary changes. Neurobiol Aging (1995) 6.86

A beta peptide immunization reduces behavioural impairment and plaques in a model of Alzheimer's disease. Nature (2001) 6.78

Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity. Science (1996) 6.71

Pathological α-synuclein transmission initiates Parkinson-like neurodegeneration in nontransgenic mice. Science (2012) 6.66

Trinucleotide repeat length instability and age of onset in Huntington's disease. Nat Genet (1993) 6.53

Subacute meningoencephalitis in a subset of patients with AD after Abeta42 immunization. Neurology (2003) 6.51

Exogenous induction of cerebral beta-amyloidogenesis is governed by agent and host. Science (2006) 6.47

Propagation of tau pathology in a model of early Alzheimer's disease. Neuron (2012) 6.23

A double-blind controlled trial of bilateral fetal nigral transplantation in Parkinson's disease. Ann Neurol (2003) 6.21

A phase 2 multiple ascending dose trial of bapineuzumab in mild to moderate Alzheimer disease. Neurology (2009) 5.94

Propagation of tau misfolding from the outside to the inside of a cell. J Biol Chem (2009) 5.76

Secondary structure analysis of the scrapie-associated protein PrP 27-30 in water by infrared spectroscopy. Biochemistry (1991) 5.53

Clinical effects of Abeta immunization (AN1792) in patients with AD in an interrupted trial. Neurology (2005) 5.51

Anatomical correlates of the distribution of the pathological changes in the neocortex in Alzheimer disease. Proc Natl Acad Sci U S A (1985) 4.97

Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques. Cell (1989) 4.73

Encephalopathy in mice produced by inoculation with scrapie brain material. Lancet (1961) 4.67

Trans-synaptic spread of tau pathology in vivo. PLoS One (2012) 4.53

Sustained therapeutic reversal of Huntington's disease by transient repression of huntingtin synthesis. Neuron (2012) 4.48

Intracerebral inoculation of pathological α-synuclein initiates a rapidly progressive neurodegenerative α-synucleinopathy in mice. J Exp Med (2012) 3.98

Peripherally applied Abeta-containing inoculates induce cerebral beta-amyloidosis. Science (2010) 3.98

Intravesicular localization and exocytosis of alpha-synuclein and its aggregates. J Neurosci (2005) 3.93

Cell-produced alpha-synuclein is secreted in a calcium-dependent manner by exosomes and impacts neuronal survival. J Neurosci (2010) 3.81

Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis. Nat Neurosci (2005) 3.69

Neuropathological evidence of graft survival and striatal reinnervation after the transplantation of fetal mesencephalic tissue in a patient with Parkinson's disease. N Engl J Med (1995) 3.64

Exosome-associated tau is secreted in tauopathy models and is selectively phosphorylated in cerebrospinal fluid in early Alzheimer disease. J Biol Chem (2011) 3.57

Cytoplasmic penetration and persistent infection of mammalian cells by polyglutamine aggregates. Nat Cell Biol (2009) 3.55

α-Synuclein propagates from mouse brain to grafted dopaminergic neurons and seeds aggregation in cultured human cells. J Clin Invest (2011) 3.53

Tafamidis for transthyretin familial amyloid polyneuropathy: a randomized, controlled trial. Neurology (2012) 3.48

A change in the conformation of prions accompanies the emergence of a new prion strain. Neuron (2002) 3.36

Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells. Proc Natl Acad Sci U S A (2011) 3.23

Effects of alpha-synuclein immunization in a mouse model of Parkinson's disease. Neuron (2005) 3.20

Recombinant prion protein induces a new transmissible prion disease in wild-type animals. Acta Neuropathol (2010) 3.19

Immunotherapy targeting pathological tau conformers in a tangle mouse model reduces brain pathology with associated functional improvements. J Neurosci (2007) 3.10

Predicting regional neurodegeneration from the healthy brain functional connectome. Neuron (2012) 3.07

Detection of oligomeric forms of alpha-synuclein protein in human plasma as a potential biomarker for Parkinson's disease. FASEB J (2006) 2.96

Synthetic tau fibrils mediate transmission of neurofibrillary tangles in a transgenic mouse model of Alzheimer's-like tauopathy. J Neurosci (2013) 2.90

Seeding of normal Tau by pathological Tau conformers drives pathogenesis of Alzheimer-like tangles. J Biol Chem (2011) 2.86

Assembly-dependent endocytosis and clearance of extracellular alpha-synuclein. Int J Biochem Cell Biol (2008) 2.78

Alpha-synuclein implicated in Parkinson's disease is present in extracellular biological fluids, including human plasma. FASEB J (2003) 2.64

Transfer of scrapie prion infectivity by cell contact in culture. Curr Biol (2002) 2.54

Trans-cellular propagation of Tau aggregation by fibrillar species. J Biol Chem (2012) 2.42

Evidence for seeding of beta -amyloid by intracerebral infusion of Alzheimer brain extracts in beta -amyloid precursor protein-transgenic mice. J Neurosci (2000) 2.41

Molecular basis of barriers for interspecies transmissibility of mammalian prions. Mol Cell (2004) 2.39

Passive amyloid immunotherapy clears amyloid and transiently activates microglia in a transgenic mouse model of amyloid deposition. J Neurosci (2004) 2.38

Purified and synthetic Alzheimer's amyloid beta (Aβ) prions. Proc Natl Acad Sci U S A (2012) 2.36

Tafamidis, a potent and selective transthyretin kinetic stabilizer that inhibits the amyloid cascade. Proc Natl Acad Sci U S A (2012) 2.27

Safety, tolerability, and antibody response of active Aβ immunotherapy with CAD106 in patients with Alzheimer's disease: randomised, double-blind, placebo-controlled, first-in-human study. Lancet Neurol (2012) 2.22

Passive immunization reduces behavioral and neuropathological deficits in an alpha-synuclein transgenic model of Lewy body disease. PLoS One (2011) 2.19

Tissue damage in the amyloidoses: Transthyretin monomers and nonnative oligomers are the major cytotoxic species in tissue culture. Proc Natl Acad Sci U S A (2004) 2.14

Exosomal cell-to-cell transmission of alpha synuclein oligomers. Mol Neurodegener (2012) 2.11

Sting of Alzheimer's failures offset by upcoming prevention trials. Nat Rev Drug Discov (2012) 2.02

In vivo microdialysis reveals age-dependent decrease of brain interstitial fluid tau levels in P301S human tau transgenic mice. J Neurosci (2011) 2.00

Seeded aggregation and toxicity of {alpha}-synuclein and tau: cellular models of neurodegenerative diseases. J Biol Chem (2010) 1.92

Uptake and neuritic transport of scrapie prion protein coincident with infection of neuronal cells. J Neurosci (2005) 1.85

Passive immunization targeting pathological phospho-tau protein in a mouse model reduces functional decline and clears tau aggregates from the brain. J Neurochem (2011) 1.82

Small misfolded Tau species are internalized via bulk endocytosis and anterogradely and retrogradely transported in neurons. J Biol Chem (2012) 1.79

Focality of upper and lower motor neuron degeneration at the clinical onset of ALS. Neurology (2007) 1.67

Intense superoxide dismutase-1 immunoreactivity in intracytoplasmic hyaline inclusions of familial amyotrophic lateral sclerosis with posterior column involvement. J Neuropathol Exp Neurol (1996) 1.67

Antibody-aided clearance of extracellular α-synuclein prevents cell-to-cell aggregate transmission. J Neurosci (2012) 1.63

Efficacy and safety of immunization with phosphorylated tau against neurofibrillary tangles in mice. Exp Neurol (2010) 1.61

Thermal stability and conformational transitions of scrapie amyloid (prion) protein correlate with infectivity. Protein Sci (1993) 1.59

Physiological release of endogenous tau is stimulated by neuronal activity. EMBO Rep (2013) 1.57

A seeding reaction recapitulates intracellular formation of Sarkosyl-insoluble transactivation response element (TAR) DNA-binding protein-43 inclusions. J Biol Chem (2011) 1.48

Neuron-to-neuron transmission of α-synuclein fibrils through axonal transport. Ann Neurol (2012) 1.46

Tau-targeted immunization impedes progression of neurofibrillary histopathology in aged P301L tau transgenic mice. PLoS One (2011) 1.45

Targeting phospho-Ser422 by active Tau Immunotherapy in the THYTau22 mouse model: a suitable therapeutic approach. Curr Alzheimer Res (2012) 1.41

Implications of ALS focality: rostral-caudal distribution of lower motor neuron loss postmortem. Neurology (2007) 1.38

Some speculations about prions, amyloid, and Alzheimer's disease. N Engl J Med (1984) 1.35

Spreading of neurodegenerative pathology via neuron-to-neuron transmission of β-amyloid. J Neurosci (2012) 1.34

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