The Pathophysiology of Fatal Familial Insomnia
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Abstract
References (42)
Thalamocortical Oscillations in the Sleeping and Aroused Brain
1993 • 3,483 citations
Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein
1992 • 1,632 citations
The Central Autonomic Network: Functional Organization, Dysfunction, and Perspective
1993 • 1,301 citations
The cellular prion protein binds copper in vivo
1997 • 1,263 citations
The role of monoamines and acetylcholine-containing neurons in the regulation of the sleep-waking cycle
1972 • 1,091 citations
Prion protein (PrP) with amino-proximal deletions restoring susceptibility of PrP knockout mice to scrapie.
1996 • 872 citations
Prion protein is necessary for normal synaptic function
1994 • 802 citations
Distinct PrP properties suggest the molecular basis of strain variation in transmissible mink encephalopathy
1994 • 663 citations
HYPOTHALAMIC REGULATION OF SLEEP IN RATS. AN EXPERIMENTAL STUDY
1946 • 655 citations
Altered circadian activity rhythms and sleep in mice devoid of prion protein
1996 • 655 citations
Brain Mechanisms and Consciousness
1955 • 631 citations
129/Ola mice carrying a null mutation in PrP that abolishes mRNA production are developmentally normal
1994 • 563 citations
Loss of cerebellar Purkinje cells in aged mice homozygous for a disrupted PrP gene
1996 • 483 citations
The sleep-waking cycle
1972 • 476 citations
The human 37-kDa laminin receptor precursor interacts with the prion protein in eukaryotic cells
1997 • 435 citations
Sleep and Sleep Regulation in Normal and Prion Protein-Deficient Mice
1997 • 296 citations
Identification of two biologically distinct strains of transmissible mink encephalopathy in hamsters
1992 • 276 citations
Forebrain inhibitory mechanisms: Cortical synchronization induced by basal forebrain stimulation
1962 • 244 citations
Hypersomnia following paramedian thalamic stroke: A report of 12 patients
1996 • 218 citations
Complementary hydropathy identifies a cellular prion protein receptor
1997 • 184 citations
Hippocampal slices from prion protein null mice: disrupted Ca2+-activated K+ currents
1996 • 184 citations
Regional distribution of protease‐resistant prion protein in fatal familial insomnia
1995 • 164 citations
Rescue of neurophysiological phenotype seen in PrP null mice by transgene encoding human prion protein
1995 • 146 citations
Sleep-wakefulness, EEG and behavioral studies of chronic cats without the thalamus: the 'athalamic' cat.
1972 • 132 citations
A unique membrane protein is expressed on early developing limbic system axons and cortical targets
1988 • 132 citations
Isolation, biochemical characterization and ultrastructural analysis of the limbic system-associated membrane protein (LAMP), a protein expressed by neurons comprising functional neural circuits.
1990 • 114 citations
Isolation, biochemical characterization and ultrastructural analysis of the limbic system-associated membrane protein (LAMP), a protein expressed by neurons comprising functional neural circuits
1990 • 107 citations
Pseudo-hypersomnia and pre-sleep behaviour with bilateral paramedian thalamic lesions
1993 • 101 citations
Sleep-wakefulness, EEG and behavioral studies of chronic cats without neocortex and striatum: the 'diencephalic' cat.
1972 • 96 citations
Cerebral metabolism in fatal familial insomnia: Relation to duration, neuropathology, and distribution of protease-resistent prion protein
1997 • 94 citations
Thalamic haemorrhage
1996 • 93 citations
Sleep-wake cycle abnormalities in fatal familial insomnia. Evidence of the role of the thalamus in sleep regulation
1995 • 91 citations
Conservation of the cellular gene encoding the scrapie prion protein
1986 • 85 citations
Fatal familial insomnia
1996 • 73 citations
Prion protein is necessary for latent learning and long-term memory retention.
1997 • 58 citations
Sleep and sleep disorders : from molecule to behavior
1997 • 50 citations
The thalamus and insomnia.
1992 • 50 citations
Fatal familial insomnia: Sleep, neuroendocrine and vegetative alterations
1995 • 38 citations
Changes in sleep-waking cycle induced by lesions of medialis dorsalis thalamic nuclei in the cat
1988 • 31 citations
Multiple roles for PrP in the prion diseases
1996 • 22 citations
A Study on the Central Representation of Sleep Behaviour
1964 • 15 citations
Molecular Regulation of Arousal States
1997 • 4 citations