PubRank

O Bugiani

Author PubWeight™ 105.52‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Neurotoxicity of a prion protein fragment. Nature 1993 3.38
2 Down patients: extracellular preamyloid deposits precede neuritic degeneration and senile plaques. Neurosci Lett 1989 2.69
3 Sporadic Creutzfeldt-Jakob disease: co-occurrence of different types of PrP(Sc) in the same brain. Neurology 1999 2.33
4 Preamyloid deposits in the cerebral cortex of patients with Alzheimer's disease and nondemented individuals. Neurosci Lett 1988 2.26
5 Neuropathological diagnostic criteria for Creutzfeldt-Jakob disease (CJD) and other human spongiform encephalopathies (prion diseases). Brain Pathol 1995 2.10
6 Vascular variant of prion protein cerebral amyloidosis with tau-positive neurofibrillary tangles: the phenotype of the stop codon 145 mutation in PRNP. Proc Natl Acad Sci U S A 1996 1.94
7 Tissue handling in suspected Creutzfeldt-Jakob disease (CJD) and other human spongiform encephalopathies (prion diseases) Brain Pathol 1995 1.86
8 Phenotypic variability of Gerstmann-Sträussler-Scheinker disease is associated with prion protein heterogeneity. J Neuropathol Exp Neurol 1998 1.79
9 Amyloid protein of Gerstmann-Sträussler-Scheinker disease (Indiana kindred) is an 11 kd fragment of prion protein with an N-terminal glycine at codon 58. EMBO J 1991 1.74
10 Substitutions at codon 22 of Alzheimer's abeta peptide induce diverse conformational changes and apoptotic effects in human cerebral endothelial cells. J Biol Chem 2000 1.71
11 Effectiveness of anthracycline against experimental prion disease in Syrian hamsters. Science 1997 1.70
12 Amyloid fibrils in Gerstmann-Sträussler-Scheinker disease (Indiana and Swedish kindreds) express only PrP peptides encoded by the mutant allele. Cell 1994 1.69
13 Prion protein amyloidosis. Brain Pathol 1996 1.64
14 Infantile neuroaxonal dystrophy: clinical spectrum and diagnostic criteria. Neurology 1999 1.62
15 Worldwide distribution of PSEN1 Met146Leu mutation: a large variability for a founder mutation. Neurology 2010 1.59
16 Alzheimer patients and Down patients: cerebral preamyloid deposits differ ultrastructurally and histochemically from the amyloid of senile plaques. Neurosci Lett 1989 1.35
17 A 7-kDa prion protein (PrP) fragment, an integral component of the PrP region required for infectivity, is the major amyloid protein in Gerstmann-Sträussler-Scheinker disease A117V. J Biol Chem 2000 1.34
18 Gerstmann-Sträussler-Scheinker disease and the Indiana kindred. Brain Pathol 1995 1.25
19 The fine structure of subcortical neurofibrillary tangles in progressive supranuclear palsy. Acta Neuropathol 1979 1.24
20 Molecular characteristics of a protease-resistant, amyloidogenic and neurotoxic peptide homologous to residues 106-126 of the prion protein. Biochem Biophys Res Commun 1993 1.23
21 Progressive supranuclear palsy 1979: an overview. Ital J Neurol Sci 1980 1.20
22 Tau protein directly interacts with the amyloid beta-protein precursor: implications for Alzheimer's disease. Nat Med 1995 1.13
23 Creutzfeldt-Jakob disease: Carnoy's fixative improves the immunohistochemistry of the proteinase K-resistant prion protein. Brain Pathol 2000 1.13
24 Prion protein preamyloid and amyloid deposits in Gerstmann-Sträussler-Scheinker disease, Indiana kindred. Proc Natl Acad Sci U S A 1992 1.09
25 Tetracycline affects abnormal properties of synthetic PrP peptides and PrP(Sc) in vitro. J Mol Biol 2000 1.09
26 CT and MR imaging of neuroaxonal leukodystrophy presenting as early-onset frontal dementia. AJNR Am J Neuroradiol 2006 1.07
27 Conformational polymorphism of the amyloidogenic and neurotoxic peptide homologous to residues 106-126 of the prion protein. J Biol Chem 1994 1.07
28 Prion proteins with different conformations accumulate in Gerstmann-Sträussler-Scheinker disease caused by A117V and F198S mutations. Am J Pathol 2001 1.07
29 Proteinase-K-resistant prion protein isoforms in Gerstmann-Sträussler-Scheinker disease (Indiana kindred). J Neuropathol Exp Neurol 1996 1.04
30 Coexistence of Alzheimer's amyloid precursor protein and amyloid protein in cerebral vessel walls. Lab Invest 1990 1.01
31 Creutzfeldt-Jakob disease with a novel four extra-repeat insertional mutation in the PrP gene. Neurology 2000 1.01
32 Familial Gerstmann-Sträussler-Scheinker disease with neurofibrillary tangles. Mol Neurobiol 1994 1.01
33 A soluble form of prion protein in human cerebrospinal fluid: implications for prion-related encephalopathies. Biochem Biophys Res Commun 1992 1.00
34 Molecular determinants of the physicochemical properties of a critical prion protein region comprising residues 106-126. Biochem J 1999 0.98
35 A68 is a component of paired helical filaments of Gerstmann-Sträussler-Scheinker disease, Indiana kindred. Brain Res 1993 0.97
36 Gerstmann-Sträussler-Scheinker disease (PRNP P102L): amyloid deposits are best recognized by antibodies directed to epitopes in PrP region 90-165. J Neuropathol Exp Neurol 1995 0.97
37 Tauopathy in human and experimental variant Creutzfeldt-Jakob disease. Neurobiol Aging 2007 0.96
38 Prion protein fragment 106-126 induces apoptotic cell death and impairment of L-type voltage-sensitive calcium channel activity in the GH3 cell line. J Neurosci Res 1998 0.96
39 An antibody raised against a conserved sequence of the prion protein recognizes pathological isoforms in human and animal prion diseases, including Creutzfeldt-Jakob disease and bovine spongiform encephalopathy. Am J Pathol 1998 0.93
40 Neurofibrillary tangles of the Indiana kindred of Gerstmann-Sträussler-Scheinker disease share antigenic determinants with those of Alzheimer disease. Brain Res 1990 0.93
41 Letter: T and B lymphocytes in normal cerebrospinal fluid. N Engl J Med 1976 0.93
42 Huntington's disease: survival of large striatal neurons in the rigid variant. Ann Neurol 1984 0.91
43 The stimulation of inducible nitric-oxide synthase by the prion protein fragment 106--126 in human microglia is tumor necrosis factor-alpha-dependent and involves p38 mitogen-activated protein kinase. J Biol Chem 2001 0.90
44 Loss of striatal neurons in Parkinson's disease: a cytometric study. Eur Neurol 1980 0.89
45 Intracellular calcium rise through L-type calcium channels, as molecular mechanism for prion protein fragment 106-126-induced astroglial proliferation. Biochem Biophys Res Commun 1996 0.89
46 Cerebral preamyloid deposits and congophilic angiopathy in aged dogs. Neurosci Lett 1990 0.89
47 Clinical and genetic features of families with frontotemporal dementia and parkinsonism linked to chromosome 17 with a P301S tau mutation. J Neural Transm (Vienna) 2007 0.88
48 A novel phenotype of sporadic Creutzfeldt-Jakob disease. J Neurol Neurosurg Psychiatry 2007 0.87
49 Ectopic white matter neurons, a developmental abnormality that may be caused by the PSEN1 S169L mutation in a case of familial AD with myoclonus and seizures. J Neuropathol Exp Neurol 2001 0.86
50 Apoptosis-mediated neurotoxicity induced by beta-amyloid and PrP fragments. Mol Chem Neuropathol 1997 0.86
51 Polymorphism at codon 129 of PRNP affects the phenotypic expression of Creutzfeldt-Jakob disease linked to E200K mutation. Ann Neurol 2000 0.86
52 Activation effects of a prion protein fragment [PrP-(106-126)] on human leucocytes. Biochem J 1996 0.86
53 Thickening of the basement membrane of cortical capillaries in Alzheimer's disease. Acta Neuropathol 1980 0.86
54 Skin fibroblasts in Huntington's disease. N Engl J Med 1978 0.86
55 Progressive supranuclear palsy with hypertrophy of the olives. An immunocytochemical study of the cytoskeleton of argyrophilic neurons. Acta Neuropathol 1988 0.86
56 Studies on peptide fragments of prion proteins. Adv Protein Chem 2001 0.85
57 Amyloid in Alzheimer's disease and prion-related encephalopathies: studies with synthetic peptides. Prog Neurobiol 1996 0.85
58 Progressing encephalomyelopathy with muscular atrophy, induced by aluminum powder. Neurobiol Aging 1982 0.85
59 Creutzfeldt-Jakob disease with a novel extra-repeat insertional mutation in the PRNP gene. Neurology 2003 0.85
60 Apoptotic cell death and impairment of L-type voltage-sensitive calcium channel activity in rat cerebellar granule cells treated with the prion protein fragment 106-126. Neurobiol Dis 2000 0.85
61 Relationship between non-fibrillary amyloid precursors and cell processes in the cortical neuropil of Alzheimer patients. Neurosci Lett 1991 0.85
62 A neurotoxic and gliotrophic fragment of the prion protein increases plasma membrane microviscosity. Neurobiol Dis 1997 0.84
63 Measurement of intracellular calcium levels by the fluorescent Ca(2+) indicator Calcium-Green. Brain Res Brain Res Protoc 2000 0.84
64 [Plurisystemic degeneration of the neuraxis: new anatomoclinical contribution]. Sem Hop 1967 0.84
65 beta PP and Tau interaction. A possible link between amyloid and neurofibrillary tangles in Alzheimer's disease. Am J Pathol 1996 0.84
66 A SOD1 gene mutation in a patient with slowly progressing familial ALS. Neurology 1999 0.84
67 Creutzfeldt-Jakob disease in the city and district of Genoa: estimated mortality rate in the six year period 1974-1979. Ital J Neurol Sci 1981 0.84
68 Chronic high frequency stimulation of the posteromedial hypothalamus in facial pain syndromes and behaviour disorders. Acta Neurochir Suppl 2007 0.83
69 Ubiquitinated neurites are associated with preamyloid and cerebral amyloid beta deposits in patients with hereditary cerebral hemorrhage with amyloidosis Dutch type. Acta Neuropathol 1993 0.83
70 A family with Alzheimer disease and strokes associated with A713T mutation of the APP gene. Neurology 2004 0.83
71 [Consensus report: tissue handling in suspected Creutzfeldt-Jakob disease and other spongiform encephalopathies (prion diseases) in the human. European Union Biomed-1 Concerted Action]. Pathologe 1996 0.83
72 Determination of solution conformations of PrP106-126, a neurotoxic fragment of prion protein, by 1H NMR and restrained molecular dynamics. Eur J Biochem 1999 0.82
73 Clusterin (SGP-2) induction in rat astroglial cells exposed to prion protein fragment 106-126. Eur J Neurosci 1996 0.82
74 Down syndrome as a key to the time sequence of brain changes in Alzheimer disease. Prog Clin Biol Res 1992 0.82
75 Activation of microglial cells by PrP and beta-amyloid fragments raises intracellular calcium through L-type voltage sensitive calcium channels. Brain Res 1999 0.82
76 Neuronal ceroid-lipofuscinosis: a clinical and morphological study of 19 patients. Am J Med Genet 1995 0.82
77 Early myoclonus and quasiperiodic EEG changes in non-familial Alzheimer's disease. Ital J Neurol Sci 1980 0.82
78 Preserved semantic access in global amnesia and hippocampal damage. Clin Neuropsychol 2001 0.81
79 Intracellular mechanisms mediating the neuronal death and astrogliosis induced by the prion protein fragment 106-126. Int J Dev Neurosci 2000 0.81
80 Alzheimer patients: preamyloid deposits are immunoreactive with antibodies to extracellular domains of the amyloid precursor protein. Neurosci Lett 1991 0.81
81 Nontraumatic dissecting aneurysm of the basilar artery. Eur Neurol 1983 0.80
82 Fatal familial insomnia: genetic, neuropathologic, and biochemical study of a patient from a new Italian kindred. Neurology 1998 0.80
83 Surface markers on lymphocytes from human cerebrospinal fluid. Predominance of T lymphocytes bearing receptors for the Fc segment of IgG. Eur Neurol 1978 0.80
84 Tendency to periodic recurrence of EEG changes in Lafora's disease. Case report. Eur Neurol 1979 0.80
85 Multiple stroke due to multiple vascular malformation of the brain. Eur Neurol 1984 0.79
86 Diffuse thalamic degeneration in fatal familial insomnia. A morphometric study. Brain Res 1997 0.79
87 Aluminum-induced decreases in choline acetyltransferase, tyrosine hydroxylase, and glutamate decarboxylase in selected regions of rabbit brain. Neurochem Pathol 1987 0.79
88 Increasing complexity of the karyotype in 50 human gliomas. Progressive evolution and de novo occurrence of cytogenetic alterations. Cancer Genet Cytogenet 1994 0.79
89 Cerebral extracellular preamyloid deposits in Alzheimer's disease, Down syndrome and nondemented elderly individuals. Prog Clin Biol Res 1989 0.78
90 [A new disease caused by accumulation of glycolipids. (Ceramide tetrahexosides)]. Minerva Pediatr 1967 0.78
91 Aphasia in thalamic haemorrhage. Lancet 1969 0.78
92 FTDP-17: phenotypical heterogeneity within P301S. Ann Neurol 2000 0.78
93 Nerve cell loss in the progressive encephalopathy induced by aluminum powder. A morphologic and semiquantitative study of the Purkinje cells. Neuropathol Appl Neurobiol 1985 0.78
94 Juvenile G M2 -gangliosidosis: a morphological and chemical study of a cerebral biopsy. Acta Neurol Belg 1972 0.78
95 Neuronal ceroid lipofuscinosis: detection of atypical forms. Neurol Sci 2000 0.77
96 Diffuse senile plaques: amorphous or fibrous? Am J Pathol 1995 0.77
97 [Early juvenile GM2 gangliosidosis (morphological and chemical study of a cerebral biopsy)]. Acta Neurol (Napoli) 1971 0.76
98 Comment on: Neurotoxicity of prion peptide 106-126 not confirmed, by Beat Kunz, Erika Sandmeier, Philipp Christen. FEBS Letters 485 (1999) 65-68. FEBS Lett 2000 0.76
99 A betaPP peptide carboxyl-terminal to Abeta is neurotoxic. Am J Pathol 1999 0.75
100 [Progressive polioencephalopathy with neurofibrillary degeneration]. Acta Neuropathol 1970 0.75
101 Fucosidosis: a neuropathological study. Riv Patol Nerv Ment 1976 0.75
102 Changes in excitability of CA1 pyramidal neurons in slices prepared from AlCl3-treated rabbits. Epilepsy Res 1990 0.75
103 [Lipofuscinsis of the nervous system. An experimental study]. J Neurol Sci 1974 0.75
104 Burst suppression and impairment of neocortical ontogenesis: electroclinical and neuropathologic findings in two infants with early myoclonic encephalopathy. Epilepsia 1993 0.75
105 [Italian Society for Alzheimer's disease: recommendations by the group for scientific coordination]. Riv Inferm 1991 0.75
106 [Metastasis of bronchial carcinoma to the choroid plexus and the roots of the cranial and spinal nerves. Anatomo-clinical observation]. Sist Nerv 1970 0.75
107 [Association of atrophy of the globus pallidus and dentate nucleus in a suckling. (Anatomo-clinical study)]. Acta Neurol (Napoli) 1971 0.75
108 [Further observations on experimental lipofuscinosis induced with tetraethylthiuram disulfide]. Acta Neurol (Napoli) 1971 0.75
109 [Chronic tubulo-interstitial nephropathy, tapetoretinal degeneration and generalized lipidosis. Analysis of an anatomoclinical case]. Arch Fr Pediatr 1971 0.75
110 Ventral root axonopathy and its relation to the neurofibrillary degeneration of lower motor neurons in aluminum-induced encephalomyelopathy. Neuropathol Appl Neurobiol 1986 0.75
111 [On the temporal transformation of abiotrophic phenotypes. From a heredoataxia to an ulcero-mutilating acropathy]. Psychiatr Neurol Neurochir 1967 0.75
112 FVEPs in Creutzfeldt-Jacob disease: waveforms and interaction with the periodic EEG pattern assessed by single sweep analysis. Clin Neurophysiol 2005 0.75
113 Nerve cell loss with aging in the putamen. Eur Neurol 1978 0.75
114 [A complex abiotrophy: from hereditary ataxia to acropathia ulcero-mutilans]. Acta Neurol (Napoli) 1969 0.75
115 Early degeneration of the cerebellar cortex, particularly the granular cells. J Neurol 1978 0.75
116 Synaptic and nonsynaptic determinants of excitability changes in aluminum-intoxicated rabbit CA1 pyramidal neurons studied in vitro. Epilepsy Res Suppl 1992 0.75
117 [Hyperlactacidemic and hyperpyruvicemic disoric encephalopathy]. Acta Neurol (Napoli) 1974 0.75
118 Endodermal sinus tumor of the pineal region presenting with a radicular pain. Eur Neurol 1986 0.75
119 Impairment of neocortical ontogenetic program leading to severe infantile encephalopathy with burst suppression. Epilepsy Res Suppl 1996 0.75
120 [The so-called lipophanerosis of the globus pallidus]. Acta Neurol (Napoli) 1970 0.75
121 Progressive dialytic encephalopathy and the problem of aluminum neurotoxicity. Clin Nephrol 1985 0.75
122 Hereditary sensory neuropathy with anhidrosis. A new family with a study of the sensory conduction velocity. Acta Neurol (Napoli) 1978 0.75
123 [Non-puerperal thrombosis of the superior longitudinal sinus in the adult. Anatomoclinical contribution]. Sist Nerv 1969 0.75
124 [Association of globular cell leukodystrophy with gliomatosis and abiotrophy]. Acta Neurol Psychiatr Belg 1968 0.75
125 [Encephalopathy with early onset and malignant evolution. (Analysis of an anatomoclinical case)]. Sist Nerv 1969 0.75
126 Cerebrospinal fluid levels of amyloid beta-protein precursor are low in Gerstmann-Sträussler-Scheinker disease, Indiana kindred. Neurology 1994 0.75
127 L-Dopa in children with progressive neurological disorders. Ann Neurol 1980 0.75
128 The thalamic hemorrhage. An anatomo-clinical study. Eur Neurol 1973 0.75
129 Primary immunodeficiency with early encephalopathy in two siblings. An anatomo-clinical study. Eur Neurol 1975 0.75
130 Desferrioxamine infusion can modify EEG tracing in haemodialysed patients. Nephrol Dial Transplant 1991 0.75
131 [Experimental lipofuscinosis induced by tetraethylthiuram disulfide]. Acta Neurol (Napoli) 1971 0.75
132 Minimal laboratory investigation of uremic patients. Kidney Int Suppl 1985 0.75
133 Addison-Schilder's disease. Report of a case with probable relapsing addisonian encephalopathy. Confin Neurol 1971 0.75
134 [Adrenocortical insufficiency and progressive encephalopathy. Clinical case]. Sist Nerv 1970 0.75
135 Is Lafora's disease really a disorder of carbohydrate metabolism? Pathologica 1979 0.75
136 [Recurrent encephalitis of the cerebral trunk. Analysis of an anatomo-clinical observation]. Encephale 1970 0.75