H A Kretzschmar

Author PubWeight™ 140.20‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Scrapie prion proteins are synthesized in neurons. Am J Pathol 1986 2.54
2 Subcellular localization of wild-type and Parkinson's disease-associated mutant alpha -synuclein in human and transgenic mouse brain. J Neurosci 2000 2.49
3 Genetic influence on the structural variations of the abnormal prion protein. Proc Natl Acad Sci U S A 2000 2.46
4 Molecular cloning of a human prion protein cDNA. DNA 1986 2.43
5 Detection of 14-3-3 protein in the cerebrospinal fluid supports the diagnosis of Creutzfeldt-Jakob disease. Ann Neurol 1998 2.35
6 Monocyte/macrophage differentiation in early multiple sclerosis lesions. Ann Neurol 1995 2.34
7 Role of microglia and host prion protein in neurotoxicity of a prion protein fragment. Nature 1996 2.20
8 Diagnosis of Creutzfeldt-Jakob disease by measurement of S100 protein in serum: prospective case-control study. BMJ 1998 2.15
9 Sporadic Creutzfeldt-Jakob disease: magnetic resonance imaging and clinical findings. Neurology 2004 2.15
10 Predictors of survival in sporadic Creutzfeldt-Jakob disease and other human transmissible spongiform encephalopathies. Brain 2004 1.96
11 The paraffin-embedded tissue blot detects PrP(Sc) early in the incubation time in prion diseases. Am J Pathol 2000 1.91
12 Diagnosis of Creutzfeldt-Jakob disease by two-dimensional gel electrophoresis of cerebrospinal fluid. Lancet 1996 1.91
13 Isolation, characterization, and organ-specific expression of two novel human zinc finger genes related to the Drosophila gene spalt. Genomics 1996 1.89
14 Tau protein and 14-3-3 protein in the differential diagnosis of Creutzfeldt-Jakob disease. Neurology 2002 1.84
15 Selective insolubility of alpha-synuclein in human Lewy body diseases is recapitulated in a transgenic mouse model. Am J Pathol 2001 1.81
16 Analysis of the systemic and intrathecal humoral immune response in progressive multifocal leukoencephalopathy. J Infect Dis 1997 1.79
17 Prion protein-deficient cells show altered response to oxidative stress due to decreased SOD-1 activity. Exp Neurol 1997 1.78
18 Early spread of scrapie from the gastrointestinal tract to the central nervous system involves autonomic fibers of the splanchnic and vagus nerves. J Virol 2001 1.73
19 Elevated levels of tau-protein in cerebrospinal fluid of patients with Creutzfeldt-Jakob disease. Neurosci Lett 1997 1.71
20 How to improve the clinical diagnosis of Creutzfeldt-Jakob disease. Brain 1999 1.71
21 Sporadic Creutzfeldt-Jakob disease: clinical and diagnostic characteristics of the rare VV1 type. Neurology 2005 1.65
22 Proteinase-resistant prion protein accumulation in Syrian hamster brain correlates with regional pathology and scrapie infectivity. Neurology 1991 1.61
23 Creutzfeldt-Jakob disease in Germany: a prospective 12-year surveillance. Brain 2007 1.56
24 [The Creutzfeld-Jakob disease. A sphinx of current neurobiology]. Dtsch Med Wochenschr 1997 1.55
25 Molecular genetics of human prion diseases in Germany. Hum Genet 1999 1.53
26 Transmission of Creutzfeldt-Jakob disease by handling of dura mater. Lancet 1993 1.52
27 Role of microglia in neuronal cell death in prion disease. Brain Pathol 1998 1.49
28 Current clinical diagnosis in Creutzfeldt-Jakob disease: identification of uncommon variants. Ann Neurol 2000 1.49
29 Efficacy of flupirtine on cognitive function in patients with CJD: A double-blind study. Neurology 2004 1.45
30 RNA aptamers specifically interact with the prion protein PrP. J Virol 1997 1.43
31 Patients with Alzheimer's disease and dementia with Lewy bodies mistaken for Creutzfeldt-Jakob disease. J Neurol Neurosurg Psychiatry 2001 1.39
32 C-MYC expression in medulloblastoma and its prognostic value. Int J Cancer 2000 1.37
33 The Heidenhain variant of Creutzfeldt-Jakob disease. Arch Neurol 1999 1.37
34 Hot spots in dynamic (18)FET-PET delineate malignant tumor parts within suspected WHO grade II gliomas. Neuro Oncol 2011 1.35
35 Alpha-synuclein accumulation in a case of neurodegeneration with brain iron accumulation type 1 (NBIA-1, formerly Hallervorden-Spatz syndrome) with widespread cortical and brainstem-type Lewy bodies. Acta Neuropathol 2000 1.33
36 Significant association of a M129V independent polymorphism in the 5' UTR of the PRNP gene with sporadic Creutzfeldt-Jakob disease in a large German case-control study. J Med Genet 2006 1.28
37 Mouse cortical cells lacking cellular PrP survive in culture with a neurotoxic PrP fragment. Neuroreport 1994 1.27
38 Detection of PrP(Sc) in subclinical BSE with the paraffin-embedded tissue (PET) blot. Arch Virol Suppl 2000 1.27
39 Effects of copper on survival of prion protein knockout neurons and glia. J Neurochem 1998 1.26
40 Fatal familial insomnia: a new Austrian family. Brain 1999 1.24
41 S-100 protein concentration in the cerebrospinal fluid of patients with Creutzfeldt-Jakob disease. J Neurol 1997 1.24
42 Pattern of cortical changes in sporadic Creutzfeldt-Jakob disease. AJNR Am J Neuroradiol 2007 1.24
43 The cerebellum-specific Munc13 isoform Munc13-3 regulates cerebellar synaptic transmission and motor learning in mice. J Neurosci 2001 1.22
44 14-3-3 CSF levels in sporadic Creutzfeldt-Jakob disease differ across molecular subtypes. Neurobiol Aging 2008 1.18
45 Cerebrospinal fluid concentration of neuron-specific enolase in diagnosis of Creutzfeldt-Jakob disease. Lancet 1995 1.17
46 Different variants of frontotemporal dementia: a neuropathological and immunohistochemical study. Acta Neuropathol 1996 1.13
47 Copper(II)-induced conformational changes and protease resistance in recombinant and cellular PrP. Effect of protein age and deamidation. J Biol Chem 2000 1.09
48 Decreased beta-amyloid1-42 in cerebrospinal fluid of patients with Creutzfeldt-Jakob disease. Neurology 2000 1.08
49 Progressive multifocal leukoencephalopathy diagnosed by amplification of JC virus-specific DNA from cerebrospinal fluid. AIDS 1994 1.06
50 Loss of glycosylation associated with the T183A mutation in human prion disease. Acta Neuropathol 2004 1.06
51 Giant axonal neuropathy: a generalized disorder of intermediate filaments with longitudinal grooves in the hair. Neuropediatrics 1994 1.06
52 Neuronal cell death in scrapie-infected mice is due to apoptosis. Brain Pathol 1995 1.04
53 Early age of onset in fatal familial insomnia. Two novel cases and review of the literature. J Neurol 2004 1.02
54 c-myc oncogene family expression in glioblastoma and survival. Surg Neurol 1999 1.01
55 Giant axonal neuropathy. A neuropathological study. Acta Neuropathol 1987 1.00
56 A neurotoxic prion protein fragment enhances proliferation of microglia but not astrocytes in culture. Glia 1996 0.97
57 Isoform pattern of 14-3-3 proteins in the cerebrospinal fluid of patients with Creutzfeldt-Jakob disease. J Neurochem 1999 0.97
58 Increase of neuron-specific enolase in patients with Creutzfeldt-Jakob disease. Neurosci Lett 1999 0.97
59 Pick's disease associated with the novel Tau gene mutation K369I. Ann Neurol 2001 0.97
60 Follow-up investigations in cerebrospinal fluid of patients with dementia with Lewy bodies and Alzheimer's disease. J Neural Transm (Vienna) 2004 0.97
61 Prion protein mutation in family first reported by Gerstmann, Sträussler, and Scheinker. Lancet 1991 0.97
62 Turnover of glial filaments in mouse spinal cord. J Neurochem 1986 0.96
63 Prion protein affects Ca2+-activated K+ currents in cerebellar purkinje cells. Neurobiol Dis 2001 0.95
64 Pyruvate dehydrogenase complex deficiency as a cause of subacute necrotizing encephalopathy (Leigh disease). Pediatrics 1987 0.95
65 Microglial expression of the prion protein. Neuroreport 1998 0.95
66 Phenotypic variability in fatal familial insomnia (D178N-129M) genotype. Neurology 1998 0.94
67 Differential constitutive and activation-dependent expression of prion protein in human peripheral blood leucocytes. Br J Haematol 2000 0.93
68 Macrophages in multiple sclerosis. Immunobiology 1996 0.92
69 Prion-induced neuronal damage--the mechanisms of neuronal destruction in the subacute spongiform encephalopathies. Curr Top Microbiol Immunol 2001 0.92
70 MRI in the classical MM1 and the atypical MV2 subtypes of sporadic CJD: an inter-observer agreement study. Eur J Neurol 2008 0.92
71 Novel twelve-generation kindred of fatal familial insomnia from germany representing the entire spectrum of disease expression. Am J Med Genet 1999 0.92
72 Neurotoxicity but not infectivity of prion proteins can be induced reversibly in vitro. Arch Virol Suppl 2000 0.91
73 Effects of oxidative stress on prion protein expression in PC12 cells. Int J Dev Neurosci 1997 0.91
74 A pathogenic presenilin-1 deletion causes abberrant Abeta 42 production in the absence of congophilic amyloid plaques. J Biol Chem 2000 0.90
75 Altered intracellular calcium homeostasis in cerebellar granule cells of prion protein-deficient mice. J Neurochem 2000 0.90
76 Immunoglobulins and virus-specific antibodies in patients with Creutzfeldt-Jakob disease. Acta Neurol Scand 2005 0.90
77 Clinical and differential diagnosis of Creutzfeldt-Jakob disease. Arch Virol Suppl 2000 0.90
78 Bcl-2 expression inversely correlates with tumour cell differentiation in medulloblastoma. Neuropathol Appl Neurobiol 2004 0.88
79 A proposal of new diagnostic pathway for fatal familial insomnia. J Neurol Neurosurg Psychiatry 2013 0.87
80 Polymorphism at codon 129 of the prion protein gene determines cerebellar pathology in Creutzfeldt-Jakob disease. Clin Neuropathol 1997 0.87
81 PrP and beta-amyloid fragments activate different neurotoxic mechanisms in cultured mouse cells. Eur J Neurosci 1997 0.87
82 Decreased CSF amyloid beta42 and normal tau levels in dementia with Lewy bodies. Neurology 2001 0.87
83 Prion protein expression in muscle cells and toxicity of a prion protein fragment. Eur J Cell Biol 1998 0.86
84 Definite multiple system atrophy in a German family. J Neurol Neurosurg Psychiatry 2009 0.85
85 [Clinically atypical CJD: diagnostic relevance of cerebrospinal fluid markers and molecular genetic analysis?]. Dtsch Med Wochenschr 2002 0.85
86 The glio-toxic mechanism of alpha-aminoadipic acid on cultured astrocytes. J Neurocytol 1998 0.83
87 Pattern of interleukin-6 receptor complex immunoreactivity between cortical regions of rapid autopsy normal and Alzheimer's disease brain. Eur Arch Psychiatry Clin Neurosci 2004 0.83
88 Prion protein fragment interacts with PrP-deficient cells. J Neurosci Res 1998 0.83
89 [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
90 A new family with frontotemporal dementia with intronic 10+3 splice site mutation in the tau gene: neuropathology and molecular effects. Neuropathol Appl Neurobiol 2005 0.82
91 Measurement of GFAP in hepatic encephalopathy by ELISA and transblots. J Neuropathol Exp Neurol 1985 0.82
92 MRI and clinical syndrome in dura mater-related Creutzfeldt-Jakob disease. J Neurol 2009 0.82
93 Mutant prion protein acquires resistance to protease in mouse neuroblastoma cells. J Gen Virol 2002 0.81
94 Polymorphisms within the prion-like protein gene (Prnd) and their implications in human prion diseases, Alzheimer's disease and other neurological disorders. Hum Genet 2001 0.81
95 Construction and characterization of murine neuroblastoma cell clones allowing inducible and high expression of the prion protein. J Gen Virol 1999 0.81
96 Putting prions into focus: application of single molecule detection to the diagnosis of prion diseases. Arch Virol Suppl 2000 0.81
97 Breaking an absolute species barrier: transgenic mice expressing the mink PrP gene are susceptible to transmissible mink encephalopathy. J Virol 2005 0.80
98 Postmortem delay and temperature conditions affect the in situ end-labeling (ISEL) assay in brain tissue of mice. Clin Neuropathol 1997 0.80
99 Recruitment of neural precursor cells from circumventricular organs of patients with cerebral ischaemia. Neuropathol Appl Neurobiol 2013 0.80
100 Evidence for neuronal apoptosis in pontosubicular neuron necrosis. Neuropathol Appl Neurobiol 1996 0.80
101 Increase of intracellular free Ca2+ in microglia activated by prion protein fragment. Glia 1997 0.79
102 Microglia and prion disease: a review. Histol Histopathol 1997 0.79
103 Spongiform central nervous system myelinopathy in African dwarf goats. J Comp Pathol 1995 0.79
104 Potential prognostic value of C-erbB-2 expression in medulloblastomas in very young children. J Pediatr Hematol Oncol 1998 0.79
105 Signs of rapidly progressive dementia in a case of intravascular lymphomatosis. Eur Arch Psychiatry Clin Neurosci 2004 0.79
106 [German consortium for frontotemporal lobar degeneration]. Nervenarzt 2011 0.79
107 A prion protein fragment primes type 1 astrocytes to proliferation signals from microglia. Neurobiol Dis 1998 0.78
108 Immunohistochemistry of primary central nervous system malignant rhabdoid tumors: report of five cases and review of the literature. Acta Neuropathol 1996 0.78
109 Somatostatin receptor scintigraphy in the management of cerebral malignant ectomesenchymoma: a case report. Pediatr Radiol 2001 0.78
110 Poly(ADP-ribose) immunostaining to detect apoptosis induced by a neurotoxic fragment of prion protein. Histochem J 1999 0.77
111 Creutzfeldt-Jakob disease and oxidative stress. Acta Neurol Scand 2000 0.77
112 [Hereditary diffuse leukencephalopathy with spheroids: a microgliopathy due to CSF1 receptor impairment]. Nervenarzt 2014 0.77
113 A novel three extra-repeat insertion in the prion protein gene (PRNP) in a patient with Creutzfeldt-Jakob disease. Neurogenetics 2004 0.77
114 Clinical range and MRI in Creutzfeldt-Jakob disease with heterozygosity at codon 129 and prion protein type 2. J Neurol Neurosurg Psychiatry 1999 0.77
115 CSF lactate dehydrogenase activity in patients with Creutzfeldt-Jakob disease exceeds that in other dementias. Dement Geriatr Cogn Disord 2004 0.77
116 Magnetic resonance imaging in Creutzfeldt-Jakob disease: evidence of focal involvement of the cortex. J Neurol Neurosurg Psychiatry 1997 0.76
117 False-positive pulvinar sign on MRI in sporadic Creutzfeldt-Jakob disease. Neurology 2004 0.76
118 [Epidemiology and clinical symptomatology of Creutzfeldt-Jakob disease]. Dtsch Med Wochenschr 2002 0.76
119 Cellular composition and distribution of gliomesenchymal nodules in the CNS of AIDS patients. Clin Neuropathol 1993 0.76
120 Expression of BARHL1 in medulloblastoma is associated with prolonged survival in mice and humans. Oncogene 2011 0.76
121 [Molecular neuropathology of Non-Alzheimer dementia]. Pathologe 2008 0.75
122 Expression of T cell receptor gamma delta antigens in human brain tissue. Neuroimmunomodulation 1996 0.75
123 Automated decontamination of surface-adherent prions. J Hosp Infect 2010 0.75
124 Influence of the blood-CSF-barrier function on S100B in neurodegenerative diseases. Acta Neurol Scand 2013 0.75
125 [Dementia and psychotic symptoms in amyotrophic lateral sclerosis]. Nervenarzt 1993 0.75
126 Unaltered apoptotic behaviour of mononuclear cells from patients with sporadic Creutzfeldt-Jakob disease. J Neurol 2001 0.75
127 Fatal spongiform encephalopathy in a patient who had handled animal feed. Lancet 1996 0.75
128 [Molecular biological identification of fungal pathogens in FFPE tissue from cases of cephalic mycosis]. Pathologe 2013 0.75
129 [Fatal familial insomnia--a rare differential diagnosis in dementia]. Fortschr Neurol Psychiatr 2008 0.75
130 Topographical reinnervation of the toad glutaeus muscle by axons of only one spinal nerve. Neuroreport 1995 0.75
131 Antigenic heterogeneity of human brain tumors defined by monoclonal antibodies. Anticancer Res 1990 0.75
132 Disease latency in Creutzfeldt-Jakob disease via dural grafting: a case report. Eur J Epidemiol 2001 0.75
133 Progressive dementia with parkinsonism in corticobasal and brainstem degeneration with neuronal inclusions. Neurology 1998 0.75
134 [Creutzfeldt-Jakob disease. Report of a case with an unusually long course and immunohistochemical localization of the prion protein and overview of current information]. Nervenarzt 1994 0.75
135 Crossed cerebellar diaschisis: a clue to the mechanism of ataxic hemiparesis in Creutzfeldt-Jakob disease? J Neurol 2001 0.75
136 Familial Creutzfeldt-Jakob disease with a novel 120-bp insertion in the prion protein gene. Ann Neurol 1999 0.75
137 Decreased prion protein expression in human peripheral blood leucocytes from patients with paroxysmal nocturnal haemoglobinuria. Br J Haematol 2001 0.75
138 Carbohydrate histochemistry of amyloid plaques in Gerstmann-Sträussler syndrome. Eur J Basic Appl Histochem 1991 0.75