Creutzfeldt-Jakob disease associated with a deletion of two repeats in the prion protein gene.

PubWeight™: 0.84‹?›

🔗 View Article (PMID 12451210)

Published in Neurology on November 26, 2002

Authors

S Capellari1, P Parchi, B D Wolff, J Campbell, R Atkinson, D M Posey, R B Petersen, P Gambetti

Author Affiliations

1: Institute of Pathology and National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, OH 44106, USA.

Articles by these authors

Validation study of WOMAC: a health status instrument for measuring clinically important patient relevant outcomes to antirheumatic drug therapy in patients with osteoarthritis of the hip or knee. J Rheumatol (1988) 35.68

The Mouse Genome Database (MGD): integrating biology with the genome. Nucleic Acids Res (2004) 8.91

The Mouse Genome Database (MGD): from genes to mice--a community resource for mouse biology. Nucleic Acids Res (2005) 8.19

Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol (1999) 6.98

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

Anterior pituitary extracts and liver fat. J Physiol (1936) 5.94

The intersections of HIV and violence: directions for future research and interventions. Soc Sci Med (2000) 5.78

Oxidative damage is the earliest event in Alzheimer disease. J Neuropathol Exp Neurol (2001) 4.80

Mitochondrial abnormalities in Alzheimer's disease. J Neurosci (2001) 4.39

Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Ann Neurol (1996) 4.10

Evaluation of the necessity for routine terminal subcultures of previously negative blood cultures. J Clin Microbiol (1980) 4.08

Fatal familial insomnia and familial Creutzfeldt-Jakob disease: disease phenotype determined by a DNA polymorphism. Science (1992) 3.93

Viscosupplementation for the treatment of osteoarthritis of the knee. Cochrane Database Syst Rev (2006) 3.65

The effect of anterior pituitary extracts on the insulin content of the pancreas. J Physiol (1939) 3.54

Short-term effects of particulate air pollution on cardiovascular diseases in eight European cities. J Epidemiol Community Health (2002) 3.29

Truncated forms of the human prion protein in normal brain and in prion diseases. J Biol Chem (1995) 3.20

The role of cutaneous diphtheria infections in a diphtheria epidemic. J Infect Dis (1975) 3.08

Complete genome sequence of Vibrio fischeri: a symbiotic bacterium with pathogenic congeners. Proc Natl Acad Sci U S A (2005) 3.05

Plasmonic nanorod metamaterials for biosensing. Nat Mater (2009) 2.94

A new model of diffuse brain injury in rats. Part I: Pathophysiology and biomechanics. J Neurosurg (1994) 2.90

Paired helical filaments from Alzheimer disease patients contain cytoskeletal components. Proc Natl Acad Sci U S A (1985) 2.86

Molecular assessment of the potential transmissibilities of BSE and scrapie to humans. Nature (1997) 2.80

Presence of soluble amyloid beta-peptide precedes amyloid plaque formation in Down's syndrome. Nat Med (1996) 2.71

Intraarticular corticosteroid for treatment of osteoarthritis of the knee. Cochrane Database Syst Rev (2006) 2.59

Prozone phenomenon in secondary syphilis. Has its time arrived? Arch Intern Med (1993) 2.55

The effect of insulin and anterior pituitary extract on the insulin content of the pancreas and the histology of the islets. J Physiol (1942) 2.48

Persistent hepatitis and enterocolitis in germfree mice infected with Helicobacter hepaticus. Infect Immun (1996) 2.47

Genetic influence on the structural variations of the abnormal prion protein. Proc Natl Acad Sci U S A (2000) 2.46

FLP-mediated DNA mobilization to specific target sites in Drosophila chromosomes. Nucleic Acids Res (1997) 2.46

Sickle-cell trait as a risk factor for sudden death in physical training. N Engl J Med (1987) 2.44

Cancer-associated hypercalcemia: morbidity and mortality. Clinical experience in 126 treated patients. Ann Intern Med (1990) 2.43

Immunohistochemical localization of glial fibrillary acidic protein in human glial neoplasms. Cancer (1980) 2.26

The primate lentiviral receptor Bonzo/STRL33 is coordinately regulated with CCR5 and its expression pattern is conserved between human and mouse. J Immunol (2000) 2.22

SSB-1 of the yeast Saccharomyces cerevisiae is a nucleolar-specific, silver-binding protein that is associated with the snR10 and snR11 small nuclear RNAs. J Cell Biol (1990) 2.21

Morvan's syndrome: peripheral and central nervous system and cardiac involvement with antibodies to voltage-gated potassium channels. Brain (2001) 2.14

Early loss of heterozygosity on 17q in ovarian cancer. The Abe Ovarian Cancer Genetics Group. Oncogene (1992) 2.13

The widespread alteration of neurites in Alzheimer's disease may be unrelated to amyloid deposition. Ann Neurol (1989) 2.07

Countercurrent immunoelectrophoresis in the evaluation of childhood infections. J Pediatr (1974) 2.06

Ubiquitin is associated with abnormal cytoplasmic filaments characteristic of neurodegenerative diseases. Proc Natl Acad Sci U S A (1988) 2.04

The Department of Military Medicine--a graduate medical education idea whose time has come. Mil Med (1989) 2.04

Proteome-based plasma biomarkers for Alzheimer's disease. Brain (2006) 1.98

A novel phenotype in familial Creutzfeldt-Jakob disease: prion protein gene E200K mutation coupled with valine at codon 129 and type 2 protease-resistant prion protein. Ann Neurol (1999) 1.98

Presenilin-1 mutations in Alzheimer's disease. Nature (2000) 1.97

Ataxic sensory neuropathy and dorsal root ganglionitis associated with Sjögren's syndrome. Ann Neurol (1990) 1.92

Understanding ethnic and other socio-demographic differences in patient experience of primary care: evidence from the English General Practice Patient Survey. BMJ Qual Saf (2011) 1.92

pH-dependent stability and conformation of the recombinant human prion protein PrP(90-231). J Biol Chem (1997) 1.85

Different patterns of truncated prion protein fragments correlate with distinct phenotypes in P102L Gerstmann-Sträussler-Scheinker disease. Proc Natl Acad Sci U S A (1998) 1.85

Fatal familial insomnia and familial Creutzfeldt-Jakob disease: different prion proteins determined by a DNA polymorphism. Proc Natl Acad Sci U S A (1994) 1.85

Membrane environment alters the conformational structure of the recombinant human prion protein. J Biol Chem (1999) 1.83

Typing prion isoforms. Nature (1997) 1.82

Prion protein expression in different species: analysis with a panel of new mAbs. Proc Natl Acad Sci U S A (1998) 1.80

Synthesis in vitro of a seven amino acid peptide encoded in the leader RNA of Rous sarcoma virus. J Mol Biol (1986) 1.78

Parkinson disease, dementia, and Alzheimer disease: clinicopathological correlations. Ann Neurol (1980) 1.78

Interobserver reliability and intraobserver reproducibility of the system of King et al. for the classification of adolescent idiopathic scoliosis. J Bone Joint Surg Am (1998) 1.74

Fatal familial insomnia and dysautonomia with selective degeneration of thalamic nuclei. N Engl J Med (1986) 1.73

The Functional Independence Measure for Children (WeeFIM). Conceptual basis and pilot use in children with developmental disabilities. Clin Pediatr (Phila) (1994) 1.73

Neurofibrillary changes in human brain. An immunocytochemical study with a neurofilament antiserum. J Neuropathol Exp Neurol (1983) 1.72

A survey of current prescribing practices of antiinflammatory and urate lowering drugs in gouty arthritis in the province of Ontario. J Rheumatol (1988) 1.71

Long-term follow-up confirms a survival advantage of the paclitaxel-cisplatin regimen over the cyclophosphamide-cisplatin combination in advanced ovarian cancer. Int J Gynecol Cancer (2003) 1.70

Excess males in preterm birth: interactions with gestational age, race, and multiple birth. Obstet Gynecol (1996) 1.69

Retracted Confocal chromoscopic endomicroscopy is superior to chromoscopy alone for the detection and characterisation of intraepithelial neoplasia in chronic ulcerative colitis. Gut (2008) 1.68

Heme oxygenase-1 is associated with the neurofibrillary pathology of Alzheimer's disease. Am J Pathol (1994) 1.62

SLCO1B1 genetic variant associated with statin-induced myopathy: a proof-of-concept study using the clinical practice research datalink. Clin Pharmacol Ther (2013) 1.61

Absence of protease-resistant prion protein in the cerebrospinal fluid of Creutzfeldt-Jakob disease. J Pathol (2001) 1.61

Morphometric analysis of rat superior cervical ganglion after axotomy and nerve growth factor treatment. J Neurocytol (1976) 1.60

Cisplatin-based chemotherapy for ovarian germ cell malignancies: the Australian experience. J Clin Oncol (1994) 1.59

Bodian's silver method stains neurofilament polypeptides. Science (1981) 1.59

Domestic violence against patients with chronic neurologic disorders. Arch Neurol (1999) 1.59

Osteoarthritis antirheumatic drug trials. III. Setting the delta for clinical trials--results of a consensus development (Delphi) exercise. J Rheumatol (1992) 1.57

The effect of combined androgen blockade on bone turnover and bone mineral densities in men treated for prostate carcinoma: longitudinal evaluation and response to intermittent cyclic etidronate therapy. Cancer (1998) 1.57

The mother-headed single-parent family: a feminist critique of the nursing literature. Nurs Outlook (1997) 1.57

Clinical features of fatal familial insomnia: phenotypic variability in relation to a polymorphism at codon 129 of the prion protein gene. Brain Pathol (1998) 1.56

Primary auditory stream segregation and perception of order in rapid sequences of tones. J Exp Psychol (1971) 1.54

Double blind randomized controlled trial of sodium meclofenamate (Meclomen) and diclofenac sodium (Voltaren): post validation reapplication of the WOMAC Osteoarthritis Index. J Rheumatol (1992) 1.53

Aggregation and fibrillization of the recombinant human prion protein huPrP90-231. Biochemistry (2000) 1.52

Research trends in British gastroenterology: publication rates in newly appointed NHS consultants over a nine year period. Gut (2004) 1.52

EMR using dextrose solution versus sodium hyaluronate for colorectal Paris type I and 0-II lesions: a randomized endoscopist-blinded study. Endoscopy (2008) 1.50

Incessant ovulation and ovarian cancer. Lancet (1991) 1.49

Sensitivity of 14-3-3 protein test varies in subtypes of sporadic Creutzfeldt-Jakob disease. Neurology (2004) 1.49

Dimensionality and clinical importance of pain and disability in hand osteoarthritis: Development of the Australian/Canadian (AUSCAN) Osteoarthritis Hand Index. Osteoarthritis Cartilage (2002) 1.47

Identification of an epitope in the C terminus of normal prion protein whose expression is modulated by binding events in the N terminus. J Mol Biol (2000) 1.44

Fungiform papillary glossitis: atopic disease in the mouth? Br J Dermatol (2005) 1.44

Pathologic correlates of diffusion MRI changes in Creutzfeldt-Jakob disease. Neurology (2009) 1.44

Helicobacter pylori-induced gastritis in the domestic cat. Infect Immun (1995) 1.43

Inherited Creutzfeldt-Jakob disease in a Dutch patient with a novel five octapeptide repeat insertion and unusual cerebellar morphology. J Neurol Neurosurg Psychiatry (2009) 1.43

Prior score availability: observations using the WOMAC osteoarthritis index. Br J Rheumatol (1991) 1.43

Prospective audit of an extramucosal technique for intestinal anastomosis. Br J Surg (1991) 1.43

Striatal [123I] FP-CIT SPECT demonstrates dopaminergic deficit in a sporadic case of Creutzfeldt-Jakob disease. Acta Neurol Scand (2008) 1.43

Familial mutations and the thermodynamic stability of the recombinant human prion protein. J Biol Chem (1998) 1.43