Abstract:
:Creutzfeldt-Jakob disease (CJD) is a rare dementia that is generally found in older people and is caused by unusual infectious pathogens or prions. Using rabbit antisera raised against hamster scrapie prion proteins (HaPrPSc), we identified by immunoblotting human CJD prion proteins (HuPrPCJD) in the brains of 14 patients dying of CJD. Extracts from 6 of the patients were transmitted to mice after prolonged incubation. The rabbit antisera raised against HaPrPSc also reacted with the mouse CJD prion proteins (MoPrPCJD) found in the brains of these experimentally infected mice. When mice were immunized with HuPrPCJD, they produced antibodies that reacted with HuPrPCJD but not with MoPrPCJD. Mice immunized with MoPrPCJD produced antibodies to neither murine nor human prion proteins. Our results provide evidence for host species-specific epitopes on prion proteins. The existence of such epitopes is consistent with the apparent lack of an immune response during prion infections and the finding that prion protein molecules are encoded by host genes.
journal_name
Ann Neuroljournal_title
Annals of neurologyauthors
Bockman JM,Prusiner SB,Tateishi J,Kingsbury DTdoi
10.1002/ana.410210611subject
Has Abstractpub_date
1987-06-01 00:00:00pages
589-95issue
6eissn
0364-5134issn
1531-8249journal_volume
21pub_type
杂志文章abstract::Visual (VEP), brainstem auditory (BAEP), and somatosensory (SEP) evoked potential tests were performed in 45 patients representing ten types of inherited disorders in which ataxia was the most prominent symptom. Comparable VEP abnormalities were present among all types of patients. Normal BAEP tests were recorded in m...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410130106
更新日期:1983-01-01 00:00:00
abstract::Excessive astrocytosis in cortical tubers in tuberous sclerosis complex (TSC) suggests that astrocytes may be important for epileptogenesis in TSC. We previously demonstrated that astrocyte-specific Tsc1 gene inactivation in mice (Tsc1 cKO mice) results in progressive epilepsy. Here, we report that glutamate transport...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.10648
更新日期:2003-08-01 00:00:00
abstract::Dopamine transporter messenger RNA (mRNA) expression was assessed by in situ hybridization over individual pigmented neurons from the substantia nigra pars compacta in midbrain sections from 7 parkinsonian and 7 age-matched, neurologically normal patients. In the normal control brains, high levels of expression of dop...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410350421
更新日期:1994-04-01 00:00:00
abstract:OBJECTIVE:To assess risk factors associated with care for stroke symptoms. METHODS:Using data from the population-based national cohort study (REasons for Geographic And Racial Differences in Stroke) conducted January 25, 2003-February 28, 2007 (N = 23,664), we assessed care-seeking behavior among 3,668 participants w...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.21357
更新日期:2008-04-01 00:00:00
abstract::The mucopolysaccharidoses are caused by inherited deficiencies of lysosomal enzymes involved in the degradative pathway of glycosaminoglycans. Lysosomal storage leads to cellular and organ dysfunction, including mental retardation. Storage lesions are found throughout the diseased brain, but little is known about the ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.10373
更新日期:2002-12-01 00:00:00
abstract:OBJECTIVE:The objective of this study was to investigate the role of CD8+ CD25+ FoxP3+ cells during the course of multiple sclerosis (MS). METHODS:Peripheral blood and cerebrospinal fluid (CSF) CD8+ T-cell clones (TCCs) recognizing autoreactive CD4+ T cells were isolated from 20 MS patients during exacerbations, 15 pa...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.21944
更新日期:2010-05-01 00:00:00
abstract:OBJECTIVE:T2-weighted magnetic resonance imaging is a sensitive tool for monitoring progression of multiple sclerosis, but it does not provide information on the severity of the underlying tissue damage. Measurement of T1 hypointensities and magnetization transfer ratio (MTR) can potentially distinguish lesions with mo...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.21113
更新日期:2007-09-01 00:00:00
abstract:OBJECTIVE:To test the hypothesis that variability in SNCA Rep1, a polymorphic dinucleotide microsatellite in the promoter region of the gene encoding α-synuclein, modifies the association between head injury and Parkinson's disease (PD) risk. METHODS:Participants in the Farming and Movement Evaluation (FAME) and the S...
journal_title:Annals of neurology
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1002/ana.22499
更新日期:2012-01-01 00:00:00
abstract::Two recent case-control studies have suggested a strong association of a missense polymorphism in exon 2 of the cathepsin D gene (CTSD) and Alzheimer disease (AD). However, these findings were not confirmed in another independent study. We analyzed this polymorphism in two large and independent AD study populations an...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/1531-8249(200101)49:1<114::aid-ana18>3.0.c
更新日期:2001-01-01 00:00:00
abstract::Changes in endoneurial fluid pressure (EFP) and morphology were studied in rat sciatic nerves frozen for 60 seconds with a cryoprobe designed for human cryoanalgesia. The onset of increased EFP was rapid, and a peak of 23 cm H2O was reached within 90 minutes after injury. EFP levels returned to normal 32 days after fr...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410100512
更新日期:1981-11-01 00:00:00
abstract:OBJECTIVE:Charcot-Marie-Tooth type 4J (CMT4J) is a rare autosomal recessive neuropathy caused by mutations in FIG4 that result in loss of FIG4 protein. This study investigates the natural history and mechanisms of segmental demyelination in CMT4J. METHODS:Over the past 9 years, we have enrolled and studied a cohort of...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.25198
更新日期:2018-04-01 00:00:00
abstract::A non-right-handed patient developed alexia without agraphia from a right occipital lobe infarction. An intracarotid amobarbital test showed left hemispheric dominance for speech. The cause of alexia in this patient could not be explained simply by the accepted disconnection hypothesis, which proposes that speech, han...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410030617
更新日期:1978-06-01 00:00:00
abstract::Measurement of conduction velocity along the H reflex arc was used to study sensorimotor peripheral nerve function in diabetic patients during short- and long-term improvement of hyperglycemia. In ten type I diabetics a slight (p less than 0.05) conduction increase occurred after 6 hours of normal glycemia induced by ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410160204
更新日期:1984-08-01 00:00:00
abstract:OBJECTIVE:Acquired epilepsy is a devastating long-term risk of various brain insults, including trauma, stroke, infections, and status epilepticus (SE). There is no preventive treatment for patients at risk. Attributable to the complex alterations involved in epileptogenesis, it is likely that multitargeted approaches ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24804
更新日期:2016-12-01 00:00:00
abstract::Interferon beta-1b reduces clinical exacerbations and disease activity in multiple sclerosis as shown by magnetic resonance imaging, but the mechanism of action is unknown. We investigated the correlation between the levels of soluble adhesion molecules and a reduction in contrast-enhancing lesions on gadopentetate di...
journal_title:Annals of neurology
pub_type: 临床试验,杂志文章
doi:10.1002/ana.410410517
更新日期:1997-05-01 00:00:00
abstract:OBJECTIVE:Lymphocytic choriomeningitis virus (LCMV) is a human pathogen and an emerging neuroteratogen. When the infection occurs during pregnancy, the virus can target and damage the fetal brain and retina. We examined the spectrum of clinical presentations, neuroimaging findings, and clinical outcomes of children wit...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.21161
更新日期:2007-10-01 00:00:00
abstract::Antibodies to Ma1 and Ma2 proteins identify a paraneoplastic disorder that affects the limbic system, brain stem, and cerebellum. Preliminary studies suggested the existence of other Ma proteins and different patterns of immune response associated with distinct neurologic symptoms and cancers. In this study, our aim w...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:
更新日期:2001-09-01 00:00:00
abstract::Analysis of the pathophysiology of hypokalemic paralysis, as it occurs in barium poisoning, chronic potassium deficiency, and thyrotoxicosis, suggests that these disorders may have a similar mechanism. An increased ratio of muscle sodium permeability to potassium permeability reduces the ionic diffusion potential, whi...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410110602
更新日期:1982-06-01 00:00:00
abstract::Layer II of the entorhinal cortex contains the cells of origin for the perforant path, plays a critical role in memory processing, and consistently degenerates in end-stage Alzheimer's disease. The extent to which neuron loss in layer II of entorhinal cortex is related to mild cognitive impairment without dementia has...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:
更新日期:2001-02-01 00:00:00
abstract::We report biochemical, immunological, and morphological findings in a patient with fatal Kearns-Sayre syndrome. Histochemical and biochemical findings from muscle biopsy specimens obtained 7 years apart documented the disease's evolution from a mild mitochondrial disorder affecting a small proportion of muscle fibers ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410210607
更新日期:1987-06-01 00:00:00
abstract::A dinucleotide repeat polymorphism in a tau intron was identified and used in a case-control study to analyze the genetic association of tau with several neurodegenerative diseases with tau pathology. Subjects with the homozygous tau AO alleles were excessively represented in the progressive supranuclear palsy (PSP) g...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410410222
更新日期:1997-02-01 00:00:00
abstract::We explored the hypothesis that components of verbal memory are subserved by separate temporal lobe structures in patients with temporal lobe structures in patients with temporal lobe epilepsy [correction]. Uptake of 18F-fluorodeoxyglucose (FDG) measured by positron emission tomography, hippocampal volume, and memory ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.10133
更新日期:2002-04-01 00:00:00
abstract::It is well recognized that plasma fluctuations resulting from oral levodopa therapy may cause an unstable clinical response in parkinsonian patients. We have therefore developed a slow-release polymer matrix system that can deliver levodopa continuously for extended periods of time (at least 225 days) after subcutaneo...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410280519
更新日期:1990-11-01 00:00:00
abstract::Human T-cell lymphotropic virus type III (HTLV-III) has been isolated from neural tissues and cerebrospinal fluid (CSF) of patients with neurological syndromes associated with the acquired immune deficiency syndrome (AIDS) and the virus may be directly involved in the pathogenesis of the syndromes. To detect HTLV-III ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410200304
更新日期:1986-09-01 00:00:00
abstract::The frequency with which patients presenting with acute or chronic noncompressive cord syndromes subsequently develop multiple sclerosis is uncertain. Magnetic resonance imaging (MRI) was performed on 121 patients with such syndromes to determine the frequency of asymptomatic brain lesions and to assess the sensitivit...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410220607
更新日期:1987-12-01 00:00:00
abstract::The pathogenetic role of anti-GM1 in chronic acquired demyelinating polyneuropathy (CADP) is uncertain. An association between antibodies and disease activity has not yet been established. In 8 patients with CADP followed longitudinally, anti-GM1 antibodies were monitored with a standardized enzyme-linked immunosorben...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410430113
更新日期:1998-01-01 00:00:00
abstract::We report the use of a new stable isotope-labeled form of levodopa (LD) to examine in vivo central LD metabolism in Parkinson's disease (PD). Eight patients representing a wide spectrum of disease severity were administered 50 mg of carbidopa orally followed in 1 hour by an intravenous bolus of 150 mg of stable isotop...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410420305
更新日期:1997-09-01 00:00:00
abstract::We performed dynamic [18F]fluorodeoxyglucose ([18F]FDG) positron emission tomographic (PET) analyses in 8 patients. Rate constants of influx (K1*), efflux (k2*), phosphorylation (k3*), and dephosphorylation (k4*) were derived for the regions of interest (ROIs), which included (1) the hypometabolic epileptogenic region...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410430615
更新日期:1998-06-01 00:00:00
abstract::We report on a 28-year-old man with long-standing intractable complex partial and secondary generalized seizures, whose magnetic resonance imaging scans 4 years apart documented progressive decrease in the left hippocampal volume. Left anterior temporal lobectomy with amygdalohippocampectomy rendered the patient seizu...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:
更新日期:1999-04-01 00:00:00
abstract::The long-term effects of brief but repetitive febrile seizures (FS) on memory have not been as thoroughly investigated as the impact of single and prolonged seizure in the developing brain. Using a heated-air FS paradigm, we subjected male rat pups to one, three, or nine episodes of brief FS on days 10 to 12 postpartu...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.10789
更新日期:2003-12-01 00:00:00