Abstract:
:Aggregated tau proteins constitute the basic matrix of neuronal inclusions specific to numerous neurodegenerative disorders. Monodimensional and two-dimensional Western blot analyses performed on cortical brain homogenates allowed discrimination between disease-specific tau protein profiles. These observations raised the issue of the physiopathological significance of such specificities. Alzheimer's disease (AD) pathological tau proteins (PTPs) (tau 74, 69, 64, 55) were compared with those of Pick's disease (PiD) (tau 64, 55) using a panel of antibodies against peptidic sequences of tau isoforms corresponding to exons 2, 3, and 10. AD and PiD could then be critically differentiated by the absence of translated tau isoforms with exon 10 in PiD PTPs, along with the absence of the phosphorylation site on Ser262. Immunohistochemical studies corroborate these findings. Indeed, Pick bodies were strongly immunostained by an anti-"exon 2" antibody but failed to reveal any anti-exon 10 reactive epitope. Tangles in AD contained exon 2, 3, and 10 epitopes. Altogether, our results demonstrated that Pick bodies develop within specific neuronal subsets that express specific patterns of 7 isoforms lacking exon 10 peptidic sequence. We conclude that neurodegenerative disorders imply attrition of selectively vulnerable neuronal subsets, a process revealed, and may be sustained by specific tau isoform patterns.
journal_name
Ann Neuroljournal_title
Annals of neurologyauthors
Delacourte A,Sergeant N,Wattez A,Gauvreau D,Robitaille Ydoi
10.1002/ana.410430209subject
Has Abstractpub_date
1998-02-01 00:00:00pages
193-204issue
2eissn
0364-5134issn
1531-8249journal_volume
43pub_type
杂志文章abstract:OBJECTIVE:Osteopontin (OPN), a pleiotropic extracellular matrix glycoprotein, has been reported to be protective against ischemic lesions, but effects of OPN on vascular functions have not been investigated. The aim of this study was to assess whether recombinant OPN (r-OPN) could prevent cerebral vasospasm after subar...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.22102
更新日期:2010-11-01 00:00:00
abstract:OBJECTIVE:To investigate in older adults without dementia the relationships between socioeconomic status (SES) in childhood and magnetic resonance imaging (MRI)-derived brain volume measures typical of brain aging and Alzheimer's disease (AD). METHODS:Using a cross-sectional and longitudinal observation approach, we i...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.22631
更新日期:2012-05-01 00:00:00
abstract::Unverricht-Lundborg disease is a clinically recognizable form of progressive myoclonus epilepsy. Recently, in several families of both Finnish and Mediterranean extraction segregating Unverricht-Lundborg disease, the gene for this disease was linked to the same region of the long arm of chromosome 21. We performed lin...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410340519
更新日期:1993-11-01 00:00:00
abstract::Charcot-Marie-Tooth (CMT) neuropathies are inherited neuromuscular disorders caused by a length-dependent neurodegeneration of peripheral nerves. More than 900 mutations in 60 different genes are causative of the neuropathy. Despite significant progress in therapeutic strategies, the disease remains incurable. The inc...
journal_title:Annals of neurology
pub_type: 杂志文章,评审
doi:10.1002/ana.23987
更新日期:2013-09-01 00:00:00
abstract:OBJECTIVE:Visual Evoked Potentials (VEPs) following optic neuritis (ON) remain chronically prolonged, although standard visual tests indicate full recovery. We hypothesized that dynamic visual processes, such as motion perception, may be more vulnerable to slowed conduction in the optic nerve, and consequently be bette...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.22692
更新日期:2012-04-01 00:00:00
abstract:OBJECTIVE:Neuronal loss, a key substrate of irreversible disability in multiple sclerosis (MS), is a recognized feature of MS cortical pathology of which the cause remains unknown. Fibrin(ogen) deposition is neurotoxic in animal models of MS, but has not been evaluated in human progressive MS cortex. The aim of this st...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24997
更新日期:2017-08-01 00:00:00
abstract::We report on a new allele at the arylsulfatase A (ARSA) locus causing late-onset metachromatic leukodystrophy (MLD). In that allele arginine84, a residue that is highly conserved in the arylsulfatase gene family, is replaced by glutamine. In contrast to alleles that cause early-onset MLD, the arginine84 to glutamine s...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410310305
更新日期:1992-03-01 00:00:00
abstract:OBJECTIVE:Oral anticoagulation treatment (OAT) resumption is a therapeutic dilemma in intracerebral hemorrhage (ICH) care, particularly for lobar hemorrhages related to amyloid angiopathy. We sought to determine whether OAT resumption after ICH is associated with long-term outcome, accounting for ICH location (ie, loba...
journal_title:Annals of neurology
pub_type: 杂志文章,meta分析,多中心研究
doi:10.1002/ana.25079
更新日期:2017-11-01 00:00:00
abstract::Tetrabenazine is considered to act in a manner similar to reserpine to reduce the involuntary movements of tardive dyskinesia or Huntington's disease and to improve psychoses. We determined that tetrabenazine also has properties of a dopamine receptor antagonist by testing the ability of tetrabenazine to block the inh...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410120308
更新日期:1982-09-01 00:00:00
abstract::Platelet-derived growth factor (PDGF) ligand is a potent glial cell mitogen. When its cognate receptor (PDGF-alphaR) is expressed on oligodendroglial lineage cells, such cells are considered capable of division, and the receptor thus serves as a phenotypic marker for oligodendrocyte precursor cells. Here we identify u...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.1015
更新日期:2001-06-01 00:00:00
abstract:OBJECTIVE:To analyze the cumulative efficacy and safety of everolimus in treating subependymal giant cell astrocytomas (SEGA) associated with tuberous sclerosis complex (TSC) from an open-label phase II study (NCT00411619). Updated data became available from the conclusion of the extension phase and are presented in th...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24523
更新日期:2015-12-01 00:00:00
abstract::Autosomal dominant spinocerebellar ataxias (SCAs) are clinically and genetically a heterogeneous group of neurodegenerative disorders. Recently, mild CAG repeat expansion in the alpha1A voltage-dependent calcium channel gene has been found to be associated with a type of autosomal dominant SCA (SCA6). We analyzed 98 J...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410420609
更新日期:1997-12-01 00:00:00
abstract::Regional cerebral blood flow (rCBF) was measured in human subjects during saccadic eye movements by a 254-channel dynamic gamma camera. Focal rCBF increases were repeatedly observed in an area within the middle precentral and premotor regions which corresponds to the frontal eye field in humans. Our findings suggest t...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410050112
更新日期:1979-01-01 00:00:00
abstract::Intranuclear inclusions are one of the ultrastructural hallmarks of oculopharyngeal muscular dystrophy (OPMD), a disorder caused by small polyalanine (GCG) expansions in the gene that codes for a ubiquitous nuclear protein called poly(A) binding protein 2 (PABP2). We studied OPMD skeletal muscle and found that 1.0 to ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:
更新日期:2000-11-01 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::Mitochondrial iron accumulation is thought to underlie the pathophysiology of Friedreich ataxia and may occur at the expense of cytosolic iron. Decreases in cytosolic iron induce expression of the transferrin receptor, some of which is released into the serum. Here, we demonstrate that serum transferrin receptor conce...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:
更新日期:2000-05-01 00:00:00
abstract::Fresh brain weight, gestational age, body weight, sex, and race were collected from autopsy records of 782 newborns over a 10-year period. The brain weight of the mature newborn does not differ between males and females or between white and black infants. For the premature, however, brain weight is heavier in white ma...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410100308
更新日期:1981-09-01 00:00:00
abstract:OBJECTIVE:Chorioamnionitis is associated with increased risk for cerebral palsy (CP) in term infants. A functional polymorphism in the interleukin-6 (IL-6) gene has been implicated in newborn brain injury. We studied whether the IL-6 -174 G/C polymorphism confers increased risk for CP in term infants. METHODS:This pop...
journal_title:Annals of neurology
pub_type: 杂志文章,随机对照试验
doi:10.1002/ana.21766
更新日期:2009-11-01 00:00:00
abstract::We prospectively recruited 10 patients who presented with urinary retention as a neurological deficit that was attributable to lateral medullary infarction. Of these, 9 patients underwent a urodynamic study, which demonstrated detrusor underactivity of the bladder in 7 patients. Urinary retention developed mainly when...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24379
更新日期:2015-04-01 00:00:00
abstract::Neural tube defects (NTDs) are among the most common birth defects in humans and yet their molecular etiology remains poorly understood. NTDs are believed to result from the complex interaction of environmental factors with a multitude of genetic risk factors in a classical multifactorial disease model. Mendelian form...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.25152
更新日期:2018-02-01 00:00:00
abstract:OBJECTIVE:Deletions of CACNA1A, encoding the α1 subunit of CaV 2.1 channels, cause epilepsy with ataxia in humans. Whereas the deletion of Cacna1a in γ-aminobutyric acidergic (GABAergic) interneurons (INs) derived from the medial ganglionic eminence (MGE) impairs cortical inhibition and causes generalized seizures in N...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.25301
更新日期:2018-09-01 00:00:00
abstract:OBJECTIVE:The primary imaging modalities used to select patients for endovascular thrombectomy (EVT) are noncontrast computed tomography (CT) and CT perfusion (CTP). However, their relative utility is uncertain. We prospectively assessed CT and CTP concordance/discordance and correlated the imaging profiles on both wit...
journal_title:Annals of neurology
pub_type: 杂志文章,多中心研究
doi:10.1002/ana.25669
更新日期:2020-03-01 00:00:00
abstract::Indirect evidence suggests that an autoimmune response to myelin basic protein (MBP) may be involved in the pathogenesis of multiple sclerosis (MS). In MS, several reports have suggested that restricted T-cell populations respond to MPB, as in inbred rodents with the MS disease model experimental allergic encephalomye...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410340313
更新日期:1993-09-01 00:00:00
abstract:OBJECTIVE:To determine the clinical consequences of the PMP22 point mutation, T118M, which has been previously considered to either cause an autosomal recessive form of Charcot-Marie-Tooth (CMT) disease or be a benign polymorphism. METHODS:We analyzed patients from five separate kindreds and characterized their periph...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.20777
更新日期:2006-02-01 00:00:00
abstract::D2 dopamine receptor densities were measured in postmortem samples of the caudate nucleus and putamen from 36 parkinsonian patients. The relationship between the age of the patient, duration of the disease, and duration of L-dopa therapy versus density of brain D2 dopamine receptors was examined using [3H]spiperone. R...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410190510
更新日期:1986-05-01 00:00:00
abstract::Huntington's disease is an inherited disorder caused by expansion of a CAG trinucleotide repeat in the IT15 gene, which leads to expansion of a polyglutamine tract within the protein called huntingtin. Despite the characterization of the IT15 gene and the mutation involved in the disease, the normal function of huntin...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410420507
更新日期:1997-11-01 00:00:00
abstract:OBJECTIVE:Mutations of the selenoprotein N gene (SEPN1) cause SEPN1-related myopathy (SEPN1-RM), a novel early-onset muscle disorder formerly divided into four different nosological categories. Selenoprotein N (SelN) is the only selenoprotein involved in a genetic disease; its function being unknown, no treatment is av...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.21644
更新日期:2009-06-01 00:00:00
abstract::Mutations in genes encoding the NADH ubiquinone oxidoreductase, complex I of the respiratory chain, cause a diverse group of diseases. They include Leber hereditary optic neuropathy, Leigh syndrome, and mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes. There is no effective treatment for t...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.20175
更新日期:2004-08-01 00:00:00
abstract::Despite many years of intensive research, multiple sclerosis (MS) defies understanding and treatment remains suboptimal. The prevailing hypothesis is that MS is immune mediated and that experimental allergic encephalomyelitis (EAE) is a suitable model to elucidate pathogenesis and devise therapy. This review examines ...
journal_title:Annals of neurology
pub_type: 杂志文章,评审
doi:10.1002/ana.20743
更新日期:2005-12-01 00:00:00
abstract:OBJECTIVE:C9orf72 expansion is the most common genetic cause of frontotemporal dementia (FTD). We examined aging trajectories of cortical thickness (CTh) and surface area in C9orf72 expansion adult carriers compared to healthy controls to characterize preclinical cerebral changes leading to symptoms. METHODS:Data were...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.25748
更新日期:2020-07-01 00:00:00