Abstract:
:The nucleotide sequence of human T-lymphotropic virus type I (HTLV-I) in central nervous system tissue was determined in 3 autopsy cases with HTLV-I-associated myelopathy (HAM)/tropical spastic paraparesis (TSP) and 1 seropositive carrier without HAM/TSP but with multiple sclerosis. All HAM/TSP samples (3 spinal cords and 2 brains) and the sample from the seropositive carrier without HAM/TSP (brain) were positive for HTLV-I env (5146-6681), pX5' (6549-7494), and pX3' (7354-8276) regions by the two-step polymerase chain reaction method. A nucleotide sequence analysis of the pX5' and pX3' polymerase chain reaction products from nucleotides 6631 to 8259 revealed heterogeneity of the HTLV-I genome in all cases. It is notable that 13 of 50 clones derived from the pX3' polymerase chain reaction products were defective in the tax open reading frame while 7 were defective in the rex open reading frame in the HAM/TSP samples. All 17 clones from 1 HAM/TSP case were defective in the pX open reading frame II. One nucleotide insertion at 7784 creating a frame shift in both tax and rex was seen in all 3 HAM/TSP cases but not in the HTLV-I carrier without HAM/TSP. The pX-defective mutants found frequently in the central nervous system may contribute to the neural damage, since the pX gene products are essential for the transactivation of various cellular genes as well as for viral replication.
journal_name
Ann Neuroljournal_title
Annals of neurologyauthors
Kira J,Koyanagi Y,Yamada T,Itoyama Y,Tateishi J,Akizuki S,Kishikawa M,Baba E,Nakamura M,Suzuki Jdoi
10.1002/ana.410360206subject
Has Abstract,Author List Incompletepub_date
1994-08-01 00:00:00pages
149-56issue
2eissn
0364-5134issn
1531-8249journal_volume
36pub_type
杂志文章abstract::In Machado-Joseph disease (MJD) gene, there is a C/G polymorphism immediately after the CAG repeat; the expanded CAG repeat tract is exclusively followed by C, whereas about half of wild-type alleles are followed by G. Using this C/G polymorphism, we have engineered the small interfering RNA (siRNA) which decreased th...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.20141
更新日期:2004-07-01 00:00:00
abstract::The past decade has seen advances in the management of patients with epilepsy. The development of practical long-term electroencephalographic techniques, with or without simultaneous video recording, has increased the accuracy of diagnosis of seizure types. The technique also provides clinicians and investigators with...
journal_title:Annals of neurology
pub_type: 杂志文章,评审
doi:10.1002/ana.410090103
更新日期:1981-01-01 00:00:00
abstract::Positron emission tomography was used to study the effect of supratentorial infarction on cerebellar metabolic rate for oxygen and cerebellar blood flow. In a control group of patients, the mean cerebellar metabolic rate for oxygen was 2.97 +/- 0.11 (standard error of the mean [SEM] ) ml-1 . min-1 . hg-1 and mean cere...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410140203
更新日期:1983-08-01 00:00:00
abstract::The role of mitochondrial DNA (mtDNA) mutations in the pathogenesis of Leber's hereditary optic neuropathy (LHON) has yet to be characterized. Several clinical features of the disease imply that nuclear genes might also be involved in its expression. We have confirmed the presence of a severe NADH:coenzyme Q1 reductas...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410440208
更新日期:1998-08-01 00:00:00
abstract::We examined the phenotypic variation and clinical genetics in nine families with generalized epilepsy with febrile seizures plus (GEFS+). This genetic epilepsy syndrome with heterogeneous phenotypes was hitherto described in only one family. We obtained genealogical information on 799 individuals and conducted detaile...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/1531-8249(199901)45:1<75::aid-art13>3.0.co
更新日期:1999-01-01 00:00:00
abstract::The rate of local cerebral glucose utilization was determined for quantification of energy metabolism in macrostructures of the sensorimotor system during propagation of focal motor seizures in 24-month-old monkeys. The rate was measured in 4 control animals and in 4 monkeys each with seizures limited to the contralat...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410070304
更新日期:1980-03-01 00:00:00
abstract::Understanding of the organization and function of a newly identified neuronal messenger molecule, nitric oxide, has progressed rapidly. Nitric oxide synthase has been purified and molecularly cloned from brain. Its localization is exclusively neuronal and endothelial. The catalytic activity of nitric oxide synthase ac...
journal_title:Annals of neurology
pub_type: 杂志文章,评审
doi:10.1002/ana.410320302
更新日期:1992-09-01 00:00:00
abstract:OBJECTIVE:Guillain-Barré syndrome (GBS) is an acute postinfectious immune-mediated polyneuropathy. Although preceding respiratory tract infections with Mycoplasma pneumoniae have been reported in some cases, the role of M. pneumoniae in the pathogenesis of GBS remains unclear. We here cultured, for the first time, M. p...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24755
更新日期:2016-10-01 00:00:00
abstract:OBJECTIVE:Multiple sclerosis (MS) is a chronic neuroinflammatory and neurodegenerative disease of unknown etiology. Although the prevalent view regards a CD4+ -lymphocyte autoimmune reaction against myelin at the root of the disease, recent studies propose autoimmunity as a secondary reaction to idiopathic brain damage...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.26014
更新日期:2021-01-06 00:00:00
abstract::To characterize the protein composition of degenerating neurons in Alzheimer disease, enriched fractions of isolated cortical neurons from postmortem Alzheimer brain were compared by ultrastructural and biochemical techniques to neuronal isolates from aged normal controls and from patients with the nonfibrillary degen...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410080504
更新日期:1980-11-01 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:OBJECTIVE:The dose-response effects of dysferlin transgenesis were analyzed to determine if the dysferlin-deficient myopathies are good candidates for gene replacement therapy. METHODS:We have generated 3 lines of transgenic mice, expressing low, mid, and high levels of full-length human dysferlin from a muscle-specif...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.21926
更新日期:2010-03-01 00:00:00
abstract::It has been known for some time that patients with Parkinson's disease (PD) have difficulty executing simultaneous motor acts, yet the pathophysiology underlying this impairment remains uncertain. We examined the possibility that these dual-task deficits stem from defective sensorimotor mechanisms producing interferen...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410310110
更新日期:1992-01-01 00:00:00
abstract::In CAG repeat diseases, affected neurons possess many cytoplasmic granules immunopositive for expanded polyglutamine stretches. Electron microscopic immunohistochemistry showed that the granules corresponded to lysosomes of primitive type. The results suggest that, in addition to the ubiquitin/proteasome pathway, muta...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.10328
更新日期:2002-10-01 00:00:00
abstract::We demonstrate that the 3-hydroxy-3-methyl-glutaryl-coenzyme A (HMG-CoA) reductase inhibitors atorvastatin and simvastatin enhance functional outcome and induce brain plasticity when administered after stroke to rats. With atorvastatin treatment initiated 1 day after stroke, animals exhibited significant increases in ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.10555
更新日期:2003-06-01 00:00:00
abstract::beta-Dystroglycan, a 43-kd transmembrane dystrophin-associated glycoprotein, plays an important role in linking dystrophin to the laminin-binding alpha-dystroglycan. alpha-/beta-Dystroglycan is encoded by a single gene on chromosome 3p21 and ubiquitously expressed in muscle and nonmuscle tissues. No known human diseas...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410400617
更新日期:1996-12-01 00:00:00
abstract::The incidence of periventricular-intraventricular hemorrhage (PV-IVH) in a group of 460 preterm infants with birth weight less than 2,250 gm, studied by cranial ultrasonography, was 39%. Sixty-four (36%) of the infants with periventricular-intraventricular hemorrhage had, in addition, periventricular intraparenchymal ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410150315
更新日期:1984-03-01 00:00:00
abstract::The seemingly unpredictable response to levodopa in patients with Parkinson's disease can be understood as an interaction between several distinct pharmacological effects of levodopa. The most important are a short-duration response with a half-life of minutes to hours and a long-duration response with a half-life of ...
journal_title:Annals of neurology
pub_type: 杂志文章,评审
doi:10.1002/ana.410390504
更新日期:1996-05-01 00:00:00
abstract::We here report the second and third mutations in alphaB-crystallin causing myofibrillar myopathy. Two patients had adult-onset muscle weakness. Patient 1 had cervical, limb girdle, and respiratory muscle weakness and died of respiratory failure. Patient 2 had proximal and distal leg muscle weakness. Both had myopathic...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.10767
更新日期:2003-12-01 00:00:00
abstract::We report an immunohistochemical study of the mitochondrial alpha-ketoglutarate dehydrogenase complex (KGDHC) in the substantia nigra in Parkinson's disease. The KGDHC, the three enzyme complex catalyzing the oxidation of alpha-ketoglutarate to succinate through succinic semialdehyde, is the rate-regulating enzyme of ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410350212
更新日期:1994-02-01 00:00:00
abstract::Infection by the human immunodeficiency virus type 1 (HIV-1) often results in neurological dysfunction including HIV dementia (HIVD). Alterations in cytokine and redox balance are thought to play important roles in the pathogenesis of HIVD, but the specific mechanisms underlying neuronal dysfunction and death are unkn...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.10828
更新日期:2004-02-01 00:00:00
abstract::Alterations of nodal and paranodal axolemma of the rat sciatic nerve were investigated in antigalactocerebroside serum-induced demyelination. A ferric ion-ferrocyanide (FeFCN) stain that appears to stain the regions with a high sodium channel density in nerve fibers was applied. When acute conduction block was initiat...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410150611
更新日期:1984-06-01 00:00:00
abstract::Mitochondria are cellular organelles crucial for energy supply and calcium homeostasis in neuronal cells, and their dysfunction causes seizure activity in some rare human epilepsies. To directly test whether mitochondrial respiratory chain enzymes are abnormal in the most common form of chronic epilepsy, temporal lobe...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:
更新日期:2000-11-01 00:00:00
abstract::Chronic measles virus infection of the brain causes subacute sclerosing panencephalitis (SSPE), a progressive, relentless fatal disorder. We report a 52-year-old male who developed focal, chronic persistent measles virus infection of the brain following interferon and ribavirin therapy for hepatitis C, and who respond...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24183
更新日期:2014-06-01 00:00:00
abstract::We measured the activities of the enzymes responsible for the metabolism of the excitotoxin quinolinic acid, 3-hydroxyanthranilate oxygenase and quinolinic acid phosphoribosyltransferase, in autopsied brain of 11 patients with olivopontocerebellar atrophy. In cerebellar cortex, severe Purkinje cell loss was evident bu...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410290119
更新日期:1991-01-01 00:00:00
abstract::The cerebrovascular response of newborn animals to hypotension has not been defined on a regional basis. Using an autoradiographic technique employing 14C-iodoantipyrine as indicator, we studied the cerebral physiological responses of newborn dogs to hypotension induced by exsanguination or by administration of Escher...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410120506
更新日期:1982-11-01 00:00:00
abstract::A persistent vegetative state (severe dementia) developed in a 30-year-old man following hypoglycemic coma. Despite the poor clinical outcome, sensory evoked response recovered between 6 and 34 months after the insult. The cerebral blood flow level at rest after 34 months was slightly above the normal range. This find...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410140414
更新日期:1983-10-01 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::To study distribution and patterns of nerve hypertrophy in chronic inflammatory demyelinating polyneuropathy (CIDP), magnetic resonance neurography with 3-dimensional reconstruction of short tau inversion recovery images was performed in 33 patients. This technique clearly showed longitudinal morphological changes fro...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24314
更新日期:2015-02-01 00:00:00
abstract::An X-linked recessive disease with, in almost all patients, a fatal course in early childhood, occurring in a five-generation family is described. The 12 affected boys had early-onset floppiness, ataxia, liability to infections especially of the upper respiratory tract, deafness, and later, a flaccid tetraplegia and a...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410330519
更新日期:1993-05-01 00:00:00