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 the TCA cycle. The mitochondrial toxin, MPP+, inhibits not only complex I but also the KGDHC. Therefore, we investigated this enzyme complex in Parkinson's disease. In the control patients (n = 6), the immunostaining of the melanized nigral neurons was generally uniform; most of the melanized neurons showed good immunostaining with some neurons showing somewhat reduced staining. In Parkinson's disease (n = 9), many melanized neurons showed reduced immunostaining for the KGDHC, and those neurons were more frequently seen in the lateral one-third of substantia nigra. The decrease in the immunostaining for the KGDHC correlated roughly with the severity of degeneration. The KGDHC is more vulnerable to degeneration than complex II, III, and IV as noted in our previous immunohistochemical study. Even if secondary, the loss may play a role in the progression of the disease.
journal_name
Ann Neuroljournal_title
Annals of neurologyauthors
Mizuno Y,Matuda S,Yoshino H,Mori H,Hattori N,Ikebe Sdoi
10.1002/ana.410350212subject
Has Abstractpub_date
1994-02-01 00:00:00pages
204-10issue
2eissn
0364-5134issn
1531-8249journal_volume
35pub_type
杂志文章abstract:OBJECTIVE:Neuronal channelopathies cause brain disorders, including epilepsy, migraine, and ataxia. Despite the development of mouse models, pathophysiological mechanisms for these disorders remain uncertain. One particularly devastating channelopathy is Dravet syndrome (DS), a severe childhood epilepsy typically cause...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.23897
更新日期:2013-07-01 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 describe a French amyotrophic lateral sclerosis (ALS) family with two distinct mutations in the Cu/Zn superoxide dismutase (SOD1) gene. The D90A mutation has been well described and clearly shown to cause recessive ALS. In this family, affected individuals are heterozygous for the D90A mutation and also carry a sin...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/1531-8249(20010201)49:2<267::aid-ana51>3.0
更新日期:2001-02-01 00:00:00
abstract::To evaluate reports of abnormal levels of free amino acids (AA) in patients with amyotrophic lateral sclerosis (ALS), we studied serum, cerebrospinal fluid, and urine AA in 12 patients with ALS and 12 controls matched for age, sex, and severity of disability. ALS patients had statistically significant elevations in se...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410030405
更新日期:1978-04-01 00:00:00
abstract::Electroencephalographic (EEG) and evoked potential data were recorded during behavioral testing from 8 dyslexic and 10 normal boys aged 9 to 11 years. Topographic mapping of their brain electrical activity revealed four discrete regions of difference between the two groups involving both hemispheres, left more than ri...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410070505
更新日期:1980-05-01 00:00:00
abstract::B cells have long played an enigmatic role in the scenario of multiple sclerosis pathogenesis. This review summarizes recent progress in our understanding of B-cell trafficking, survival, and differentiation in the central nervous system (CNS). We propose four possible routes of intrathecal immunoglobulin-producing ce...
journal_title:Annals of neurology
pub_type: 杂志文章,评审
doi:10.1002/ana.20890
更新日期:2006-06-01 00:00:00
abstract::Neurotrophic factors have been demonstrated to prevent the development of peripheral neuropathy in animal models, but the therapeutic use of these factors in human disease has been limited by the short serum half-life and dose-limiting side effects of these potent peptides. We used peripheral subcutaneous inoculation ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.10061
更新日期:2002-01-01 00:00:00
abstract::If patients with left-sided hemispatial neglect bisect lines incorrectly because hemianopia or sensory hemiinattention prevents them from seeing how far the line extends to the left, a strategy that ensures their seeing the left side of the line in their normal field should improve performance. If patients have hemisp...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410050210
更新日期:1979-02-01 00:00:00
abstract::Several mutations in the amyloid precursor protein (APP) gene have been found to associate with pathologic deposition of the beta-amyloid peptide (Abeta) in neuritic plaques or in the walls of cerebral vessels. We report a mutation at a novel site in APP in a three-generation Iowa family with autosomal dominant dement...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.1009
更新日期:2001-06-01 00:00:00
abstract:OBJECTIVE:Narcolepsy is caused by the loss of hypocretin/orexin neurons in the hypothalamus, which is likely the result of an autoimmune process. Recently, concern has been raised over reports of narcolepsy in northern Europe following H1N1 vaccination. METHODS:The study is a retrospective analysis of narcolepsy onset...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.22587
更新日期:2011-09-01 00:00:00
abstract::The activities of monoacylglycerol and diacylglycerol lipases were 6 to 8 times higher in plasma membrane and synaptosomal plasma membrane fractions of nucleus basalis from patients with Alzheimer's disease than in those membranes from normal human brains. Membranes from the hippocampus region of Alzheimer-affected br...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410230317
更新日期:1988-03-01 00:00:00
abstract:OBJECTIVE:Abnormal cortical excitability is evident in various movement disorders that compromise fine motor control. Here we tested whether skilled finger movements can be restored in musicians with focal hand dystonia through behavioral training assisted by transcranial direct current stimulation to the motor cortex ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24151
更新日期:2014-05-01 00:00:00
abstract::The Pilot Stroke Data Bank obtained information on 94 patients with intracerebral hemorrhage. These data were used to identify factors predictive of 30-day outcome from among 85 demographic, historical, clinical, and laboratory variables generally available to clinicians on the day of admission. The 9 univariate facto...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410240213
更新日期:1988-08-01 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::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:OBJECTIVE:To examine changes in the response properties of meningeal nociceptors that might lead to migraine pain and examine endogenous processes that could play a role in mediating them using a clinically relevant model of migraine triggering, namely infusion of the nitric oxide (NO) donor nitroglycerin (NTG). METHO...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.23873
更新日期:2013-06-01 00:00:00
abstract::The developmental sequence of sleep cycles has been found to be a useful index of central nervous system maturation in premature infants. To determine the effects of severe reversible stress on the maturation of sleep cycles, 6- to 8-hour sleep studies were done on 10 premature infants with severe hyaline membrane dis...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410060610
更新日期:1979-12-01 00:00:00
abstract::Oxidative damage to DNA may play a role in both normal aging and in neurodegenerative diseases. We examined whether Alzheimer's disease (AD) is associated with increased oxidative damage to nDNA and mtDNA in postmortem brain tissue. We measured the oxidized nucleoside, 8-hydroxy-2'-deoxyguanosine (OH8dG), in DNA isola...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410360510
更新日期:1994-11-01 00:00:00
abstract::Although interictal spikes are thought to share pathophysiological mechanisms with partial-onset seizure discharges, positron emission tomographic studies of the interictal state have paradoxically shown focal hypometabolism whereas seizures produce hypermetabolism. To address this question, we performed functional ma...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410350614
更新日期:1994-06-01 00:00:00
abstract::A patient with generalized convulsions noted that seizures were reliably precipitated by mental arithmetic. The interictal electroencephalogram revealed only a mild, diffuse, nonspecific disturbance, but bursts of generalized epileptiform activity with no obvious clinical expression accompanied efforts at mental arith...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410110609
更新日期:1982-06-01 00:00:00
abstract::A patient is described with the dermatological features of blue rubber bleb nevus syndrome (BRBNS), focal seizures, and lateralized neurological signs. CAT scan demonstrated a nonenhanced density in the region of the vein of Galen. Neuropathological examination showed that this density was a clot within a vein of Gale...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410030522
更新日期:1978-05-01 00:00:00
abstract:OBJECTIVE:To assess the ability for visual search and recognition of roadside targets and safety errors during a landmark and traffic sign identification task in drivers with Parkinson's disease (PD). METHODS:Seventy-nine drivers with PD and 151 neurologically normal older adults underwent a battery of visual, cogniti...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.20958
更新日期:2006-10-01 00:00:00
abstract::We measured neurotransmitter markers in autopsied brain of infants with glycine encephalopathy (GE). Because patients with GE develop intractable seizures, special attention was devoted to those neurotransmitter systems implicated in human epilepsy. Mean levels of glycine in the frontal cortex of GE patients were thre...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410240319
更新日期:1988-09-01 00:00:00
abstract::Two brothers with profound neonatal hypotonia and hyporeflexia and electrodiagnostic testing consistent with lower motor neuron pathology were found to have a leukodystrophy. Using single-strand conformational polymorphism analysis and direct sequencing, a mutation within exon 3 of the gene encoding proteolipid protei...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410360618
更新日期:1994-12-01 00:00:00
abstract::A recent observation has shown that a common polymorphism in the alpha1-antichymotrypsin (ACT) gene modifies the apolipoprotein E (ApoE) epsilon4-associated Alzheimer's disease (AD) risk identifying the combination of the ACT/AA and ApoE epsilon4/epsilon4 genotypes as a potential susceptibility marker for AD. We analy...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410400420
更新日期:1996-10-01 00:00:00
abstract:OBJECTIVE:Cortical spreading depolarizations (CSDs) are intense and ubiquitous depolarization waves relevant for the pathophysiology of migraine and brain injury. CSDs disrupt the blood-brain barrier (BBB), but the mechanisms are unknown. METHODS:A total of six CSDs were evoked over 1 hour by topical application of 30...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.25298
更新日期:2018-09-01 00:00:00
abstract::Recent studies reported the presence of anti-ganglioside antibodies in occasional patients with motor neuron disease. We found polyclonal serum IgM anti-GM1 antibodies by an anti-GM1 enzyme-linked immunosorbent assay (ELISA) in 9 (19%) of 48 patients with motor neuron disease. A comparable frequency of IgM anti-GM1 an...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410270517
更新日期:1990-05-01 00:00:00
abstract:OBJECTIVE:The prevalence of mitochondrial disease has proven difficult to establish, predominantly as a result of clinical and genetic heterogeneity. The phenotypic spectrum of mitochondrial disease has expanded significantly since the original reports that associated classic clinical syndromes with mitochondrial DNA (...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24362
更新日期:2015-05-01 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::Regular, evidence-based assignment of patients to etiologic stroke categories is essential to enable valid comparison among studies. We designed an algorithm (SSS-TOAST) that incorporated recent advances in stroke imaging and epidemiology to identify the most probable TOAST category in the presence of evidence for mul...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.20617
更新日期:2005-11-01 00:00:00