Abstract：
:Phenylketonuria (PKU) is a recessive autosomal disorder caused by a severe deficiency of phenylalanine-4-hydroxilase activity which leads to the accumulation of L-phenylalanine (Phe) in the tissues and plasma of patients. The main clinical features are retarded development and intellectual impairment. Recent studies have shown that oxidative stress may be involved in neuropathology of hyperphenylalaninemia. Lipoic acid (LA) is considered a potent antioxidant which is well absorbed from diet and can easily cross the blood-brain barrier. We investigated the neuroprotective effects of lipoic acid against oxidative stress caused by Phe in vivo and in vitro. Lipoic acid prevented the inhibition provoked by Phe on the activities of catalase, superoxide dismutase, glutathione peroxidase, and glucose-6-phosphate dehydrogenase. It also prevented Phe alterations on total radical-trapping antioxidant potential, thiobarbituric acid-reactive substances, glutathione concentration and on production of reactive species. It is concluded that lipoic acid may be an efficient antioxidant in the CNS against oxidative stress induced by hyperphenylalaninemia. If the present results are confirmed in PKU patients, it is possible that supplementation of lipoic acid may contribute to the treatment of PKU as an adjuvant therapeutic approach to Phe-restricted dietary treatment and amino acid mixture.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Moraes TB,Zanin F,da Rosa A,de Oliveira A,Coelho J,Petrillo F,Wajner M,Dutra-Filho CSdoi
10.1016/j.jns.2010.01.016subject
Has Abstractpub_date
2010-05-15 00:00:00pages
89-95issue
1-2eissn
0022-510Xissn
1878-5883pii
S0022-510X(10)00043-2journal_volume
292pub_type
杂志文章abstract：:Subacute sclerosing panencephalitis (SSPE) is a rare infectious central nervous system disease with a poor prognosis. Nineteen patients, 18 males and one female, ranging in age from 18 to 22, mean 19.6+/-1.5 years with SSPE were evaluated. We treated 9 patients with oral isoprinosine and 10 patients with alpha-interfe...
journal_title：Journal of the neurological sciences
pub_type： 临床试验,杂志文章
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abstract：BACKGROUND:Direct oral anticoagulants (DOACs) can reduce the frequency of cardioembolic stroke with non-valvular atrial fibrillation as well as or better compared to vitamin K antagonists (VKAs). However, whether taking DOACs prior to stroke can prevent acute major cerebral artery occlusion (MCAO) has not been fully el...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2019.05.023
更新日期：2019-07-15 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(93)90129-m
更新日期：1993-11-01 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(88)90178-5
更新日期：1988-03-01 00:00:00
abstract：:Corticosteroids (Cs) are widely used for treatment of multiple sclerosis (MS) acute relapses because of the potent immunosuppressive and anti-inflammatory properties. As for patients with relapsing-remitting (RR) MS, short-term administrations of Cs markedly less severity of symptoms and promote faster recovery of cli...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2004.04.019
更新日期：2004-08-15 00:00:00
abstract：:Subacute sclerosing panencephalitis is characterized by the insidious onset of diffuse cerebral dysfunction associated later with myoclonus and typical electroencephalographic changes. The disease progresses relentlessly to coma and death within 2 years. We report a case of acute onset and rapid course associated with...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/s0022-510x(96)00261-4
更新日期：1997-02-27 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2012.11.013
更新日期：2013-02-15 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2016.09.025
更新日期：2016-11-15 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
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更新日期：2017-03-15 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2010.10.016
更新日期：2011-02-15 00:00:00
abstract：:Parkinson's disease (PD) is a common disorder of the central nervous system in the elderly. The pathogenesis of PD is a complex process, with genetics as an important contributing factor. This factor may stem from mitochondrial gene variations and mutations as well as from nuclear gene variations and mutations. More r...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
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更新日期：2014-11-15 00:00:00
abstract：:Noninvasive ventilatory assistance, in ALS patients, with the bilevel intermittent positive air pressure (Bipap) was studied, in a prospective and controlled trial, by the authors. Twenty ALS bulbar patients, fulfilling El Escorial criteria for probable or definite disease, were selected. For the follow-up all patient...
journal_title：Journal of the neurological sciences
pub_type： 临床试验,杂志文章,随机对照试验
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更新日期：1995-05-01 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2008.08.029
更新日期：2009-01-15 00:00:00
abstract：:The case of a patient with focal epilepsy is reported who underwent presurgical evaluation by stereotactic intracranial electroencephalographic (EEG) recording. A subdural semi-grid electrode, consisting of three multi-channel strip electrodes, was implanted over the temporal lobe and temporo-occipital region; one mul...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2009.12.003
更新日期：2010-03-15 00:00:00
abstract：:A single micro-injection of Tumour Necrosis Factor alpha (TNF) or gamma Interferon (IFN-gamma) into the lumbosacral spinal cord of the rat produced meningitis and mononuclear cuffs within the cord, an inflammatory response remarkably similar in pattern to that observed during experimental autoimmune encephalomyelitis ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(90)90010-k
更新日期：1990-12-01 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2012.08.005
更新日期：2012-12-15 00:00:00
abstract：:Glucose-regulated protein (GRP) 94 is a member of the stress protein family, which is localized in the endoplasmic reticulum (ER). Spinal cord injury (SCI) induced ER stress that results in apoptosis. However, the role of GRP94 in injury of the central nervous system remains unknown. In this study, we performed SCI in...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2011.06.024
更新日期：2011-10-15 00:00:00
abstract：:Synaptosomes and plasma membrane preparations from brain of 30-day-old rats were incubated with glutaric acid at final concentrations ranging from 10 nM to 1 mM for the determination of glutamate uptake and binding, respectively. [3H]Glutamate uptake into synaptosomes was inhibited by approximately 50% by 1 mM glutari...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(99)00307-x
更新日期：2000-02-15 00:00:00
abstract：:Baló's concentric sclerosis (BCS) and tumefactive demyelination (TD) are considered atypical forms of multiple sclerosis (MS). Baló lesions are characterized by concentric rings corresponding to alternating bands of demyelination and relatively preserved myelin (Hu and Lucchinetti, 2009). Tumefactive lesions are pseud...
journal_title：Journal of the neurological sciences
pub_type： 信件
doi：10.1016/j.jns.2014.11.024
更新日期：2015-01-15 00:00:00
abstract：:Neuropsychological impairment is a common manifestation in multiple sclerosis (MS) and is found in 40-60% of patients. The pattern of cognitive impairment in MS is characterized by difficulties in recent memory, sustained attention, executive functions and information processing speed. These cognitive deficits have a ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2005.07.021
更新日期：2006-06-15 00:00:00
abstract：BACKGROUND:Previous studies to estimate burden of neurological disorders in Africa are limited to inpatients in urban hospitals. The spectrum of neurological conditions in rural Africa remains unclear. OBJECTIVE:To determine the spectrum of neurological presentations in an outpatient setting in rural Zimbabwe. METHOD...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2016.01.065
更新日期：2016-03-15 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(85)90039-5
更新日期：1985-11-01 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(85)90018-8
更新日期：1985-01-01 00:00:00
abstract：:A 66-year-old man presented with right cerebellar infarction and ischemic lesions in the left dorsal thalamus and right upper parietal lobe. Angiography showed occlusion of the right internal carotid artery proximal to an ipsilateral proatlantal artery type I, 70% stenosis of the left internal carotid artery, and apla...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/0022-510x(93)90178-2
更新日期：1993-07-01 00:00:00
abstract：BACKGROUND:Cerebrovascular reactivity (CVR) is defined as the change in cerebral blood flow, or blood velocity, in response to a vasoactive stimulus. There is a possible association between impaired CVR and vasospasm after aneurysmal subarachnoid hemorrhage. Most studies on CVR and vasospasm have used healthy subjects ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2015.12.024
更新日期：2016-04-15 00:00:00
abstract：INTRODUCTION:Whether motor neuron diseases (MNDs) can be considered in some cases of paraneoplastic syndromes is controversial. We report a case of rapidly progressive motor neuronopathy following a diagnosis of breast carcinoma, with a presence of anti-Ri antibodies, and a novel SOD1 gene mutation. OBSERVATION:An 80-...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2013.11.025
更新日期：2014-02-15 00:00:00
abstract：:The outcome of outpatient neurologic consultations has not been previously evaluated. There is also no data regarding patients' perceptions of outpatient neurologic consultations. In this study, we assessed the physician's evaluations of the outcome and utility of neurologic consultations by reviewing office records o...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2008.09.036
更新日期：2009-01-15 00:00:00
abstract：:The present study was performed to investigate the effects of Ca(2+)/calmodulin-dependent protein kinase II (CaMKII) antisense oligodeoxynucleotides (ODNs) on the assembly of the CaMKII·GluR6·PSD-95 signaling module, GluR6 serine phosphorylation and c-Jun N-terminal kinase 3 (JNK3) activation. A further aim was to det...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2011.10.012
更新日期：2012-03-15 00:00:00
abstract：:This is a case report of PCR proven herpes simplex (HSV-1) encephalitis in a 26 years old immunocompetent adult taking an unusual course of acute intracerebral haematoma after successful and complete recovery with acyclovir therapy. This transient late complication was associated with a negative repeat CSF PCR for HSV...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2006.10.020
更新日期：2007-01-31 00:00:00
abstract：:For over a decade, four immunomodulatory therapies have been available for the treatment of relapsing remitting multiple sclerosis. However, few direct comparative data were available to facilitate the choice of treatment. This choice has been influenced by the perception that interferon-beta preparations have greater...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/S0022-510X(09)70007-3
更新日期：2009-02-01 00:00:00
