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cystathionine/eпилептички напад
Веза се чува у привремену меморију
Страна 1 од 30 резултати
[Gamma-aminobutyric acid B receptor regulates the expression of hydrogen sulfide/cystathionine-beta-synthase system in recurrent febrile seizures].
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OBJECTIVE
Febrile seizure (FS) is one of the most common seizure types in children. Our previous studies have demonstrated that both gamma-aminobutyric acid B receptor (GABABR) and hydrogen sulfide (H2S) are involved in the pathogenesis of FS. This study was designed to explore the effect of GABABR
[Alteration of hydrogen sulfide/cystathionine-beta-synthase system in rats with recurrent febrile seizures].
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OBJECTIVE
To study the alteration of hydrogen sulfide (H(2)S)/ cystathionine-beta-synthase (CBS) system during recurrent febrile seizures (FS) in the hippocampus of developing rats.
METHODS
The rats were randomly divided into control group (n=8) and hyperthermia-treated group (n=22). Which was
Vitamin B6-dependent seizures: pathology and chemical findings in brain.
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A 13 1/2-year-old child died with vitamin B6-dependent seizures in progress. Microscopic findings in brain included an abnormally sparse quantity of central myelinated fibers in the cerebral hemispheres. Glutamic acid concentrations were elevated and GABA concentrations reduced in the frontal and
Seizure evolution and amino acid imbalances in murine succinate semialdehyde dehydrogenase (SSADH) deficiency.
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Mice with targeted deletion of the GABA catabolic enzyme succinic semialdehyde dehydrogenase (SSADH) manifest lethal tonic-clonic seizures, amenable to pharmacologic rescue, at 3-4 weeks of life. In the current report, we characterized amino acid profiles in SSADH(-/-) brain utilizing whole brain
Hydrogen sulfide may improve the hippocampal damage induced by recurrent febrile seizures in rats.
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The aim of the present study was to investigate the possible role of hydrogen sulfide (H(2)S) in the pathogenesis of recurrent febrile seizures (FS) in rats. On a rat model of recurrent FS, the ultrastructure of hippocampal neurons, the plasma level of H(2)S, the expressions of cystathionine
[Cystathionine betasynthase and MTHFR deficiencies in adults].
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BACKGROUND
Homocysteine lies at an important metabolic branch point; it may be either converted to cystathionine through the transsulfuration pathway, or methylated to form methionine. Hyperhomocysteinemia may result from hereditary defects affecting one of these reactions.
BACKGROUND
Cystathionine
Amino acid metabolism in the brain with convulsive disorders. Part I: Free amino acid patterns in the brain of E1 mouse with convulsive seizure.
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To clarify the biochemical mechanism of convulsions from a view point of the amino acid metabolism, the free amino acid patterns in brains of El mice were investigated. The free amino acid levels in the brain excluding the cerebellum were measured by an amino acid autoanalyzer. 1) In the convulsion
Electroencephalographic abnormalities in homocystinuria due to cystathionine synthase deficiency.
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Nineteen homocystinuric patients--13 children and 6 adults--were identified in the course of a selective screening for homocystinuria due to cystathionine synthase deficiency. Treatment with high doses (300-1200 mg/day) of pyridoxine was carried out in 17 patients, of whom 15 were completely
Hydrogen sulfide and carbon monoxide are in synergy with each other in the pathogenesis of recurrent febrile seizures.
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1. The purpose of the present study was to investigate the interaction between hydrogen sulfide (H(2)S) and carbon monoxide (CO) during recurrent febrile seizures (FS) 2.H(2)S and CO are important intra- and intercellular messengers, regulating various brain functions. Our recent studies showed that
Modulating effect of hydrogen sulfide on gamma-aminobutyric acid B receptor in recurrent febrile seizures in rats.
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Hydrogen sulfide (H2S) is recognized as a new neuromodulator in regulating various brain functions. Some of our recent studies showed that H2S alleviates the hippocampal damage induced by recurrent febrile seizures (FS). In the present study, we used a rat model of recurrent FS and found that sodium
Cystathionine beta-synthase, a key enzyme for homocysteine metabolism, is preferentially expressed in the radial glia/astrocyte lineage of developing mouse CNS.
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Cystathionine beta-synthase (CBS; EC 4.2.1.22) is a key enzyme in the generation of cysteine from methionine. A deficiency of CBS leads to homocystinuria, an inherited human disease characterized by mental retardation, seizures, psychiatric disturbances, skeletal abnormalities, and vascular
Psychiatric manifestations of homocystinuria due to cystathionine beta-synthase deficiency: prevalence, natural history, and relationship to neurologic impairment and vitamin B6-responsiveness.
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Homocystinuria commonly affects the central nervous system (CNS), primarily as mental retardation, seizures, and stroke. Case reports have long suggested a predisposition to schizophrenia, but no careful study of predisposition to psychiatric illness has been performed. Accordingly, we evaluated 63
The natural history of homocystinuria due to cystathionine beta-synthase deficiency.
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An international questionnaire survey has been conducted to define better the natural history of homocystinuria due to cystathionine beta-synthase deficiency and permit evaluation of treatment. Data were compiled for 629 patients. Among patients not discovered by newborn screening, B6-responsive
Amino acid metabolism in the brain with convulsive disorders. Part 2: The effects of anticonvulsants on convulsions and free amino acid patterns in the brain of El mouse.
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To elucidate the changes of free amino acid patterns in brains of El mice induced by several anticonvulsants, the free amino acid levels in brains were measured by an amino acid autoanalyzer 24 hours after intraperitoneal injection of PB, PHT or vitamin B6 or subcutaneous injection of ACTH. Compared
Homocystinuria in Thai patient--Phramongkutklao Hospital experience.
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Homocystinuria is a rare autosomal recessive disorder of amino acid metabolism. Classic (type I) homocystinuria is the most common type and occurs as a consequence of a deficiency of cystathionine-b-synthase, producing increased blood and urine homocysteine. The authors report a 15-year-old Thai
Најкомплетнија база лековитог биља подржана науком
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