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basal ganglia diseases/tyrosine
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14 resultados
Dopamine D1 activation potentiates striatal NMDA receptors by tyrosine phosphorylation-dependent subunit trafficking.
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Interactions between dopaminergic and glutamatergic afferents in the striatum are essential for motor learning and the regulation of movement. An important mechanism for these interactions is the ability of dopamine, through D1 receptors, to potentiate NMDA glutamate receptor function. Here we show
Reversal of dopamine neurons and locomotor ability degeneration in aged rats with smilagenin.
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The purpose of this paper is to study the effect of smilagenin (SMI) (PYM50028), a sapogenin compound originally identified from Chinese medicinal herb, on dopamine neurons and locomotor ability in aged rats. Experiments were carried out on young and aged Sprague-Dawley rats, which were daily
[Japanese originality, clinical symptoms to the causative gene 1 Segawa disease].
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Segawa disease was first reported in 1971 as 'Hereditary progressive basal ganglia disease with marked diurnal fluctuation'. In 1976, after experience of a 51 year old patient with 43 years non-treatment periods, I confirmed this disease as dystonia. Polysomnographies revealed selective involvement
Structure--activity relationships of n-substituted dopamine and 2-amino-6,7-dihydroxy-1,2,3,4-tetrahydronaphthalene analogues: behavioral effects in lesioned and reserpinized mice.
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N,N-Disubstituted dopamine and 2-amino-6,7-dihydroxy-1,2,3,4-tetrahydronaphthalene (6,7-ADTN) analogues were synthesized and tested intraperitoneally in mice for dopamine agonism. Compounds inducing asymmetric postures in unilaterally caudectomized mice were further tested in mice treated with
6R-Tetrahydrobiopterin induces dopamine synthesis in a human neuroblastoma cell line, LA-N-1. A cellular model of DOPA-responsive dystonia.
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Dopa-responsive dystonia (DRD) is an extrapyramidal disorder caused by deficit of 5,6,7,8-tetrahydrobiopterin (BH4), cofactor for tyrosine hydroxylase (TH). In these patients the nigrostriatal dopaminergic neurons normally express TH and the cellular machinery for the dopamine uptake. LA-N-1 is a
Partial dopamine loss enhances activated caspase-3 activity: differential outcomes in striatal projection systems.
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Parkinson's disease (PD) is a basal ganglia disorder. Motor symptoms develop insidiously following substantial neurodegeneration of the dopamine (DA) neurons in the nigrostriatal system and produce slowed, infrequent movements, postural instability, and gait changes. A thorough understanding of
Progressive supranuclear palsy: clinico-pathological and biochemical studies.
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Ten autopsy cases of Progressive Supranuclear Palsy (PSP) are reported. Age at onset ranged from 16 to 67 years and the duration of illness 3 to 24 years. The clinical features were aggressive mental retardation in 4 cases with early onset, paroxysmal dysequilibrium, ophthalmoplegia, rigidity and
[Segawa disease].
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Segawa disease (hereditary progressive dystonia with marked diurnal fluctuation) is an autosomal dominant, childhood onset, postural dystonia and the first hereditary basal ganglia disorder whose causative enzyme and gene defect were clarified. The initial symptom is unilateral pes equinovarus with
Pteridines and mono-amines: relevance to neurological damage.
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Patients with phenylalanine hydroxylase deficiency show increased concentrations of biopterins and neopterins, and reduced concentrations of serotonin and catecholamines, when phenylalanine concentrations are raised. The pterin rise reflects increased synthesis of dihydroneopterin and
Up-regulation of trkB mRNA expression in the rat striatum after seizures.
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The present study investigates the expression of a tyrosine kinase receptor (trkB), its specific ligands brain-derived neurotrophic factor (BDNF) and neurotrophin-4 (NT-4) mRNAs in the striatum after seizures. The result showed an increase of trkB mRNA expression, both with and without tyrosine
[Segawa disease].
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The first report of Segawa disease was a report of two girls, cousin each other, with dystonic posture, under the title of "Hereditary progressive basal ganglia disorder" in 1971. After accumulation of cases with an adult case, I confirmed this disease does not transform to Parkinson's disease in
The pedunculopontine nucleus in developmental disorders of the basal ganglia.
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The pedunculopontine nucleus (PPN), which is located in the upper brainstem, contains cholinergic and non-cholinergic neurons, and has afferent and efferent connections to the basal ganglia and spinal cord. The PPN is known to be affected in adult-onset basal ganglia diseases, and we speculated that
Dystonia-plus syndromes.
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Dystonia-plus syndromes represent a heterogeneous group of diseases, where dystonia is accompanied by other neurological features and gene mutations can be detected frequently. Symptomatic dystonias and complex neurodegenerative diseases with dystonia as part of the clinical presentation are
Manganese alters rat brain amino acids levels.
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Manganese (Mn) is an essential element and it acts as a cofactor for a number of enzymatic reactions, including those involved in amino acid, lipid, protein, and carbohydrate metabolism. Excessive exposure to Mn can lead to poisoning, characterized by psychiatric disturbances and an extrapyramidal
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