Alteration of Striatal Dopaminergic Neurotransmission in a Mouse Model of DYT11 Myoclonus-Dystonia
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منابع مشابه
Alteration of Striatal Dopaminergic Neurotransmission in a Mouse Model of DYT11 Myoclonus-Dystonia
BACKGROUND DYT11 myoclonus-dystonia (M-D) syndrome is a neurological movement disorder characterized by myoclonic jerks and dystonic postures or movement that can be alleviated by alcohol. It is caused by mutations in SGCE encoding ε-sarcoglycan (ε-SG); the mouse homolog of this gene is Sgce. Paternally-inherited Sgce heterozygous knockout (Sgce KO) mice exhibit myoclonus, motor impairment and ...
متن کاملAbnormal nuclear envelopes in the striatum and motor deficits in DYT11 myoclonus-dystonia mouse models.
DYT11 myoclonus-dystonia (M-D) is a movement disorder characterized by myoclonic jerks with dystonic symptoms and caused by mutations in paternally expressed SGCE, which codes for ε-sarcoglycan. Paternally inherited Sgce heterozygous knock-out (KO) mice exhibit motor deficits and spontaneous myoclonus. Abnormal nuclear envelopes have been reported in cellular and mouse models of early-onset DYT...
متن کاملReduction of striatal dopaminergic neurotransmission elevates striatal proenkephalin mRNA.
Dopamine-containing neurons have been shown to make direct contact with enkephalin-like immunoreactive neurons in the striatum of rat brain (Kubota et al., 1986). Chronic blockade of dopaminergic neurotransmission with the neuroleptic drug haloperidol has been reported to increase the striatal concentration of methionineenkephalin and proenkephalin m R N A (ENK mRNA) (Tang et al., 1983). We inv...
متن کاملDeep brain stimulation for myoclonus-dystonia syndrome with double mutations in DYT1 and DYT11
Myoclonus-dystonia syndrome (MDS) is a rare autosomal dominant inherited disorder characterized by the presentation of both myoclonic jerks and dystonia. Evidence is emerging that deep brain stimulation (DBS) may be a promising treatment for MDS. However, there are no studies reporting the effects of DBS on MDS with double mutations in DYT1 and DYT11. Two refractory MDS patients with double mut...
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deployable scissor type structures are composed of the so-called scissor-like elements (sles), which are connected to each other at an intermediate point through a pivotal connection and allow them to be folded into a compact bundle for storage or transport. several sles are connected to each other in order to form units with regular polygonal plan views. the sides and radii of the polygons are...
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ژورنال
عنوان ژورنال: PLoS ONE
سال: 2012
ISSN: 1932-6203
DOI: 10.1371/journal.pone.0033669