2008
DOI: 10.1073/pnas.0806065105
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Differential involvement of striosome and matrix dopamine systems in a transgenic model of dopa-responsive dystonia

Abstract: Dopa-responsive dystonia (DRD) is a hereditary dystonia character-ized by a childhood onset of fixed dystonic posture with a dramatic and sustained response to relatively low doses of levodopa. DRD is thought to result from striatal dopamine deficiency due to a reduced synthesis and activity of tyrosine hydroxylase (TH), the synthetic enzyme for dopamine. The mechanisms underlying the genesis of dystonia in DRD present a challenge to models of basal ganglia movement control, given that striatal dopamine defici… Show more

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Cited by 88 publications
(82 citation statements)
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“…Like the changes seen in D 1 -and D 2 -like autoradiography, the loss of μ-opioid receptors was more pronounced in the caudal versus the rostral striatum. Exaggerated striatal caudal pathology is noted in Parkinson's disease (44 -46), in a primate disease model of Parkinson's disease (47), and in both the human form of dopamine responsive dystonia and in a mouse model of this condition (25). Taken together, the findings provide support for the idea that imbalance of basal ganglia direct and indirect pathway signaling produced by a regionally regulated loss of D 1 -expressing cells, loss of striatal μ-opioid receptors, and up-regulation of D 2 -cells may underpin the acute behavioural response to morphine seen in this model.…”
Section: Discussionmentioning
confidence: 99%
“…Like the changes seen in D 1 -and D 2 -like autoradiography, the loss of μ-opioid receptors was more pronounced in the caudal versus the rostral striatum. Exaggerated striatal caudal pathology is noted in Parkinson's disease (44 -46), in a primate disease model of Parkinson's disease (47), and in both the human form of dopamine responsive dystonia and in a mouse model of this condition (25). Taken together, the findings provide support for the idea that imbalance of basal ganglia direct and indirect pathway signaling produced by a regionally regulated loss of D 1 -expressing cells, loss of striatal μ-opioid receptors, and up-regulation of D 2 -cells may underpin the acute behavioural response to morphine seen in this model.…”
Section: Discussionmentioning
confidence: 99%
“…Because hind-limb clasping was also observed in DPSPts Ϫ/Ϫ mice, we reanalyzed the duration of clasping posture in DPS-Pts Ϫ/Ϫ mice from previous study (16) and compared with that in Spr Ϫ/Ϫ mice. The duration of the clasping posture for the DPS-Pts Ϫ/Ϫ mice was 19.66 Ϯ 0.09 s during 25 s, which was significantly longer than the duration for the Spr Ϫ/Ϫ mice shown in Fig.…”
Section: Phenylalanine and Tyrosine Levels In The Brain Of Biopterindmentioning
confidence: 99%
“…The dysfunction of striatal matrix neurons is especially severe for the indirect pathway neurons of the striatum, which in the sensorimotor striatum receive powerful inputs from the motor cortex (4)(5)(6). In different models of parkinsonian states, the gradients and compartmental expression of dopaminergic deficits vary (7)(8)(9), but it seems likely that dysfunctional dopaminergic modulation of corticostriatal motor pathways leading through the matrix compartment is a major contributor to the motor symptoms of Parkinson's disease, and that in a range of movement disorders, compartmentally selective striatal dysfunction is present (10)(11)(12)(13)(14)(15)(16)(17).…”
mentioning
confidence: 99%