دورية أكاديمية
Cell-autonomous alteration of dopaminergic transmission by wild type and mutant (ΔE) TorsinA in transgenic mice
| العنوان: | Cell-autonomous alteration of dopaminergic transmission by wild type and mutant (ΔE) TorsinA in transgenic mice |
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| المؤلفون: | Michelle E. Page, Li Bao, Pierrette Andre, Joshua Pelta-Heller, Emily Sluzas, Pedro Gonzalez-Alegre, Alexey Bogush, Loren E. Khan, Lorraine Iacovitti, Margaret E. Rice, Michelle E. Ehrlich |
| المصدر: | Neurobiology of Disease, Vol 39, Iss 3, Pp 318-326 (2010) |
| بيانات النشر: | Elsevier, 2010. |
| سنة النشر: | 2010 |
| المجموعة: | LCC:Neurosciences. Biological psychiatry. Neuropsychiatry |
| مصطلحات موضوعية: | Dystonia, Tyrosine hydroxylase, Dopamine, DYT1, TorsinA, Striatum, Neurosciences. Biological psychiatry. Neuropsychiatry, RC321-571 |
| الوصف: | Early onset torsion dystonia is an autosomal dominant movement disorder of variable penetrance caused by a glutamic acid, i.e. ΔE, deletion in DYT1, encoding the protein TorsinA. Genetic and structural data implicate basal ganglia dysfunction in dystonia. TorsinA, however, is diffusely expressed, and therefore the primary source of dysfunction may be obscured in pan-neuronal transgenic mouse models. We utilized the tyrosine hydroxylase (TH) promoter to direct transgene expression specifically to dopaminergic neurons of the midbrain to identify cell-autonomous abnormalities. Expression of both the human wild type (hTorsinA) and mutant (ΔE-hTorsinA) protein resulted in alterations of dopamine release as detected by microdialysis and fast cycle voltammetry. Motor abnormalities detected in these mice mimicked those noted in transgenic mice with pan-neuronal transgene expression. The locomotor response to cocaine in both TH-hTorsinA and TH-ΔE-hTorsinA, in the face of abnormal extracellular DA levels relative to non-transgenic mice, suggests compensatory, post-synaptic alterations in striatal DA transmission. This is the first cell-subtype-specific DYT1 transgenic mouse that can serve to differentiate between primary and secondary changes in dystonia, thereby helping to target disease therapies. |
| نوع الوثيقة: | article |
| وصف الملف: | electronic resource |
| اللغة: | English |
| تدمد: | 1095-953X 73567779 |
| Relation: | http://www.sciencedirect.com/science/article/pii/S0969996110001324; https://doaj.org/toc/1095-953X |
| DOI: | 10.1016/j.nbd.2010.04.016 |
| URL الوصول: | https://doaj.org/article/4816615fc72d4c10bd0cda7356777982 |
| رقم الأكسشن: | edsdoj.4816615fc72d4c10bd0cda7356777982 |
| قاعدة البيانات: | Directory of Open Access Journals |
Full Text via ClinicalKey 
Full Text Finder | تدمد: | 1095953X 73567779 |
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| DOI: | 10.1016/j.nbd.2010.04.016 |