کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
4341992 | 1295852 | 2008 | 11 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Dopamine D1 receptor-mediated aggregation of N-terminal fragments of mutant huntingtin and cell death in a neuroblastoma cell line
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کلمات کلیدی
pKaCREBDMEMPICNGSeGFPGAPDHcAMP - cAMPG-protein-coupled receptors - G-پروتئین گیرندهDulbecco's modified Eagle's medium - Medal of Eagle اصلاح شده DulbeccoMTT - MTTCell survival - بقای سلولیHuntington's disease - بیماری هانتینگتونRoom temperature - دمای اتاقnormal goat serum - سرم طبیعی بزenhanced green fluorescent protein - پروتئین فلورسنت سبز افزایش یافته استProtein kinase - پروتئین کینازprotease inhibitor cocktail - کوکتل مهار کننده پروتئازglyceraldehyde-3-phosphate dehydrogenase - گلیسرالیدید-3-فسفات دهیدروژنازDopamine receptors - گیرنده های دوپامین
موضوعات مرتبط
علوم زیستی و بیوفناوری
علم عصب شناسی
علوم اعصاب (عمومی)
پیش نمایش صفحه اول مقاله
چکیده انگلیسی
Huntington's disease (HD) is an inherited neurodegenerative disorder caused by abnormal CAG repeat expansion in the IT15 gene encoding huntingtin protein (htt). Mutated htt is predicted to acquire toxic properties in specific brain regions. For instance, striatal neurons expressing dopamine receptors predominantly degenerate in HD patients. Although the basis of this specific vulnerability remains unclear, a great deal of evidence has documented the ability of the dopamine system to modulate the toxicity of expanded htt. To investigate the relationship between dopamine receptors and expanded htt, we transfected enhanced green fluorescent proteins (EGFP) tagged to normal (25 CAG) or mutant (103 CAG) htt in SK-N-MC neuroblastoma cells that endogenously express D1 receptors. Forming nuclear and cytoplasmic aggregates, mutant htt-EGFP was toxic to cells beyond 24 h post-transfection. Remarkably, low doses of a selective D1 receptors agonist or forskolin, an activator of adenylate cyclase, accelerated the formation of mutant htt nuclear aggregates, whereas the number of cytoplasmic aggregates was decreased. These effects were associated with a minor increase in cell death. Understanding the functional bases of these effects may further elucidate the role of dopamine receptors signaling in the complex pathophysiology of HD.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Neuroscience - Volume 153, Issue 3, 15 May 2008, Pages 762-772
Journal: Neuroscience - Volume 153, Issue 3, 15 May 2008, Pages 762-772
نویسندگان
P. Robinson, M. Lebel, M. Cyr,