کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
8839934 | 1613766 | 2018 | 28 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Pleiotropic neuropathological and biochemical alterations associated with Myo5a mutation in a rat Model
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کلمات کلیدی
ALDHMesencephalonPBSDOPACDTNBNGS5,5′-dithiobis(2-nitrobenzoic acid) - 5،5'-dithiobis (2-nitrobenzoic acid)SNpc - SNPCAutophagy - اتوفاژیStriatum - استریاتومSprague Dawley - اسپراگ داولیaldehyde dehydrogenases - الdehyde dehydrogenasesImmunohistochemistry - ایمونوهیستوشیمیIHC - ایمونوهیستوشیمیTem - این استsubstantia nigra pars compacta - توده سیاه پارس متراکمMutant - جهش یافتهDopamine - دوپامینdihydroxyphenylacetic acid - دی هیدروکسی فنیل اسیدهای اسیدmicroRNAs - ریز آرانایelectron transport chain - زنجیره انتقال الکترونbrain stem - ساقه مغزDOPAL - عصبانی کردنfrontal cortex - قشر جلوییPhosphate-buffered saline - محلول نمک فسفات با خاصیت بافریCerebellum - مخچهMiRNA - میکروRNA، ریزآرانای، miRNATransmission electron microscopy - میکروسکوپ الکترونی عبوریHippocampus - هیپوکامپ ETc - و غیرهWestern blot - وسترن بلاتolfactory bulb - پیاز بویایی
موضوعات مرتبط
علوم زیستی و بیوفناوری
علم عصب شناسی
علوم اعصاب (عمومی)
پیش نمایش صفحه اول مقاله
چکیده انگلیسی
In this study, we analyze the neuropathological and biochemical alterations involved in the pathogenesis of a neurodegenerative/movement disorder during different developmental stages in juvenile rats with a mutant Myosin5a (Myo5a). In mutant rats, a spontaneous autosomal recessive mutation characterized by the absence of Myo5a protein expression in the brain is associated with a syndrome of locomotor dysfunction, altered coat color, and neuroendocrine abnormalities. Myo5a encodes a myosin motor protein required for transport and proper distribution of subcellular organelles in somatodendritic processes in neurons. Here we report marked hyperphosphorylation of alpha-synuclein and tau, as well as region-specific buildup of the autotoxic dopamine metabolite, 3,4-dihydroxyphenyl-acetaldehyde (DOPAL), related to decreased aldehyde dehydrogenases activity and neurodegeneration in mutant rats. Alpha-synuclein accumulation in mitochondria of dopaminergic neurons is associated with impaired enzymatic respiratory complex I and IV activity. The behavioral and biochemical lesions progress after 15â¯days postnatal, and by 30-40â¯days the animals must be euthanized because of neurological impairment. Based on the obtained results, we propose a pleiotropic pathogenesis that links the Myo5a gene mutation to deficient neuronal development and progressive neurodegeneration. This potential model of a neurodevelopmental disorder with neurodegeneration and motor deficits may provide further insight into molecular motors and their associated proteins responsible for altered neurogenesis and neuronal disease pathogenesis.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Brain Research - Volume 1679, 15 January 2018, Pages 155-170
Journal: Brain Research - Volume 1679, 15 January 2018, Pages 155-170
نویسندگان
Kerstin K. Landrock, Patti Sullivan, Heidi Martini-Stoica, David S. Goldstein, Brett H. Graham, Shinya Yamamoto, Hugo J. Bellen, Richard A. Gibbs, Rui Chen, Marcello D'Amelio, George Stoica,