کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
8956360 | 1646148 | 2018 | 35 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Motoneuron degeneration in the trigeminal motor nucleus innervating the masseter muscle in Dystonia musculorum mice
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کلمات کلیدی
ISHLCN2BP230PFADAPIBPAG1bullous pemphigoid antigen 1DSTCTBATF3MBPGFAPDRGMo54′,6-diamidino-2-phenylindole - 4 '، 6-دیامیدینو-2-فنیلینولAUC - AUCC/EBP homologous protein - C / EBP پروتئین همولوگIn situ hybridization - Hybridization در محلelectromyography - الکترومیوگرافیEMG - الکترومیوگرافیIHC - ایمونوهیستوشیمیImmunohistochemistry - ایمونوهیستوشیمیNeurodegeneration - تولید نوروژنیکCHOP - تکه کردنCNS - دستگاه عصبی مرکزیDystonin - دیستونینpostnatal days - روزهای پس از تولدcentral nervous system - سیستم عصبی مرکزیperipheral nervous system - سیستم عصبی پیرامونیMandibular nerve - عصب مندیبولmasseter muscle - عضله مسطحactivating transcription factor 3 - فعال کردن عامل رونویسی 3Lipocalin 2 - لیپوکالین 2area under the curve - منطقه تحت منحنیknockout - ناکاوتneurofilament - نوروفیلامنتMotor neuron - نورون حرکتیTrigeminal motor nucleus - هسته موتور دردیHematoxylin and Eosin - هماتوکسیلین و ائوزینparaformaldehyde - پارافرمالدهیدGlial fibrillary acidic protein - پروتئین اسیدی فیبریلاسیون گلایالMyelin basic protein - پروتئین پایه میلینChAT - چتPNS - کارمندان دولتcholera toxin B subunit - کلسترول توکسین B واحدcholine acetyltransferase - کولین استیل ترانسفرازdorsal root ganglia - گانگلیس ریشه پشتی
موضوعات مرتبط
علوم زیستی و بیوفناوری
بیوشیمی، ژنتیک و زیست شناسی مولکولی
بیولوژی سلول
پیش نمایش صفحه اول مقاله
چکیده انگلیسی
Dystonia musculorum (dt) mice, which have a mutation in the Dystonin (Dst) gene, are used as animal models to investigate the human disease known as hereditary sensory and autonomic neuropathy type VI. Massive neuronal cell death is observed, mainly in the peripheral nervous system (PNS) of dt mice. We and others have recently reported a histopathological feature of these mice that neurofilament (NF) accumulates in various areas of the central nervous system (CNS), including motor pathways. Although dt mice show motor disorder and growth retardation, the causes for these are still unknown. Here we performed histopathological analyses on motor units of the trigeminal motor nucleus (Mo5 nucleus), because they are a good system to understand neuronal responses in the mutant CNS, and abnormalities in this system may lead to problems in mastication, with subsequent growth retardation. We report that motoneurons with NF accumulation in the Mo5 nuclei of DstGt homozygous mice express the stress-induced genes CHOP, ATF3, and lipocalin 2 (Lcn2). We also show a reduced number of Mo5 motoneurons and a reduced size of Mo5 nuclei in DstGt homozygous mice, possibly due to apoptosis, given the presence of cleaved caspase 3-positive Mo5 motoneurons. In the mandibular (V3) branches of the trigeminal nerve, which contains axons of Mo5 motoneurons and trigeminal sensory neurons, there was infiltration of Iba1-positive macrophages. Finally, we report atrophy of the masseter muscles in DstGt homozygous mice, which showed abnormal nuclear localization of myofibrils and increased expression of atrogin-1 mRNA, a muscle atrophy-related gene and weaker masseter muscle strength with uncontrolled muscle activity by electromyography (EMG). Taken together, our findings strongly suggest that mastication in dt mice is affected due to abnormalities of Mo5 motoneurons and masseter muscles, leading to growth retardation at the post-weaning stages.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Neurochemistry International - Volume 119, October 2018, Pages 159-170
Journal: Neurochemistry International - Volume 119, October 2018, Pages 159-170
نویسندگان
M. Ibrahim Hossain, Masao Horie, Nozomu Yoshioka, Masayuki Kurose, Kensuke Yamamura, Hirohide Takebayashi,