کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
8258689 | 1534612 | 2018 | 40 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Disruption of AT-hook 1 domain in MeCP2 protein caused behavioral abnormality in mice
دانلود مقاله + سفارش ترجمه
دانلود مقاله ISI انگلیسی
رایگان برای ایرانیان
کلمات کلیدی
TRDTranscriptional Repression DomainMethyl-CpG binding domainXCIHMGAMCGHMCMBDMCHMecp2MUTGFAPCRISPR/Cas9 - CRISPR / Cas9Iba-1 - IBA-1base pair - جفت پایهMutant - جهش یافتهRett syndrome - سندروم رتX chromosome inactivation - غیر فعال کردن کروموزوم Xionized calcium-binding adapter molecule-1 - مولکول-آداپتور اتصال دهنده کلسیم یونیزه-1Inhibitory neurons - نورونهای مهارکنندهwide type - نوع گسترده ایhydroxymethylcytosine - هیدروکسی متیل سیتوزینAntibody - پادتَن یا آنتیبادیGlial fibrillary acidic protein - پروتئین اسیدی فیبریلاسیون گلایالmethyl-CpG binding protein 2 - پروتئین متصل CpG متیل 2
موضوعات مرتبط
علوم زیستی و بیوفناوری
بیوشیمی، ژنتیک و زیست شناسی مولکولی
سالمندی
پیش نمایش صفحه اول مقاله
چکیده انگلیسی
MECP2 is the causative gene for autism spectrum disorders, including Rett syndrome, a regressive neurodevelopmental rare disease mainly occurring in girls. Except for the distinct methyl-CpG binding domain and the transcriptional repression domain in MeCP2, three AT-hook-like domains have recently been identified. Several mutations in AT-hook 1 domain have been reported in autism cases or Rett database. However, the role of AT-hook 1 domain is still unclear. In this study, we generated a mouse line carrying deletion of eight conserved amino acids in AT-hook 1 domain by clustered regularly interspaced short palindromic repeats (CRISPR)/Cas9 technology. Mecp2ÎAT-hook1/y mutant male mice exhibited low locomotor activity, motor incoordination and cognitive deficit. In addition, these mutant mice exhibited increased anxiety. Moreover, pain insensitivity was noted in the mutant males. However, the social interactions were unaffected in AT-hook 1 mutant mice. Thinner CA1 region of the hippocampus was observed in the mutant mice. On the molecular basis, Western blot analysis showed increased expression of mutant MeCP2 protein in the cortex. Additionally, several genes expressed specifically in inhibitory neurons were markedly changed in the cerebrum. Taken together, these data demonstrate that disruption of AT-hook 1 domain in MeCP2 caused behavioral abnormality in mice, which suggests that AT-hook 1 is a critical region for the function of MeCP2 protein.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease - Volume 1864, Issue 2, February 2018, Pages 347-358
Journal: Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease - Volume 1864, Issue 2, February 2018, Pages 347-358
نویسندگان
Miaojing Xu, Pingping Song, Wei Huang, Rongni He, Yong He, Xiao Zhou, Yong Gu, Suyue Pan, Yafang Hu,