Interplay of LIS1 and MeCP2: Interactions and Implications With the Neurodevelopmental Disorders Lissencephaly and Rett Syndrome

Liraz Keidar, Gabi Gerlitz, Aditya Kshirsagar, Michael Tsoory, Tsviya Olender, Xing Wang, Ying Yang, Yu Sheng Chen, Yun Gui Yang, Irina Voineagu, Orly Reiner

نتاج البحث: نشر في مجلةمقالةمراجعة النظراء

8 اقتباسات (Scopus)

ملخص

LIS1 is the main causative gene for lissencephaly, while MeCP2 is the main causative gene for Rett syndrome, both of which are neurodevelopmental diseases. Here we report nuclear functions for LIS1 and identify previously unrecognized physical and genetic interactions between the products of these two genes in the cell nucleus, that has implications on MeCP2 organization, neuronal gene expression and mouse behavior. Reduced LIS1 levels affect the association of MeCP2 with chromatin. Transcriptome analysis of primary cortical neurons derived from wild type, Lis1±, MeCP2−/y, or double mutants mice revealed a large overlap in the differentially expressed (DE) genes between the various mutants. Overall, our findings provide insights on molecular mechanisms involved in the neurodevelopmental disorders lissencephaly and Rett syndrome caused by dysfunction of LIS1 and MeCP2, respectively.

اللغة الأصليةالإنجليزيّة
رقم المقال370
دوريةFrontiers in Cellular Neuroscience
مستوى الصوت13
المعرِّفات الرقمية للأشياء
حالة النشرنُشِر - 14 أغسطس 2019
منشور خارجيًانعم

بصمة

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