Mechanism of Repeat-Associated MicroRNAs in Fragile X Syndrome

المؤلفون المشاركون

Kelley, Karen
Chang, Shin-Ju E.
Lin, Shi-Lung

المصدر

Neural Plasticity

العدد

المجلد 2012، العدد 2012 (31 ديسمبر/كانون الأول 2012)، ص ص. 1-10، 10ص.

الناشر

Hindawi Publishing Corporation

تاريخ النشر

2012-06-20

دولة النشر

مصر

عدد الصفحات

10

التخصصات الرئيسية

الأحياء
الطب البشري

الملخص EN

The majority of the human genome is comprised of non-coding DNA, which frequently contains redundant microsatellite-like trinucleotide repeats.

Many of these trinucleotide repeats are involved in triplet repeat expansion diseases (TREDs) such as fragile X syndrome (FXS).

After transcription, the trinucleotide repeats can fold into RNA hairpins and are further processed by Dicer endoribonuclases to form microRNA (miRNA)-like molecules that are capable of triggering targeted gene-silencing effects in the TREDs.

However, the function of these repeat-associated miRNAs (ramRNAs) is unclear.

To solve this question, we identified the first native ramRNA in FXS and successfully developed a transgenic zebrafish model for studying its function.

Our studies showed that ramRNA-induced DNA methylation of the FMR1 5′-UTR CGG trinucleotide repeat expansion is responsible for both pathological and neurocognitive characteristics linked to the transcriptional FMR1 gene inactivation and the deficiency of its protein product FMRP.

FMRP deficiency often causes synapse deformity in the neurons essential for cognition and memory activities, while FMR1 inactivation augments metabotropic glutamate receptor (mGluR)-activated long-term depression (LTD), leading to abnormal neuronal responses in FXS.

Using this novel animal model, we may further dissect the etiological mechanisms of TREDs, with the hope of providing insights into new means for therapeutic intervention.

نمط استشهاد جمعية علماء النفس الأمريكية (APA)

Kelley, Karen& Chang, Shin-Ju E.& Lin, Shi-Lung. 2012. Mechanism of Repeat-Associated MicroRNAs in Fragile X Syndrome. Neural Plasticity،Vol. 2012, no. 2012, pp.1-10.
https://search.emarefa.net/detail/BIM-1002330

نمط استشهاد الجمعية الأمريكية للغات الحديثة (MLA)

Kelley, Karen…[et al.]. Mechanism of Repeat-Associated MicroRNAs in Fragile X Syndrome. Neural Plasticity No. 2012 (2012), pp.1-10.
https://search.emarefa.net/detail/BIM-1002330

نمط استشهاد الجمعية الطبية الأمريكية (AMA)

Kelley, Karen& Chang, Shin-Ju E.& Lin, Shi-Lung. Mechanism of Repeat-Associated MicroRNAs in Fragile X Syndrome. Neural Plasticity. 2012. Vol. 2012, no. 2012, pp.1-10.
https://search.emarefa.net/detail/BIM-1002330

نوع البيانات

مقالات

لغة النص

الإنجليزية

الملاحظات

Includes bibliographical references

رقم السجل

BIM-1002330