RNA Editing pp 309-330 | Cite as

RNA Editing in Neurological and Neurodegenerative Disorders

  • Pedro Henrique Costa Cruz
  • Yukio KawaharaEmail author
Part of the Methods in Molecular Biology book series (MIMB, volume 2181)


手机体育投注平台The brain is one of the organs that are preferentially targeted by adenosine-to-inosine (A-to-I) RNA editing, a posttranscriptional modification. This chemical modification affects neuronal development and functions at multiple levels, leading to normal brain homeostasis by increasing the complexity of the transcriptome. This includes modulation of the properties of ion channel and neurotransmitter receptors by recoding, redirection of miRNA targets by changing sequence complementarity, and suppression of immune response by altering RNA structure. Therefore, from another perspective, it appears that the brain is highly vulnerable to dysregulation of A-to-I RNA editing. Here, we focus on how aberrant A-to-I RNA editing is involved in neurological and neurodegenerative diseases of humans including epilepsy, amyotrophic lateral sclerosis, psychiatric disorders, developmental disorders, brain tumors, and encephalopathy caused by autoimmunity. In addition, we provide information regarding animal models to better understand the mechanisms behind disease phenotype.

Key words

ADARs AGS ALS Astrocytoma Autism Epilepsy Glioblastoma Prader-Willi syndrome Psychiatric disorders 



手机体育投注平台This review is dedicated to the memory of the late Professor Marie Öhman who contributed greatly to the field of RNA editing. This work was supported by Grants-in-Aid KAKENHI (17K19352) from the Ministry of Education, Culture, Sports, Science and Technology (MEXT) of Japan and by grants from SENSHIN Medical Research Foundation, The Mochida Memorial Foundation for Medical and Pharmaceutical Research, and the Takeda Science Foundation (to Y.K.). P.H.C.C. was supported by the MEXT scholarship.


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Authors and Affiliations

  1. 1.Department of RNA Biology and Neuroscience, Graduate School of MedicineOsaka UniversityOsakaJapan

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