RNA editing is an alteration in the primary nucleotide sequences resulting from 
a chemical change in the base. RNA editing is observed in eukaryotic mRNA, 
transfer RNA, ribosomal RNA, and non-coding RNAs (ncRNA). The most common RNA 
editing in the mammalian central nervous system is a base modification, where 
the adenosine residue is base-modified to inosine (A to I). Studies from ADAR 
(adenosine deaminase that act on RNA) mutants in Caenorhabditis elegans, 
Drosophila, and mice clearly show that the RNA editing process is an absolute 
requirement for nervous system homeostasis and normal physiology of the animal. 
Understanding the mechanisms of editing and findings of edited substrates has 
provided a better knowledge of the phenotype due to defective and hyperactive 
RNA editing. A to I RNA editing is catalyzed by a family of enzymes knows as 
ADARs. ADARs modify duplex RNAs and editing of duplex RNAs formed by ncRNAs can 
impact RNA functions, leading to an altered regulatory gene network. Such 
altered functions by A to I editing is observed in mRNAs, microRNAs (miRNA) but 
other editing of small and long ncRNAs (lncRNAs) has yet to be identified. Thus, 
ncRNA and RNA editing may provide key links between neural development, nervous 
system function, and neurological diseases. This review includes a summary of 
seminal findings regarding the impact of ncRNAs on biological and pathological 
processes, which may be further modified by RNA editing. NcRNAs are 
non-translated RNAs classified by size and function. Known ncRNAs like miRNAs, 
smallRNAs (smRNAs), PIWI-interacting RNAs (piRNAs), and lncRNAs play important 
roles in splicing, DNA methylation, imprinting, and RNA interference. Of note, 
miRNAs are involved in development and function of the nervous system that is 
heavily dependent on both RNA editing and the intricate spatiotemporal 
expression of ncRNAs. This review focuses on the impact of dysregulated A to I 
editing and ncRNAs in neurodegeneration.