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Small nucleolar RNA SNORD116 is a non-coding RNA of the box C/D snoRNA family, found in a tandemly repeated cluster within the imprinted 15q11–q13 region of human chromosome 15. Unlike typical snoRNAs, SNORD116 lacks known canonical targets and is thus referred to as an “orphan” snoRNA. It may guide 2′-O-methylation of yet-unidentified substrates, but it is also processed into long non-coding RNAs implicated in regulating mRNA splicing patterns and stability, particularly of genes rapidly induced in neurons. Loss of SNORD116 expression, due most commonly to paternal microdeletion, causes or contributes to the core symptoms of Prader–Willi syndrome, including neurodevelopmental, metabolic, and endocrine dysfunctions. SNORD116 is not currently considered a therapeutic drug target, nor are there drugs known to modulate its function. Its critical biological functions are still being characterized, but its unique roles in RNA metabolism, neuronal regulation, and imprinting make it a subject of intense biomedical research.
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