Target intelligence / Profile preview

U8 small nucleolar RNA (U8)

Target
U8
Molecular classification
Small nucleolar RNA (snoRNA), C/D box snoRNA, Non-coding RNA (ncRNA)
01

Overview

U8 small nucleolar RNA is a vertebrate-specific non-coding RNA that forms part of a small nucleolar ribonucleoprotein complex (snoRNP) essential for the biogenesis of the large subunit of ribosomes. Encoded by the SNORD118 gene in humans, U8 snoRNA is classified as a C/D box snoRNA but is unusual in that it is not involved in 2'-O-methylation of rRNA. Instead, U8 is directly required for the cleavage and correct processing of precursor rRNA to yield mature 5.8S and 28S rRNAs, thereby enabling ribosome assembly. U8 snoRNA mutations lead to the rare neurodegenerative condition known as leukoencephalopathy with brain calcifications and cysts (LCC or Labrune syndrome), making SNORD118 a disease gene but not a typical drug or therapeutic protein target[2][3][1][4][5]. U8 is found only in vertebrates and is absent from yeast and most non-vertebrate eukaryotes. Although upregulation of U8 has been observed in certain cancers, its role as a therapeutic target or biomarker remains unclear[2][4].

Other names
SNORD1185.4S RNA (historical)CeN21 (Caenorhabditis elegans)CeR-2 (Caenorhabditis elegans)
02

Biological functions

Ribosome biogenesisProcessing of precursor ribosomal RNA (pre-rRNA) to generate mature 5.8S and 28S rRNAsRegulation of rRNA cleavage (not 2'-O-methylation, which is the typical function of many C/D box snoRNAs)
03

Disease associations

Leukoencephalopathy with brain calcifications and cysts (LCC, also called Labrune syndrome)RibosomopathyPossibly proto-oncogenic activities (upregulated in some cancers, but direct role unclear)

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