Small nucleolar RNA SNORD100

Small nucleolar RNA SNORD100 (SNORD100), also known as U100, is a non-coding RNA molecule that belongs to the C/D box snoRNAs family. These molecules are critical for the biogenesis of ribosomes and are involved in the chemical modification of ribosomal RNAs (rRNAs), specifically in the methylation of ribose sugars. SNORD100 is located within the introns of host genes and is processed from pre-mRNA transcripts. This small nucleolar RNA plays a significant role in the modification and processing of rRNA, which is essential for the proper assembly and function of ribosomes, the cellular machines responsible for protein synthesis.

Function

SNORD100 is involved in the site-specific 2'-O-methylation of rRNA molecules. This modification is crucial for the correct folding and structural stability of rRNA, thereby influencing ribosome assembly and function. The presence of 2'-O-methylation helps to fine-tune the ribosome's ability to accurately and efficiently translate mRNA into proteins. SNORD100, like other C/D box snoRNAs, associates with specific proteins to form small nucleolar ribonucleoproteins (snoRNPs). These complexes guide the snoRNA to its specific target sites on the rRNA molecule for methylation.

Biogenesis and Localization

SNORD100 is transcribed as part of a host gene's intron. After transcription, it is liberated from the intron via splicing and then processed into its mature form. This processing involves the formation of the characteristic C and D boxes that are critical for the snoRNA's function. Once processed, SNORD100 is transported to the nucleolus, the site of ribosome biogenesis, where it performs its function in rRNA modification.

Clinical Significance

While the specific clinical implications of SNORD100 are still under investigation, the general dysregulation of snoRNAs has been associated with various diseases, including cancer. Alterations in the expression levels or mutations in snoRNAs can disrupt normal ribosome biogenesis and function, potentially leading to disease. As such, understanding the role and regulation of SNORD100 and other snoRNAs could provide insights into their potential involvement in disease mechanisms and offer new avenues for therapeutic intervention.

Related Pages

• Ribosome
• RNA
• Nucleolus
• Methylation
• Non-coding RNA
• Ribosomal RNA

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