Nucleolar protein 56 (NOP56)

Nucleolar protein 56 (NOP56) is a highly conserved nucleolar protein that functions as an essential subunit of the box C/D small nucleolar ribonucleoprotein (snoRNP) complexes, which are responsible for the 2′-O-methylation and processing of precursor ribosomal RNA (pre-rRNA)[1][2][3][4]. NOP56 is involved in the assembly and function of the 60S ribosomal subunit, as well as in ribosome biogenesis by forming a core part of the snoRNPs together with fibrillarin, NOP58, and SNU13 proteins[2][4]. It plays a vital role in the processing of rRNA and the synthesis of mature ribosomes, and thus is crucial for proper cellular protein synthesis[1][2][4]. Defects in the NOP56 gene, including expanded nucleotide repeats, are causally associated with spinocerebellar ataxia 36 (SCA36), a progressive neurodegenerative disease primarily affecting the cerebellum[3]. Abnormal NOP56 expression or function has also been reported to impact cancer progression, likely through dysregulation of ribosome biogenesis and cellular metabolism[1]. NOP56 is not considered a classical drug target such as a receptor, enzyme, or transporter; rather, it is a critical housekeeping factor for fundamental cell biological processes[1][2][3][4].

Other names

NOL5ASCA36Nucleolar protein 5Aspinocerebellar ataxia 36NOP56 ribonucleoprotein homolognucleolar protein 56NOP56 ribonucleoproteinnucleolar protein 5A (56 kDa with KKE/D repeat)