Recombinant Human Nucleolar protein 56 (NOP56)

Uniprot Id

O00567

Target Species

Human

Target Name

NOP56

Target Full Name

Nucleolar protein 56

Target Function

Involved in the early to middle stages of 60S ribosomal subunit biogenesis. Core component of box C/D small nucleolar ribonucleoprotein (snoRNP) particles. Required for the biogenesis of box C/D snoRNAs such U3, U8 and U14 snoRNAs.

Target Involvement

Spinocerebellar ataxia 36 (SCA36)

Target Subcellular Location

Nucleus, nucleolus. Cytoplasm. Nucleus, nucleoplasm.

Target Protein Families

NOP5/NOP56 family

Target Synonyms

NOP56; NOL5ANucleolar protein 56; Nucleolar protein 5A

Target Background

Nop56p is a yeast nucleolar protein that is part of a complex with the nucleolar proteins Nop58p and fibrillarin. Nop56p is required for assembly of the 60S ribosomal subunit and is involved in pre-rRNA processing. The protein encoded by this gene is similar in sequence to Nop56p and is also found in the nucleolus. Expansion of a GGCCTG repeat from 3-8 copies to 1500-2500 copies in an intron of this gene results in spinocerebellar ataxia 36. Multiple transcript variants encoding several different isoforms have been found for this gene, but the full-length nature of most of them has not been determined.