ribosome biogenesis protein Nop10; H/ACA ribonucleoprotein complex subunit 3; snoRNP protein NOP10; nucleolar protein family A member 3 (NOP10, NOLA3)

Function

• component of H/ACA small nucleolar ribonucleoprotein complex (H/ACA snoRNP complex)
• within the H/ACA snoRNP complex, forms a stable core with DKC1; the NOP10/DKC1 heterodimers bind NHP2
• involved in ribosome biogenesis, 18S rRNA pseudouridylation &
• role in telomere maintenance

Structure

belongs to the NOP10 family

Compartment

• nucleus, nucleolus. nucleus, Cajal body
• also localized to Cajal bodies (coiled bodies)

Pathology

• defects in NOP10 are a cause of autosomal recessive dyskeratosis congenita

More general terms

• nuclear protein

Additional terms

• nucleolar protein 10 (NOL10)

Component of

• H/ACA small nucleolar ribonucleoprotein complex (H/ACA snoRNP complex)

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=55505
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:55505
• OMIM: https://mirror.omim.org/entry/224230
• OMIM: https://mirror.omim.org/entry/606471
• UniProt: http://www.uniprot.org/uniprot/Q9NPE3.html