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

Properties

SIZE: entity length = 64 aa MW = 8 kD COMPARTMENT: cell nucleus

Database Correlations

OMIM correlations MORBIDMAP 606471 UniProt Q9NPE3 Pfam PF04135 Entrez Gene 55505 Kegg hsa:55505

References

UniProt :accession Q9NPE3