Nop10 Background Information Nop10, also known as NOLA3 (nucleolar protein family A member 3), is a 64 amino acid protein that localizes to the nucleolus, as well as to cajal bodies, and exists as a component of the multi-protein H/ACA small nucleolar ribonucleoprotein (H/ACA snoRNP) complex. Working in tandem with other members of the H/ACA snoRNP complex, Nop10 plays an essential role in telomere maintenance and ribosome biogenesis and is also thought to be required for the proper processing and trafficking of the TERT (telomerase reverse transcriptase) holoenzyme. Defects in the gene encoding Nop10 are the cause of dyskeratosis congenita autosomal recessive (ARDKC), a rare and progressive bone marrow failure syndrome that is characterized by reticulated skin hyperpigmentation, nail dystrophy and mucosal leukoplakia.
