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Fibrillarin is a nucleolar protein involved in the processing of pre-18S ribosomal RNA. Nop1p is the yeast homologue of a protein found in all eukaryotes and archea generally called fibrillarin. Fibrillarin/Nop1p is extraordinarily conserved, so that the yeast and human proteins are 67% identical, and the human protein can functionally replace the yeast protein. Patients with the autoimmune disease scleroderma often have strong circulating autoantibodies to a ~34kDa protein which was subsequently found to be fibrillarin. Recent studies show that knock-out of the fibrillarin gene in mice results in embryonic lethality, although mice with only one functional fibrillarin/Nop1p gene were viable.