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fibrillarin


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The component in a small nucleolar ribonucleoprotein (snoRNP) particle that catalyses the site-specific 2′-hydroxyl methylation of ribose moieties in pre-ribosomal RNA, site-specificity being provided by a guide RNA. It is a highly conserved protein (321 aa), associates with U3, U8, and U13 small nuclear RNAs and is found in the coiled body of the nucleolus. Antibodies to fibrillarin are found in various autoimmune diseases including scleroderma, systematic sclerosis, CREST syndrome, and other connective tissue diseases.

Subjects: Medicine and Health — Chemistry.


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