USB1 [YLR132C]
U six biogenesis protein 1; Putative poly(U)-specific 3'-to-5' RNA exonuclease; involved in 3'-end processing of U6 snRNA removing uridines and generating a terminal 2′,3′ cyclic phosphate; essential protein that localizes to the nucleus and mitochondria; overexpression suppresses the respiratory defects of oxa1 and mtf2 mutants; homolog of S.pombe gene, mpn1 and human gene, hUSB1; mutations in hUSB1 are associated with a rare genodermatosis, poikiloderma with neutropenia (OMIM 604173); Belongs to the 2H phosphoesterase superfamily. USB1 family.
Synonyms: USB1, USB1p, YLR132C, Q12208, YLR132Cp ...