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The SBDS protein participates in ribosome biogenesis; however, effects beyond reduced translation efficiency are thought to be involved in SDS progression. Impaired mitochondrial function has been reported for cells lacking either SBDS or Sdo1p, the <jats:italic>Saccharomyces cerevisiae<\/jats:italic> SBDS ortholog. To better understand how the loss of SBDS\/Sdo1p leads to mitochondria damage, we utilized the <jats:italic>S. cerevisiae<\/jats:italic> model of SDS. Yeast deleted for <jats:italic>SDO1<\/jats:italic> show increased oxidative damage to mitochondrial proteins and a marked decrease in protein levels and activity of mitochondrial superoxide dismutase 2 (Sod2p), a key enzyme involved in defense against oxidants. Immature forms of Sod2p are observed in <jats:italic>sdo1<\/jats:italic>\u2206 cells suggesting a defect in proteolysis of the presequence. Yeast deleted for <jats:italic>CYM1<\/jats:italic>, encoding a presequence protease, display a similar reduction in Sod2p activity as <jats:italic>sdo1<\/jats:italic>\u2206 cells, as well as elevated oxidative damage, to mitochondrial proteins. Sod2p protein levels and activity are largely restored in a <jats:italic>por1<\/jats:italic>\u2206 <jats:italic>sdo1<\/jats:italic>\u2206 strain, lacking the major mitochondrial voltage\u2010dependent anion channel. Together these results indicate that mitochondrial insufficiency in <jats:italic>sdo1<\/jats:italic>\u2206 cells may be linked to the accumulation of immature presequence containing proteins and this effect is a consequence, at least in part, from loss of counter\u2010regulation of Por1p by Sdo1p.<\/jats:p>","DOI":"10.1002\/jcb.28660","type":"journal-article","created":{"date-parts":[[2019,4,2]],"date-time":"2019-04-02T17:11:34Z","timestamp":1554225094000},"page":"13867-13880","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Decreased accumulation of superoxide dismutase 2 within mitochondria in the yeast model of Shwachman\u2010Diamond syndrome"],"prefix":"10.1002","volume":"120","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3199-1743","authenticated-orcid":false,"given":"Laran T.","family":"Jensen","sequence":"first","affiliation":[{"name":"Department of Biochemistry, Faculty of Science Mahidol University 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