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One of the most severe types of Dystonia is Early-Onset Torsion Dystonia (EOTD). The best studied and validated EOTD-associated mutation, torsinA\u0394E, is a deletion of a C-terminal glutamate residue in the AAA+ ATPase, torsinA. TorsinA appears to be an Endoplasmic Reticulum (ER)\/Nuclear Envelope chaperone with multiple roles in the secretory pathway and in determining subcellular architecture. Many functions are disabled in the torsinA\u0394E variant, and torsinA\u0394E is also less stable than wild-type torsinA and is a substrate for ER-associated degradation. Nevertheless, the molecular factors involved in torsinA and torsinA\u0394E's biogenesis and degradation have not been fully explored. To identify conserved cellular factors that can alter torsinA\u0394E protein levels, we designed a new high-throughput, automated, genome-wide screen utilizing our validated Saccharomyces cerevisiae torsinA expression system. By analyzing the non-essential yeast deletion collection, we identified 365 deletion strains with altered torsinA\u0394E steady-state levels. One notable hit was EUG1, which encodes a member of the protein disulfide isomerase family (PDIs). PDIs reside in the ER and catalyze the formation of disulfide bonds, mediate protein quality control, and aid in nascent protein folding. We validated the role of select human PDIs in torsinA biogenesis in mammalian cells and found that overexpression of PDIs reduced the levels of torsinA and torsinA\u0394E. Together, our data report the first genome-wide screen to identify cellular factors that reduce expression levels of the EOTD-associated protein torsinA\u0394E. More generally, the identified hits contribute to dissect the cellular machinery involved in folding and degrading a torsinA variant, and constitute potential therapeutic factors for EOTD. This screen can also be readily adapted to identify factors impacting the levels of any protein of interest, considerably expanding the applicability of yeast in both basic and applied research.<\/jats:p>","DOI":"10.1242\/dmm.029926","type":"journal-article","created":{"date-parts":[[2017,8,3]],"date-time":"2017-08-03T00:30:24Z","timestamp":1501720224000},"source":"Crossref","is-referenced-by-count":12,"title":["A novel high-throughput yeast genetic screen for factors modifying protein levels of the Early-Onset Torsion Dystonia-associated variant torsinA\u0394E"],"prefix":"10.1242","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6217-1380","authenticated-orcid":false,"given":"Luc\u00eda F.","family":"Zacchi","sequence":"first","affiliation":[{"name":"School of Chemistry and Molecular Biosciences, The University of Queensland, St Lucia, QLD, 4072, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3820-1612","authenticated-orcid":false,"given":"John C.","family":"Dittmar","sequence":"additional","affiliation":[{"name":"Department of Biological Sciences, Columbia University, New York, New York 10027, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael J.","family":"Mihalevic","sequence":"additional","affiliation":[{"name":"Department of Microbiology and Molecular Genetics, University of Pittsburgh School of Medicine, 5117 Centre Avenue, UPCI Research Pavilion, 2.42e, Pittsburgh, PA 15213, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4936-9207","authenticated-orcid":false,"given":"Annette M.","family":"Shewan","sequence":"additional","affiliation":[{"name":"School of Chemistry and Molecular Biosciences, The University of Queensland, St Lucia, QLD, 4072, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4823-7758","authenticated-orcid":false,"given":"Benjamin L.","family":"Schulz","sequence":"additional","affiliation":[{"name":"School of Chemistry and Molecular Biosciences, The University of Queensland, St Lucia, QLD, 4072, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeffrey L.","family":"Brodsky","sequence":"additional","affiliation":[{"name":"Department of Biological Sciences, A320 Langley Hall, University of Pittsburgh, Pittsburgh, PA 15260, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2247-6459","authenticated-orcid":false,"given":"Kara A.","family":"Bernstein","sequence":"additional","affiliation":[{"name":"Department of Microbiology and Molecular Genetics, University of Pittsburgh School of Medicine, 5117 Centre Avenue, UPCI Research Pavilion, 2.42e, Pittsburgh, PA 15213, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"237","published-online":{"date-parts":[[2017,1,1]]},"reference":[{"key":"2021042612332138400_DMM029926C1","doi-asserted-by":"publisher","first-page":"863","DOI":"10.1002\/mds.25475","article-title":"Phenomenology and classification of dystonia: a consensus update","volume":"28","author":"Albanese","year":"2013","journal-title":"Mov. 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