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The mutations associated with CA affect functionally diverse genes; furthermore, the underlying genetic basis of a given CA is unknown in many patients. Exome sequencing has emerged as a cost-effective technology to discover novel genetic mutations, including autosomal recessive CA (ARCA). Five recent studies that describe how exome sequencing performed on a diverse pool of ARCA patients revealed 14 unique mutations in\n                    <jats:italic>STUB1<\/jats:italic>\n                    , a gene that encodes carboxy terminus of Hsp70-interacting protein (CHIP). CHIP mediates protein quality control through chaperone and ubiquitin ligase activities and is implicated in alleviating proteotoxicity in several neurodegenerative diseases. However, these recent studies linking\n                    <jats:italic>STUB1<\/jats:italic>\n                    mutations to various forms of ataxia are the first indications that CHIP is directly involved in the progression of a human disease. Similar exome-sequencing studies have revealed novel mutations in ubiquitin-related proteins associated with CA and other neurological disorders. This review provides an overview of CA, describes the benefits and limitations of exome sequencing, outlines newly discovered\n                    <jats:italic>STUB1<\/jats:italic>\n                    mutations, and theorizes on how CHIP and other ubiquitin-related proteins function to prevent neurological deterioration.\n                  <\/jats:p>","DOI":"10.1038\/hgv.2014.18","type":"journal-article","created":{"date-parts":[[2014,10,23]],"date-time":"2014-10-23T03:18:36Z","timestamp":1414034316000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":37,"title":["Emerging evidence of coding mutations in the ubiquitin\u2013proteasome system associated with cerebellar ataxias"],"prefix":"10.1038","volume":"1","author":[{"given":"Sarah M","family":"Ronnebaum","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cam","family":"Patterson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7382-2783","authenticated-orcid":false,"given":"Jonathan C","family":"Schisler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2014,10,23]]},"reference":[{"key":"BFhgv201418_CR1","doi-asserted-by":"publisher","first-page":"227","DOI":"10.1007\/s11910-012-0267-6","volume":"12","author":"A Sailer","year":"2012","unstructured":"Sailer A, Houlden H . 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