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Based on the involvement of such processes in FRDA, initial pathophysiological hypotheses focused on reactive oxygen species (ROS) production as a key component of the mechanism. With further study, a variety of other events appear to be involved, including abnormalities of mitochondrially related metabolism and dysfunction in mitochondrial biogenesis. Consequently, present therapies focus not only on free radical damage, but also on control of metabolic abnormalities and correction of mitochondrial biogenesis. Understanding the multitude of abnormalities in FRDA thus offers possibilities for treatment of this disorder.<\/jats:p>","DOI":"10.1042\/ns20180060","type":"journal-article","created":{"date-parts":[[2018,10,11]],"date-time":"2018-10-11T09:55:57Z","timestamp":1539251757000},"update-policy":"https:\/\/doi.org\/10.1042\/crossmark_policy","source":"Crossref","is-referenced-by-count":57,"title":["Role of frataxin protein deficiency and metabolic dysfunction in Friedreich ataxia, an autosomal recessive mitochondrial disease"],"prefix":"10.1042","volume":"2","author":[{"given":"Elisia","family":"Clark","sequence":"first","affiliation":[{"name":"Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, PA, U.S.A."},{"name":"Penn\/CHOP Center of Excellence in Friedreich Ataxia, Philadelphia, PA, U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph","family":"Johnson","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, PA, U.S.A."},{"name":"Penn\/CHOP Center of Excellence in Friedreich Ataxia, Philadelphia, PA, U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yi\u00a0Na","family":"Dong","sequence":"additional","affiliation":[{"name":"Division of Neurology, Children\u2019s Hospital of Philadelphia, Philadelphia, PA, U.S.A."},{"name":"Penn\/CHOP Center of Excellence in Friedreich Ataxia, Philadelphia, PA, U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth","family":"Mercado-Ayon","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, PA, U.S.A."},{"name":"Penn\/CHOP Center of Excellence in Friedreich Ataxia, Philadelphia, PA, U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nathan","family":"Warren","sequence":"additional","affiliation":[{"name":"Division of Neurology, Children\u2019s Hospital of Philadelphia, Philadelphia, PA, U.S.A."},{"name":"Penn\/CHOP Center of Excellence in Friedreich Ataxia, Philadelphia, PA, U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mattieu","family":"Zhai","sequence":"additional","affiliation":[{"name":"Division of Neurology, Children\u2019s Hospital of Philadelphia, Philadelphia, PA, U.S.A."},{"name":"Penn\/CHOP Center of Excellence in Friedreich Ataxia, Philadelphia, PA, U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emily","family":"McMillan","sequence":"additional","affiliation":[{"name":"Division of Neurology, Children\u2019s Hospital of Philadelphia, Philadelphia, PA, U.S.A."},{"name":"Penn\/CHOP Center of Excellence in Friedreich Ataxia, Philadelphia, PA, U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amy","family":"Salovin","sequence":"additional","affiliation":[{"name":"Division of Neurology, Children\u2019s Hospital of Philadelphia, Philadelphia, PA, U.S.A."},{"name":"Penn\/CHOP Center of Excellence in Friedreich Ataxia, Philadelphia, PA, U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hong","family":"Lin","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, PA, U.S.A."},{"name":"Penn\/CHOP Center of Excellence in Friedreich Ataxia, Philadelphia, PA, U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7168-214X","authenticated-orcid":false,"given":"David\u00a0R.","family":"Lynch","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, PA, U.S.A."},{"name":"Penn\/CHOP Center of Excellence in Friedreich Ataxia, Philadelphia, PA, U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"288","published-online":{"date-parts":[[2018,11,2]]},"reference":[{"key":"2021111608020792600_B1","doi-asserted-by":"publisher","first-page":"433","DOI":"10.1007\/BF01878006","article-title":"ber degenerative Atrophie der spinalen Hinterstrange","volume":"26","author":"Friedreich","year":"1863","journal-title":"Virchows Arch. 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