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The primary cause of FRDA is the presence of expanded DNA triplet (GAA) repeats in the first intron of the <jats:italic>fxn<\/jats:italic> gene on chromosome 9q13. The expanded DNA repeats in <jats:italic>fxn<\/jats:italic> inhibit expression of the protein frataxin, which leads to neuronal degeneration. The aim of the study was to identify differentially expressed plasma proteins in FRDA patients for their diagnostic\/prognostic applications. Clinically suspected FRDA patients (n = 42) were assessed on the International Co\u2010Operative Ataxia Rating Scale (ICARS), and genetic confirmation was performed by analyzing (GAA) repeats via PCR. Eighteen patients were confirmed to be homozygous for FRDA, with ICARS scores of 40 \u00b1 8. Plasma proteomics of homozygous FRDA patients and age\u2010 and gender\u2010matched healthy controls was done using two\u2010dimensional difference in\u2010gel electrophoresis and LC\u2010MS\/MS. Quantitative proteomic analysis (fold change \u22651.5; <jats:italic>P<\/jats:italic> &lt; 0.05) revealed 13 differentially expressed protein spots. These proteins were found to be associated with neuropathy (\u03b11\u2010antitrypsin), ataxia (apolipoprotein A\u2010I), oxidative stress (albumin), altered lipid metabolism (apolipoprotein C\u2010II, C\u2010III), etc. Further investigations of these differentially expressed proteins can aid in identifying prognostic\/diagnostic markers for FRDA. \u00a9 2013 Wiley Periodicals, Inc.<\/jats:p>","DOI":"10.1002\/jnr.23262","type":"journal-article","created":{"date-parts":[[2013,8,30]],"date-time":"2013-08-30T14:41:04Z","timestamp":1377873664000},"page":"1483-1491","source":"Crossref","is-referenced-by-count":20,"title":["Quantitative profiling and identification of differentially expressed plasma proteins in friedreich's ataxia"],"prefix":"10.1002","volume":"91","author":[{"given":"Vishnu","family":"Swarup","sequence":"first","affiliation":[{"name":"Department of Biochemistry All India Institute of Medical Sciences New Delhi 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