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Biomarkers associated with disease progression and severity could aid in disease prognostication. We evaluated the utility of glial fibrillary acidic protein (GFAP) and neurofilament light (NfL) in distinguishing patients with SCA7 from healthy controls and estimating patient prognosis. GFAP and NfL levels were measured in 23 plasma and 20 cerebrospinal fluid (CSF) samples from asymptomatic (N = 3) and symptomatic SCA7 participants (N = 10) and from healthy controls (N = 8). GFAP concentrations were elevated in the plasma (82.7 pg\/mL) and CSF (9318 pg\/mL) of patients with SCA7 compared to controls (plasma: 48.0 pg\/mL; CSF: 89,056 pg\/mL). Similarly, NfL plasma (21.6 pg\/mL) and CSF (2615.0 pg\/mL) levels were also significantly upregulated in SCA7 compared to controls (plasma: 8.2 pg\/mL; CSF: 414.6 pg\/mL). Higher levels of NfL, but not of GFAP, significantly discriminated symptomatic SCA7 patients from controls (area under de curve, AUC: 0.898, p = 0.0059, in plasma, and AUC: 1.0, p = 0.0012, in CSF). The levels of both biomarkers increased overtime, with plasma NfL levels strongly associated with a worse score in the scale for the assessment and rating of ataxia (SARA) (Spearman r: 0.8354, p = 0.0007; regression analysis: \u03b2: 0.021, 95% CI: 0.008\u20130.035, p = 0.0048). These findings suggest that NfL could serve as a valuable biomarker for monitoring disease progression and prognosis in SCA7 patients.<\/jats:p>","DOI":"10.3390\/ijms26115070","type":"journal-article","created":{"date-parts":[[2025,5,25]],"date-time":"2025-05-25T20:26:50Z","timestamp":1748204810000},"page":"5070","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Evaluating Glial Fibrillary Acidic Protein and Neurofilament Light as Potential Biomarkers for Spinocerebellar Ataxia 7"],"prefix":"10.3390","volume":"26","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0447-4089","authenticated-orcid":false,"given":"Rana","family":"Hanna Al-Shaikh","sequence":"first","affiliation":[{"name":"Department of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA"},{"name":"Department of Neurology, Mayo Clinic, Jacksonville, FL 32224, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karen","family":"Jansen-West","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA"},{"name":"Neuroscience Graduate Program, Mayo Graduate School, Mayo Clinic College of Medicine, Jacksonville, FL 32224, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Audrey","family":"Strongosky","sequence":"additional","affiliation":[{"name":"Department of Neurology, Mayo Clinic, Jacksonville, FL 32224, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zoe","family":"Parrales","sequence":"additional","affiliation":[{"name":"Department of Neurology, Mayo Clinic, Jacksonville, FL 32224, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0190-0089","authenticated-orcid":false,"given":"Judith A.","family":"Dunmore","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuping","family":"Song","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tania F.","family":"Gendron","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA"},{"name":"Neuroscience Graduate Program, Mayo Graduate School, Mayo Clinic College of Medicine, Jacksonville, FL 32224, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juan C.","family":"Guevara","sequence":"additional","affiliation":[{"name":"Department of Science, University of Barcelona, 08007 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2305-1073","authenticated-orcid":false,"given":"Helio A. G.","family":"Teive","sequence":"additional","affiliation":[{"name":"Movement Disorders Unit, Neurology Service, Internal Medicine Department, Hospital de Cl\u00ednicas, Federal University of Paran\u00e1, Curitiba 80060-90, PR, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jaros\u0142aw","family":"Dulski","sequence":"additional","affiliation":[{"name":"Department of Neurology, Mayo Clinic, Jacksonville, FL 32224, USA"},{"name":"Division of Neurological and Psychiatric Nursing, Medical University of Gda\u0144sk, 80-211 Gdansk, Poland"},{"name":"Neurology Department, St. Adalbert Hospital, Copernicus PL Ltd., 80-462 Gdansk, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jaros\u0142aw","family":"S\u0142awek","sequence":"additional","affiliation":[{"name":"Division of Neurological and Psychiatric Nursing, Medical University of Gda\u0144sk, 80-211 Gdansk, Poland"},{"name":"Neurology Department, St. Adalbert Hospital, Copernicus PL Ltd., 80-462 Gdansk, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leonard","family":"Petrucelli","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA"},{"name":"Neuroscience Graduate Program, Mayo Graduate School, Mayo Clinic College of Medicine, Jacksonville, FL 32224, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5487-1053","authenticated-orcid":false,"given":"Zbigniew K.","family":"Wszolek","sequence":"additional","affiliation":[{"name":"Department of Neurology, Mayo Clinic, Jacksonville, FL 32224, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4894-4858","authenticated-orcid":false,"given":"Mercedes","family":"Prudencio","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA"},{"name":"Neuroscience Graduate Program, Mayo Graduate School, Mayo Clinic College of Medicine, Jacksonville, FL 32224, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,5,24]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1186\/1750-1172-8-14","article-title":"Autosomal dominant cerebellar ataxia type III: A review of the phenotypic and genotypic characteristics","volume":"8","author":"Fujioka","year":"2013","journal-title":"Orphanet. 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