Cerebrospinal fluid glial fibrillary acidic protein, in contrast to amyloid beta protein, is associated with disease symptoms in Huntington's disease

Huntington's disease (HD) is a hereditary neurodegenerative disease, currently lacking disease-modifying treatments. Biomarkers are needed for objective assessment of disease progression. Evidence supports both complex protein aggregation and astrocyte activation in HD. This study assesses the...

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Bibliographic Details
Published in:Italian journal of neurological sciences 2024-04, Vol.459, p.122979-122979, Article 122979
Main Authors: Korpela, Sara, Sundblom, Jimmy, Zetterberg, Henrik, Constantinescu, Radu, Svenningsson, Per, Paucar, Martin, Niemelä, Valter
Format: Article
Language:English
Subjects:
amyloid beta
Amyloid beta-Peptides
Biomarker
Biomarkers
biomarkörer
Cerebrospinal fluid
Disease Progression
Genetics
GFAP
Glial Fibrillary Acidic Protein
Humans
Huntington Disease
Huntington Disease - genetics
Huntington's disease
Huntingtons sjukdom
Medicin och hälsovetenskap
Neurodegeneration
Neurodegenerative Diseases - complications
Neurologi
Neurology
Neurosciences
Neurovetenskaper
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Summary:Huntington's disease (HD) is a hereditary neurodegenerative disease, currently lacking disease-modifying treatments. Biomarkers are needed for objective assessment of disease progression. Evidence supports both complex protein aggregation and astrocyte activation in HD. This study assesses the 42 amino acid long amyloid beta (Aβ42) and glial fibrillary acidic protein (GFAP) as potential biomarkers in the cerebrospinal fluid (CSF) of HD mutation carriers. CSF from participants was obtained from three sites in Sweden. Clinical symptoms were graded with the composite Unified Huntington's disease rating scale (cUHDRS). Protein concentrations were measured using ELISA. Pearson correlations were calculated to assess disease progression association. Results were adjusted for age and collection site. The study enrolled 28 manifest HD patients (ManHD), 13 premanifest HD gene-expansion carriers (PreHD) and 20 controls. Aβ42 levels did not differ between groups and there was no correlation with measures of disease progression. GFAP concentration was higher in ManHD (424 ng/l, SD 253) compared with both PreHD (266 ng/l, SD 92.4) and controls (208 ng/l, SD 83.7). GFAP correlated with both cUHDRS (r = −0.77, p 
ISSN:0022-510X
1878-5883
0392-0461
1878-5883
DOI:10.1016/j.jns.2024.122979