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• W2100566384 abstract "<h3>Objective:</h3> We measured the levels of mutant huntingtin (mtHtt) and total huntingtin (tHtt) in blood leukocytes from Prospective Huntington At-Risk Observational Study (PHAROS) subjects at 50% risk of carrying the Huntington disease mutation using a homogeneous time-resolved fluorescence (HTRF) assay to assess its potential as a biomarker. <h3>Methods:</h3> Peripheral blood mononuclear cells from consenting PHAROS subjects were analyzed by HTRF using antibodies that simultaneously measured mtHtt and tHtt. mtHtt levels were normalized to tHtt, double-stranded DNA, or protein and analyzed according to cytosine-adenine-guanine repeat length (CAGn), demographics, predicted time to clinical onset or known time since clinical onset, and available clinical measures. <h3>Results:</h3> From 363 assayed samples, 342 met quality control standards. Levels of mtHtt and mt/tHtt were higher in 114 subjects with expanded CAG repeats (CAG ≥37) compared with 228 subjects with nonexpanded CAG repeats (CAG <37) (<i>p</i> < 0.0001). Analysis of relationships to predicted time to onset or to phenoconversion suggested that the HTRF signal could mark changes during the Huntington disease prodrome or after clinical onset. <h3>Conclusions:</h3> The HTRF assay can effectively measure mtHtt in multicenter sample sets and may be useful in trials of therapies targeting huntingtin." @default.
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• W2100566384 date "2013-08-21" @default.
• W2100566384 modified "2023-09-27" @default.
• W2100566384 title "HTRF analysis of soluble huntingtin in PHAROS PBMCs" @default.
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• W2100566384 doi "https://doi.org/10.1212/wnl.0b013e3182a55ede" @default.
• W2100566384 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/3795598" @default.
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