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Record W3109409261 · doi:10.1093/braincomms/fcaa172

Longitudinal expression changes are weak correlates of disease progression in Huntington’s disease

2020· article· en· W3109409261 on OpenAlexaff
Christopher T. Mitchell, Irina Krier, Jamshid Arjomand, Beth Borowsky, Sarah J. Tabrizi, Blair R. Leavitt, Natalie Arran, Eric Axelson, Éric Bardinet, N Bechtel, Jenny Callaghan, James C. Campbell, Melissa Campbell, David M. Cash, Alan Coleman, David Craufurd, R. Dar Santos, Joji Decolongon, Eve M. Dumas, Alexandra Dürr, Nick C. Fox, E Frajman, Chris Frost, Stephen L. Hicks, NZ Hobbs, Alexandra Hoffman, C Jauffret, Hans J. Johnson, Rebecca Jones, Caroline K. Jurgens, Damián Justo, Stephen Keenan, C. Kennard, P Kraus, Nayana Lahiri, B Landwehrmeier, Douglas R. Langbehn, S Lee, Stéphane Lehéricy, Cécilia Marelli, C Milchman, W Monaco, K Nigaud, Roger J. Ordidge, Anthony O’Regan, Gail Owen, Tracey Pepple, Sarah Queller, Joy Read, Ralf Reilmann, R. A. C. Roos, H. Diana Rosas, M Say, Rachael I. Scahill, Julie C. Stout, Aaron Sturrock, Ellen P. Hart, Allan J. Tobin, Romain Valabrègue, Simon J.A. van den Bogaard, Jeroen van der Grond, C Wang, Kathryn B. Whitlock, Edward J. Wild, M-N Witjes-Ane, Ruth Luthi‐Carter

Bibliographic record

VenueBrain Communications · 2020
Typearticle
Languageen
FieldNeuroscience
TopicGenetic Neurodegenerative Diseases
Canadian institutionsUniversity of British Columbia
FundersUniversity of LeicesterMedical Research CouncilÉcole Polytechnique Fédérale de LausanneCHDI Foundation
KeywordsHuntington's diseaseDiseaseConcordanceMedicinePathogenesisOncologyInternal medicine

Abstract

fetched live from OpenAlex

Huntington's disease is a severe but slowly progressive hereditary illness for which only symptomatic treatments are presently available. Clinical measures of disease progression are somewhat subjective and may require years to detect significant change. There is a clear need to identify more sensitive, objective and consistent measures to detect disease progression in Huntington's disease clinical trials. Whereas Huntington's disease demonstrates a robust and consistent gene expression signature in the brain, previous studies of blood cell RNAs have lacked concordance with clinical disease stage. Here we utilized longitudinally collected samples from a well-characterized cohort of control, Huntington's disease-at-risk and Huntington's disease subjects to evaluate the possible correlation of gene expression and disease status within individuals. We interrogated these data in both cross-sectional and longitudinal analyses. A number of changes in gene expression showed consistency within this study and as compared to previous reports in the literature. The magnitude of the mean disease effect over 2 years' time was small, however, and did not track closely with motor symptom progression over the same time period. We therefore conclude that while blood-derived gene expression indicators can be of value in understanding Huntington's disease pathogenesis, they are insufficiently sensitive to be of use as state biomarkers.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

How this classification was reachedexpand

Full frame distilled prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.

metaresearch head score (Codex)0.000
metaresearch head score (Gemma)0.004
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: Observational
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.144
Threshold uncertainty score0.667

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0000.004
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0000.000
Science and technology studies0.0000.000
Scholarly communication0.0000.000
Open science0.0010.001
Research integrity0.0000.000
Insufficient payload (model declined to judge)0.0000.000

Machine scores (provisional)

The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.

Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.

Opus teacher head0.086
GPT teacher head0.332
Teacher spread0.246 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; a candidate call from one teacher head, not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designObservational
Domainnot available
GenreEmpirical

How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".

Quick stats

Citations9
Published2020
Admission routes1
Has abstractyes

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