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Evaluation of the role of the D2 dopamine receptor in myoclonus dystonia

2000· article· en· W4238500757 on OpenAlexaff
Christine Klein, Nadia Gurvich, Miguel Sena‐Esteves, Susan Bressman, Mitchell F. Brin, Barbara J. Ebersole, Stephen J. Fink, Lars Forsgren, Jennifer Friedman, David A. Grimes, Gösta Holmgren, Mårtin Kyllerman, Anthony E. Lang, Deborah de Leon, Joanne Leung, Cassandra Prioleau, Deborah Raymond, Gunnar Sanner, Rachel Saunders‐Pullman, P. Vieregge, Jan Wahlström, Xandra O. Breakefield, Patricia L. Kramer, Laurie J. Ozelius, Stuart C. Sealfon

Bibliographic record

VenueAnnals of Neurology · 2000
Typearticle
Languageen
FieldMedicine
TopicGlycogen Storage Diseases and Myoclonus
Canadian institutionsToronto General HospitalUniversity of Toronto
Fundersnot available
KeywordsDopamine receptor D2DystoniaMyoclonusGeneticsAgonistMutationReceptorBiologyMutantDopamine receptorGeneNeuroscience

Abstract

fetched live from OpenAlex

A novel Val154→Ile mutation in the D2 dopamine receptor (DRD2) on chromosome 11q23 has recently been shown to be associated with myoclonus dystonia (M-D) in one large family. Sequence analysis of the DRD2 gene in 5 M-D patients from different families did not reveal any mutations, nor was there evidence of linkage to the 11q23 region in the DRD2 gene in four other families. Receptor binding and signal transduction assays of the DRD2 mutant and wild-type receptors revealed identical agonist and antagonist affinities and functional responses. These studies suggest that M-D is genetically heterogeneous. The molecular mechanisms through which the Val→Ile mutation may contribute to M-D remain to be determined. Ann Neurol 2000;47:369–373

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)0.000
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: none
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.656
Threshold uncertainty score0.652

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0000.000
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 science0.0000.000
Research integrity0.0000.000
Insufficient payload (model declined to judge)0.0010.000

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 head0.037
GPT teacher head0.313
Teacher spread0.277 · 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

Citations1
Published2000
Admission routes1
Has abstractyes

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