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Record W4200625369 · doi:10.1038/s42003-021-02895-4

Huntingtin structure is orchestrated by HAP40 and shows a polyglutamine expansion-specific interaction with exon 1

2021· article· en· W4200625369 on OpenAlex
Rachel Harding, Justin C. Deme, Johannes F. Hevler, Sem Tamara, Alexander Lemak, Jeffrey P. Cantle, Magdalena M. Szewczyk, Nola Begeja, Siobhan Goss, Xiaobing Zuo, P. Loppnau, Alma Seitova, Ashley Hutchinson, Lixin Fan, Ray Truant, Matthieu Schapira, Jeffrey B. Carroll, Albert J. R. Heck, Susan M. Lea, C.H. Arrowsmith

Why this work is in the frame

A frame that forgets how it found something cannot be audited. These are the routes that admitted this work.

affAt least one author lists a Canadian institution in the pinned OpenAlex snapshot.
fundA Canadian funder is recorded on the work.

Bibliographic record

VenueCommunications Biology · 2021
Typearticle
Languageen
FieldNeuroscience
TopicGenetic Neurodegenerative Diseases
Canadian institutionsPrincess Margaret Cancer CentreStructural Genomics ConsortiumMcMaster UniversityUniversity of Toronto
FundersOffice of ScienceGenentechInnovative Medicines InitiativeOntario Genomics InstituteNational Cancer InstituteEuropean Federation of Pharmaceutical Industries and AssociationsFrederick National Laboratory for Cancer ResearchWellcome TrustMerck KGaAHuntington Society of CanadaNational Institutes of HealthOntario GenomicsGenome CanadaCHDI FoundationBayerPfizerHuntington's Disease Society of AmericaArgonne National LaboratoryU.S. Department of Energy
KeywordsHuntingtinPolyglutamine tractExonHuntingtin ProteinMutantTrinucleotide repeat expansionWild typeDimerBiologyChemistryCell biologyBiophysicsGeneticsGeneAllele

Abstract

fetched live from OpenAlex

Huntington's disease results from expansion of a glutamine-coding CAG tract in the huntingtin (HTT) gene, producing an aberrantly functioning form of HTT. Both wildtype and disease-state HTT form a hetero-dimer with HAP40 of unknown functional relevance. We demonstrate in vivo and in cell models that HTT and HAP40 cellular abundance are coupled. Integrating data from a 2.6 Å cryo-electron microscopy structure, cross-linking mass spectrometry, small-angle X-ray scattering, and modeling, we provide a near-atomic-level view of HTT, its molecular interaction surfaces and compacted domain architecture, orchestrated by HAP40. Native mass spectrometry reveals a remarkably stable hetero-dimer, potentially explaining the cellular inter-dependence of HTT and HAP40. The exon 1 region of HTT is dynamic but shows greater conformational variety in the polyglutamine expanded mutant than wildtype exon 1. Our data provide a foundation for future functional and drug discovery studies targeting Huntington's disease and illuminate the structural consequences of HTT polyglutamine expansion.

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.

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: Bench or experimental · Consensus signal: Bench or experimental
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.019
Threshold uncertainty score0.504

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)0.0000.000

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 head0.056
GPT teacher head0.305
Teacher spread0.249 · 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