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Record W1613990573 · doi:10.1212/wnl.0000000000002016

Large-scale assessment of polyglutamine repeat expansions in Parkinson disease

2015· article· en· W1613990573 on OpenAlexfundno aff
Lisa Wang, Jan Aasly, Grazia Annesi, Soraya Bardien, Maria Bozi, Alexis Brice, Jonathan Carr, Sun Ju Chung, Carl E Clarke, David Crosiers, Angela Deutschländer, Gertrud Eckstein, Matthew J. Farrer, Stefano Goldwurm, Gaëtan Garraux, Georgios M. Hadjigeorgiou, Andrew A. Hicks, Nobutaka Hattori, Christine Klein, Beom S. Jeon, Yun Joong Kim, Suzanne Lesage, Juei-Jueng Lin, Timothy Lynch, Peter Lichtner, Anthony E. Lang, Vincent Mok, Barbara Jasińska‐Myga, George D. Mellick, Karen Morrison, Grzegorz Opala, Lasse Pihlstrøm, Peter P. Pramstaller, Sung S. Park, Aldo Quattrone, Ekaterina Rogaeva, Owen A. Ross, Leonidas Stefanis, Joanne Stockton, Peter A. Silburn, Jessie Theuns, Eng King Tan, Hiroyuki Tomiyama, Mathias Toft, Christine Van Broeckhoven, Ryan J. Uitti, Karin Wirdefeldt, Zbigniew K. Wszołek, Georgia Xiromerisiou, Kuo-Chu Yueh, Yi Zhao, Thomas Gasser, Demetrius M. Maraganore, Rejko Krüger, Manu Sharma, R.S Boyle, A Sellbach, John D. O’Sullivan, Greg T. Sutherland, G. Siebert, N. Dissanayaka, Barbara Pickut, Sebastiaan Engelborghs, Bram Meeus, Peter Paul De Deyn, Patrick Cras, Christophe Tzourio, Philippe Amouyel, Marie‐Anne Loriot, Eugénie Mutez, Aurélie Duflot, Jean-Philippe Legendre, Nawal Waucquier, Olaf Rieß, Daniela Berg, Claudia Schulte, Ana Djarmati, Johann Hagenah, Katja Lohman, Georg Auburger, Rüdiger Hilker, Simone van de Loo, Efthimios Dardiotis, Vaïa Tsimourtou, Styliani Ralli, Persa Kountra, Gianna Patramani, Cristina Vogiatzi, Manabu Funayama, Hiroyo Yoshino, Yuanzhe Li, Yoko Imamichi, Tatsushi Toda, Wataru Satake, Enza Maria Valente, Alessandro Ferraris, Bruno Dallapiccola, Tàmara Ialongo, Laura Brighina, B. Corradi, Carlo Ferrarese, Monza Roberto Piolti, Patrizia Tarantino, Ferdinanda Annesi, Monica Gagliardi, Sung Sup Park, Gabriela Kłodowska-Duda, Magdalena Boczarska‐Jedynak, Andrea Carmine Belin, Dagmar Galter, Marie Westerlund, Christer Nilsson, Andreas Puschmann, J-J Lin, J. Eric Ahlskog, Mariza de Andrade, Timothy G. Lesnick, Walter A. Rocca, Harvey Checkoway

Bibliographic record

VenueNeurology · 2015
Typearticle
Languageen
FieldNeuroscience
TopicGenetic Neurodegenerative Diseases
Canadian institutionsnot available
FundersNational Institute of Neurological Disorders and StrokeDepartment of Medicine, University of TorontoHelse Sør-Øst RHFJapan Society for the Promotion of ScienceNational Medical Research CouncilMedical Research CouncilAllerganFondation pour la Recherche sur AlzheimerSt. Olavs Hospital Universitetssykehuset i TrondheimSingapore Millennium FoundationNorges ForskningsrådUniversité de LiègeChinese University of Hong KongCentre National de la Recherche ScientifiqueSeoul National UniversityUniversity of TorontoUniversity of UlsanBundesministerium für Bildung und ForschungŚląski Uniwersytet Medyczny w KatowicachMinistry of Education, Culture, Sports, Science and TechnologyNational and Kapodistrian University of AthensHallym UniversityDeutsche ForschungsgemeinschaftWellcome TrustUniversity College DublinQueen Elizabeth Hospital Birmingham CharityUniversity of ThessalyInstitut National de la Santé et de la Recherche MédicaleEU Joint Programme – Neurodegenerative Disease ResearchDuke-NUS Medical SchoolNational Institute of Environmental Health SciencesSeoul National University HospitalGriffith UniversityHelmholtz Zentrum MünchenMichael J. Fox Foundation for Parkinson's ResearchValeant Pharmaceuticals InternationalUniversiteit AntwerpenAlnylam PharmaceuticalsNovartis PharmaEuropean CommissionUniwersytet Śląski w KatowicachFritz Thyssen StiftungParkinson's UKNational Institutes of HealthUniversity Hospitals Birmingham NHS Foundation TrustInstituut Born-BungeMayo Clinic
KeywordsTrinucleotide repeat expansionSpinocerebellar ataxiaDiseaseGeneticsMachado–Joseph diseaseOdds ratioBiologyGeneMedicineInternal medicineAllele

Abstract

fetched live from OpenAlex

OBJECTIVES: We aim to clarify the pathogenic role of intermediate size repeat expansions of SCA2, SCA3, SCA6, and SCA17 as risk factors for idiopathic Parkinson disease (PD). METHODS: We invited researchers from the Genetic Epidemiology of Parkinson's Disease Consortium to participate in the study. There were 12,346 cases and 8,164 controls genotyped, for a total of 4 repeats within the SCA2, SCA3, SCA6, and SCA17 genes. Fixed- and random-effects models were used to estimate the summary risk estimates for the genes. We investigated between-study heterogeneity and heterogeneity between different ethnic populations. RESULTS: We did not observe any definite pathogenic repeat expansions for SCA2, SCA3, SCA6, and SCA17 genes in patients with idiopathic PD from Caucasian and Asian populations. Furthermore, overall analysis did not reveal any significant association between intermediate repeats and PD. The effect estimates (odds ratio) ranged from 0.93 to 1.01 in the overall cohort for the SCA2, SCA3, SCA6, and SCA17 loci. CONCLUSIONS: Our study did not support a major role for definite pathogenic repeat expansions in SCA2, SCA3, SCA6, and SCA17 genes for idiopathic PD. Thus, results of this large study do not support diagnostic screening of SCA2, SCA3, SCA6, and SCA17 gene repeats in the common idiopathic form of PD. Likewise, this largest multicentered study performed to date excludes the role of intermediate repeats of these genes as a risk factor for PD.

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)0.001
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 score0.417
Threshold uncertainty score0.619

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0000.001
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 head0.036
GPT teacher head0.309
Teacher spread0.273 · 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".

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Citations32
Published2015
Admission routes1
Has abstractyes

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