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Record W1982701732 · doi:10.1212/wnl.0000000000001012

Global investigation and meta-analysis of the <i>C9orf72</i> (G <sub>4</sub> C <sub>2</sub> ) <sub>n</sub> repeat in Parkinson disease

2014· article· en· W1982701732 on OpenAlexfundno aff
Jessie Theuns, Aline Verstraeten, Kristel Sleegers, Eline Wauters, Ilse Gijselinck, Stefanie Smolders, David Crosiers, Ellen Corsmit, Ellen Elinck, Manu Sharma, Rejko Krüger, Suzanne Lesage, Alexis Brice, Sun Ju Chung, Mi‐Jung Kim, Young Jin Kim, Owen A. Ross, Zbigniew K. Wszołek, Ekaterina Rogaeva, Zhengrui Xi, Anthony E. Lang, Christine Klein, Anne Weißbach, George D. Mellick, Peter A. Silburn, Georgios M. Hadjigeorgiou, Efthimios Dardiotis, Nobutaka Hattori, Kotaro Ogaki, Eng‐King Tan, Yi Zhao, Jan Aasly, Enza Maria Valente, Simona Petrucci, Grazia Annesi, Aldo Quattrone, Carlo Ferrarese, Laura Brighina, Angela Deutschländer, Andreas Puschmann, Christer Nilsson, Gaëtan Garraux, Mark S. LeDoux, Ronald F. Pfeiffer, Magdalena Boczarska‐Jedynak, Grzegorz Opala, Demetrius M. Maraganore, Sebastiaan Engelborghs, Peter Paul De Deyn, Patrick Cras, Marc Cruts, Christine Van Broeckhoven

Bibliographic record

VenueNeurology · 2014
Typearticle
Languageen
FieldMedicine
TopicParkinson's Disease Mechanisms and Treatments
Canadian institutionsnot available
FundersNational Institute of Environmental Health SciencesNational Institute of Neurological Disorders and StrokeDepartment of Medicine, University of TorontoMedical Research CouncilHealth Science Center, University of TennesseeNational Institutes of HealthFondation Médicale Reine ElisabethUniwersytet Śląski w KatowicachVlaamse regeringŚląski Uniwersytet Medyczny w KatowicachUniversiteit AntwerpenBundesministerium für Bildung und ForschungUniversité Pierre et Marie CurieUniversity of TorontoParkinsonfondenFondation pour la Recherche sur AlzheimerMinistero della SaluteUniversità degli Studi di Milano-BicoccaFondation pour la Recherche MédicaleDeutsches Zentrum für Neurodegenerative ErkrankungenBelgian Federal Science Policy OfficeHermann und Lilly Schilling-Stiftung für Medizinische ForschungUniversity of UlsanInstituut Born-BungeMetLife FoundationGriffith UniversityAgence Nationale de la RechercheFonds Wetenschappelijk OnderzoekRijksuniversiteit GroningenSingapore General HospitalSkånes universitetssjukhusUniversitair Medisch Centrum GroningenEli Lilly and CompanyUniversity of ThessalyInstitut National de la Santé et de la Recherche MédicaleCentre National de la Recherche ScientifiqueNorges Teknisk-Naturvitenskapelige UniversitetNational Medical Research CouncilLunds UniversitetParkinson VerenigingMichael J. Fox Foundation for Parkinson's Research
KeywordsC9orf72Amyotrophic lateral sclerosisFrontotemporal dementiaParkinsonismDementiaTrinucleotide repeat expansionMedicineCohortDiseaseParkinson's diseasePopulationFrontotemporal lobar degenerationFamily historyPediatricsInternal medicineGeneticsBiologyAllele

Abstract

fetched live from OpenAlex

OBJECTIVES: The objective of this study is to clarify the role of (G4C2)n expansions in the etiology of Parkinson disease (PD) in the worldwide multicenter Genetic Epidemiology of Parkinson's Disease (GEO-PD) cohort. METHODS: C9orf72 (G4C2)n repeats were assessed in a GEO-PD cohort of 7,494 patients diagnosed with PD and 5,886 neurologically healthy control individuals ascertained in Europe, Asia, North America, and Australia. RESULTS: A pathogenic (G4C2)n>60 expansion was detected in only 4 patients with PD (4/7,232; 0.055%), all with a positive family history of neurodegenerative dementia, amyotrophic lateral sclerosis, or atypical parkinsonism, while no carriers were detected with typical sporadic or familial PD. Meta-analysis revealed a small increase in risk of PD with an increasing number of (G4C2)n repeats; however, we could not detect a robust association between the C9orf72 (G4C2)n repeat and PD, and the population attributable risk was low. CONCLUSIONS: Together, these findings indicate that expansions in C9orf72 do not have a major role in the pathogenesis of PD. Testing for C9orf72 repeat expansions should only be considered in patients with PD who have overt symptoms of frontotemporal lobar degeneration/amyotrophic lateral sclerosis or apparent family history of neurodegenerative dementia or motor neuron disease.

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)0.001
metaresearch head score (Gemma)0.000
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesMeta-epidemiology (narrow)
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: Observational
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.276
Threshold uncertainty score1.000

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0010.000
Meta-epidemiology (narrow)0.0010.000
Meta-epidemiology (broad)0.0020.001
Bibliometrics0.0000.001
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 head0.020
GPT teacher head0.243
Teacher spread0.223 · 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.

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

Citations63
Published2014
Admission routes1
Has abstractyes

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