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Record W4239446863 · doi:10.1080/21678421.2017.1368577

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2017· article· en· W4239446863 on OpenAlexfundno aff

Bibliographic record

VenueAmyotrophic Lateral Sclerosis and Frontotemporal Degeneration · 2017
Typearticle
Languageen
FieldEconomics, Econometrics and Finance
TopicDiverse Scientific and Economic Studies
Canadian institutionsnot available
FundersNational Health and Medical Research CouncilMedical Research CouncilNatural Sciences and Engineering Research Council of CanadaNational Institutes of HealthGerman Network for Motor Neuron DiseasesFondation CharcotVetenskapsrådetWellcome TrustAgence Nationale de la RechercheLudwig-Maximilians-Universität MünchenDeutsche ForschungsgemeinschaftMotor Neurone Disease Research Institute of AustraliaCytokineticsBundesministerium für Bildung und ForschungRoyal Brisbane and Women's Hospital FoundationCanadian Institutes of Health ResearchWeston Brain InstituteAssociation pour la Recherche sur la Sclérose Latérale Amyotrophique et autres Maladies du MotoneuroneTarget ALSEU Joint Programme – Neurodegenerative Disease ResearchMotor Neurone Disease AssociationMuscular Dystrophy Association
KeywordsGeography

Abstract

fetched live from OpenAlex

Since Charcot's description in 1869 and naming in 1874, ALS has been the coordinate degeneration of both corticospinal ('upper') motor neurons (CSMN; their axons in the lateral CST were observed as sclerotic, thus L[ateral] S[clerosis]) and spinal ('lower') motor neurons (SMN; 'anterior horn cells') that define ALS.Though not as absolutely purely motor system as long thought, before improved lifespan support enabled identification of cognitive/other involvement in ALS, these two neuron subtypes are still the defining and core, selectively vulnerable subtypes.Thus, it is critical to elucidate why these two quite distinct populations degenerate coordinately, in cortex and spinal cord, though developmentally born from distinct progenitor domains, with different neurotransmitter systems, synaptic types, and surrounding interneuron and astroglial types.Why do variants in genes expressed in every neuron type, including hundreds-thousands in cortex, cause disease risk, with (relatively) selective vulnerability?What is common with subtypes involved in FTD that might clarify shared vulnerabilities with ALS?Why do involved neurons function so well in people who later develop ALS (eg Lou Gehrig), indicating lack of early dysgenesis?Might complexity of evolutionary advancement in primate-human corticospinal system partially explain fragility/selective vulnerability of component neurons?Length and/or metabolic demands alone cannot-sensory DRG neurons and many cortical projection neurons are similarly long, yet not similarly involved.Bulbar ALS affects shorter neurons rather than longer CSMN.Some SMN subtypes survive.

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 machine prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.

metaresearch head score (Codex)0.002
metaresearch head score (Gemma)0.003
Version: metacan-v3-hybrid-931329e0061cValidation status: machine_predicted_unvalidated
Candidate categoriesInsufficient payload (model declined to judge)
Consensus categoriesInsufficient payload (model declined to judge)
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Not applicable · Consensus signal: Not applicable
GenreCandidate signal: Other · Consensus signal: Other
Teacher disagreement score0.108
Threshold uncertainty score0.154

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0020.003
Meta-epidemiology (narrow)0.0020.001
Meta-epidemiology (broad)0.0010.001
Bibliometrics0.0020.001
Science and technology studies0.0020.000
Scholarly communication0.0050.002
Open science0.0020.005
Research integrity0.0040.003
Insufficient payload (model declined to judge)0.8920.866

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 head0.083
GPT teacher head0.225
Teacher spread0.141 · 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; the direct Gemma label and the distilled Codex classifier agree on what is shown here.

Study designNot applicable
Domainnot available
GenreOther

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
Published2017
Admission routes1
Has abstractno

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