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Record W2811189142 · doi:10.7554/elife.36354

Nanoscale dysregulation of collagen structure-function disrupts mechano-homeostasis and mediates pulmonary fibrosis

2018· article· en· W2811189142 on OpenAlexafffund
Mark G. Jones, Orestis G. Andriotis, James Roberts, Kerry Lunn, Victoria Tear, Lucy Cao, Kjetil Ask, David E. Smart, Alessandra Bonfanti, Peter Johnson, Aiman Alzetani, Franco Conforti, Regan Doherty, Chester Lai, Benjamin C. Johnson, Konstantinos N. Bourdakos, Sophie Fletcher, Ben G. Marshall, Sanjay Jogai, Christopher J. Brereton, Serena Chee, Christian H. Ottensmeier, Patricia J. Sime, Jack Gauldie, Martin Kolb, Sumeet Mahajan, Aurélie Fabre, Atul Bhaskar, Wolfgang Jarolimek, Luca Richeldi, Katherine Ma O’Reilly, Phillip Monk, Philipp J. Thurner, Donna E. Davies

Bibliographic record

VenueeLife · 2018
Typearticle
Languageen
FieldBiochemistry, Genetics and Molecular Biology
TopicMicrobial metabolism and enzyme function
Canadian institutionsMcMaster UniversitySt. Joseph’s Healthcare Hamilton
FundersH2020 European Research CouncilMedical Research FoundationMedical Research CouncilWellcomeBritish Lung FoundationEngineering and Physical Sciences Research CouncilCanadian Institutes of Health ResearchNational Institute for Health and Care ResearchUniversity of AberdeenUniversity Hospital Southampton NHS Foundation TrustWellcome Trust
KeywordsExtracellular matrixFibrosisHomeostasisLysyl oxidaseCell biologyMechanosensitive channelsChemistryElastinCollagen VIFibrillogenesisPulmonary fibrosisPathologyBiologyMedicineFibrilBiochemistry

Abstract

fetched live from OpenAlex

Matrix stiffening with downstream activation of mechanosensitive pathways is strongly implicated in progressive fibrosis; however, pathologic changes in extracellular matrix (ECM) that initiate mechano-homeostasis dysregulation are not defined in human disease. By integrated multiscale biomechanical and biological analyses of idiopathic pulmonary fibrosis lung tissue, we identify that increased tissue stiffness is a function of dysregulated post-translational collagen cross-linking rather than any collagen concentration increase whilst at the nanometre-scale collagen fibrils are structurally and functionally abnormal with increased stiffness, reduced swelling ratio, and reduced diameter. In ex vivo and animal models of lung fibrosis, dual inhibition of lysyl oxidase-like (LOXL) 2 and LOXL3 was sufficient to normalise collagen fibrillogenesis, reduce tissue stiffness, and improve lung function in vivo. Thus, in human fibrosis, altered collagen architecture is a key determinant of abnormal ECM structure-function, and inhibition of pyridinoline cross-linking can maintain mechano-homeostasis to limit the self-sustaining effects of ECM on progressive fibrosis.

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)0.000
metaresearch head score (Gemma)0.000
Version: metacan-v3-hybrid-931329e0061cValidation 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 score0.001
Threshold uncertainty score0.005

Distilled classifier scores by category (both heads)

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 integrity0.0000.001
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 head0.005
GPT teacher head0.211
Teacher spread0.206 · 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 source (direct Gemma or distilled Codex), not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designBench or experimental
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

Citations170
Published2018
Admission routes2
Has abstractyes

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