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Record W2960739547 · doi:10.1038/s41598-019-46639-1

Bioactive Thymosin Alpha-1 Does Not Influence F508del-CFTR Maturation and Activity

2019· article· en· W2960739547 on OpenAlexafffund
Andrea Armirotti, Valeria Tomati, Elizabeth Matthes, Guido Veit, Deborah M. Cholon, Puay‐Wah Phuan, Clarissa Braccia, Daniela Guidone, Martina Gentzsch, Gergely L. Lukács, A. S. Verkman, Luis J. V. Galietta, John W. Hanrahan, Nicoletta Pedemonte

Bibliographic record

VenueScientific Reports · 2019
Typearticle
Languageen
FieldMedicine
TopicCystic Fibrosis Research Advances
Canadian institutionsMcGill University
FundersNational Institute of Diabetes and Digestive and Kidney DiseasesAgenzia Italiana del Farmaco, Ministero della SaluteCanadian Institutes of Health ResearchUniversity of California, San FranciscoUniversity of North Carolina at Chapel HillFondazione per la Ricerca sulla Fibrosi CisticaMinistero della SaluteCystic Fibrosis CanadaMcGill UniversityCystic Fibrosis Foundation TherapeuticsCystic Fibrosis FoundationFondazione TelethonE-RareU.S. Department of Health and Human ServicesNational Institutes of HealthGovernment of Canada
KeywordsCysteamineCystic fibrosis transmembrane conductance regulatorCystic fibrosisAutophagyThymosinMutantChemistryMedicineBiochemistryPharmacologyCell biologyBiologyInternal medicine

Abstract

fetched live from OpenAlex

Deletion of phenylalanine 508 (F508del) in the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel is the most frequent mutation causing cystic fibrosis (CF). F508del-CFTR is misfolded and prematurely degraded. Recently thymosin a-1 (Tα-1) was proposed as a single molecule-based therapy for CF, improving both F508del-CFTR maturation and function by restoring defective autophagy. However, three independent laboratories failed to reproduce these results. Lack of reproducibility has been ascribed by the authors of the original paper to the use of DMSO and to improper handling. Here, we address these potential issues by demonstrating that Tα-1 changes induced by DMSO are fully reversible and that Tα-1 peptides prepared from different stock solutions have equivalent biological activity. Considering the negative results here reported, six independent laboratories failed to demonstrate F508del-CFTR correction by Tα-1. This study also calls into question the autophagy modulator cysteamine, since no rescue of mutant CFTR function was detected following treatment with cysteamine, while deleterious effects were observed when bronchial epithelia were exposed to cysteamine plus the antioxidant food supplement EGCG. Although these studies do not exclude the possibility of beneficial immunomodulatory effects of thymosin α-1, they do not support its utility as a corrector of F508del-CFTR.

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 score0.002
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 integrity0.0000.000
Insufficient payload (model declined to judge)0.0020.000

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 head0.010
GPT teacher head0.294
Teacher spread0.284 · 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

Citations8
Published2019
Admission routes2
Has abstractyes

Explore more

Same venueScientific Reports→Same topicCystic Fibrosis Research Advances→French-language works237,207→