Evidence map›Paper›PMID 36979906›Full record

ArticleBiomedicines2023

Mechanotransduction Impairment in Primary Fibroblast Model of Krabbe Disease.

Roberta Mezzena, Ambra Del Grosso, Roberto Maria Pellegrino, Husam B R Alabed, Carla Emiliani, Ilaria Tonazzini, Marco Cecchini

Open access · goldFull text read
In one paragraph

Article in Biomedicines, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.

0numbers the graph read from it
0cells of the map it votes in
3citing papers in PubMed
1.1field-weighted citation impact, top 25% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

3 citing papers in PubMed, 6 citations in OpenAlex.

  1. Article
  2. Article
  3. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

7 authors at 2 institutions in 1 country.

Roberta MezzenaNEST, Istituto Nanoscienze-CNR and Scuola Normale Superiore, Piazza San Silvestro 12, 56127 Pisa, Italy.ORCID 0000-0002-3886-8230
Ambra Del GrossoNEST, Istituto Nanoscienze-CNR and Scuola Normale Superiore, Piazza San Silvestro 12, 56127 Pisa, Italy.ORCID 0000-0002-1838-9064
Roberto Maria PellegrinoDepartment of Chemistry, Biology, and Biotechnologies, University of Perugia, 06123 Perugia, Italy.ORCID 0000-0001-5594-383X
Husam B R AlabedDepartment of Chemistry, Biology, and Biotechnologies, University of Perugia, 06123 Perugia, Italy.ORCID 0000-0002-9229-088X
Carla EmilianiDepartment of Chemistry, Biology, and Biotechnologies, University of Perugia, 06123 Perugia, Italy.
Ilaria TonazziniNEST, Istituto Nanoscienze-CNR and Scuola Normale Superiore, Piazza San Silvestro 12, 56127 Pisa, Italy.ORCID 0000-0002-7323-2174
Marco CecchiniNEST, Istituto Nanoscienze-CNR and Scuola Normale Superiore, Piazza San Silvestro 12, 56127 Pisa, Italy.ORCID 0000-0002-9688-2356
Scuola Normale Superiore · ITUniversity of Perugia · IT

Funding

European Leukodystrophy Association 2018-008F2
6 · The paper itself

Abstract

Krabbe disease (KD) is a genetic disorder caused by the absence of the galactosylceramidase (GALC) functional enzyme. No cure is currently available. Here, we investigate the mechanotransduction process in primary fibroblasts collected from the twitcher mouse, a natural KD murine model. Thanks to mechanotransduction, cells can sense their environment and convert external mechanical stimuli into biochemical signals that result in intracellular changes. In GALC-deficient fibroblasts, we show that focal adhesions (FAs), the protein clusters necessary to adhere and migrate, are increased, and that single-cell migration and wound healing are impaired. We also investigate the involvement of the autophagic process in this framework. We show a dysregulation in the FA turnover: here, the treatment with the autophagy activator rapamycin boosts cell migration and improves the clearance of FAs in GALC-deficient fibroblasts. We propose mechanosensing impairment as a novel potential pathological mechanism in twitcher fibroblasts, and more in general in Krabbe disease.

Indexed as

autophagyfocal adhesionsKrabbe diseasemechanotransductionmigration

Identifiers

PMID36979906
PMCPMC10046230
OpenAlexW4327740626

What OpenQuestion holds

Textfull text, public
LicenceCC BY
measurements read28
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.