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UPRmt activation improves pathological alterations in cellular models of mitochondrial diseases

Overview of attention for article published in Orphanet Journal of Rare Diseases, May 2022
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  • In the top 25% of all research outputs scored by Altmetric
  • High Attention Score compared to outputs of the same age (85th percentile)
  • High Attention Score compared to outputs of the same age and source (92nd percentile)

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Title
UPRmt activation improves pathological alterations in cellular models of mitochondrial diseases
Published in
Orphanet Journal of Rare Diseases, May 2022
DOI 10.1186/s13023-022-02331-8
Pubmed ID
Authors

Juan M. Suárez-Rivero, Carmen J. Pastor-Maldonado, Suleva Povea-Cabello, Mónica Álvarez-Córdoba, Irene Villalón-García, Marta Talaverón-Rey, Alejandra Suárez-Carrillo, Manuel Munuera-Cabeza, Diana Reche-López, Paula Cilleros-Holgado, Rocío Piñero-Perez, José A. Sánchez-Alcázar

Abstract

Mitochondrial diseases represent one of the most common groups of genetic diseases. With a prevalence greater than 1 in 5000 adults, such diseases still lack effective treatment. Current therapies are purely palliative and, in most cases, insufficient. Novel approaches to compensate and, if possible, revert mitochondrial dysfunction must be developed. In this study, we tackled the issue using as a model fibroblasts from a patient bearing a mutation in the GFM1 gene, which is involved in mitochondrial protein synthesis. Mutant GFM1 fibroblasts could not survive in galactose restrictive medium for more than 3 days, making them the perfect screening platform to test several compounds. Tetracycline enabled mutant GFM1 fibroblasts survival under nutritional stress. Here we demonstrate that tetracycline upregulates the mitochondrial Unfolded Protein Response (UPRmt), a compensatory pathway regulating mitochondrial proteostasis. We additionally report that activation of UPRmt improves mutant GFM1 cellular bioenergetics and partially restores mitochondrial protein expression. Overall, we provide compelling evidence to propose the activation of intrinsic cellular compensatory mechanisms as promising therapeutic strategy for mitochondrial diseases.

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Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 16 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 16 100%

Demographic breakdown

Readers by professional status Count As %
Researcher 6 38%
Other 2 13%
Student > Bachelor 1 6%
Student > Doctoral Student 1 6%
Student > Master 1 6%
Other 1 6%
Unknown 4 25%
Readers by discipline Count As %
Biochemistry, Genetics and Molecular Biology 5 31%
Neuroscience 3 19%
Medicine and Dentistry 2 13%
Pharmacology, Toxicology and Pharmaceutical Science 1 6%
Nursing and Health Professions 1 6%
Other 0 0%
Unknown 4 25%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 11. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 28 May 2022.
All research outputs
#2,852,658
of 23,312,088 outputs
Outputs from Orphanet Journal of Rare Diseases
#387
of 2,673 outputs
Outputs of similar age
#62,914
of 441,851 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#8
of 101 outputs
Altmetric has tracked 23,312,088 research outputs across all sources so far. Compared to these this one has done well and is in the 87th percentile: it's in the top 25% of all research outputs ever tracked by Altmetric.
So far Altmetric has tracked 2,673 research outputs from this source. They typically receive more attention than average, with a mean Attention Score of 7.7. This one has done well, scoring higher than 85% of its peers.
Older research outputs will score higher simply because they've had more time to accumulate mentions. To account for age we can compare this Altmetric Attention Score to the 441,851 tracked outputs that were published within six weeks on either side of this one in any source. This one has done well, scoring higher than 85% of its contemporaries.
We're also able to compare this research output to 101 others from the same source and published within six weeks on either side of this one. This one has done particularly well, scoring higher than 92% of its contemporaries.