Distinctive serum miRNA profile in mouse models of striated muscular pathologies.
Biomarkers are critically important for disease diagnosis and monitoring. In particular, close monitoring of disease evolution is eminently required for the evaluation of therapeutic treatments. Classical monitoring methods in muscular dystrophies are largely based on histological and molecular anal...
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2013
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oai:doaj.org-article:5331d55271d5433a82a47bf22aaec1212021-11-18T07:57:51ZDistinctive serum miRNA profile in mouse models of striated muscular pathologies.1932-620310.1371/journal.pone.0055281https://doaj.org/article/5331d55271d5433a82a47bf22aaec1212013-01-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/pmid/23418438/?tool=EBIhttps://doaj.org/toc/1932-6203Biomarkers are critically important for disease diagnosis and monitoring. In particular, close monitoring of disease evolution is eminently required for the evaluation of therapeutic treatments. Classical monitoring methods in muscular dystrophies are largely based on histological and molecular analyses of muscle biopsies. Such biopsies are invasive and therefore difficult to obtain. The serum protein creatine kinase is a useful biomarker, which is however not specific for a given pathology and correlates poorly with the severity or course of the muscular pathology. The aim of the present study was the systematic evaluation of serum microRNAs (miRNAs) as biomarkers in striated muscle pathologies. Mouse models for five striated muscle pathologies were investigated: Duchenne muscular dystrophy (DMD), limb-girdle muscular dystrophy type 2D (LGMD2D), limb-girdle muscular dystrophy type 2C (LGMD2C), Emery-Dreifuss muscular dystrophy (EDMD) and hypertrophic cardiomyopathy (HCM). Two-step RT-qPCR methodology was elaborated, using two different RT-qPCR miRNA quantification technologies. We identified miRNA modulation in the serum of all the five mouse models. The most highly dysregulated serum miRNAs were found to be commonly upregulated in DMD, LGMD2D and LGMD2C mouse models, which all exhibit massive destruction of striated muscle tissues. Some of these miRNAs were down rather than upregulated in the EDMD mice, a model without massive myofiber destruction. The dysregulated miRNAs identified in the HCM model were different, with the exception of one dysregulated miRNA common to all pathologies. Importantly, a specific and distinctive circulating miRNA profile was identified for each studied pathological mouse model. The differential expression of a few dysregulated miRNAs in the DMD mice was further evaluated in DMD patients, providing new candidates of circulating miRNA biomarkers for DMD.Nicolas VignierFatima AmorPaul FogelAngélique DuvalletJérôme PoupiotSabine CharrierMichel ArockMarie MontusIsabelle NelsonIsabelle RichardLucie CarrierLaurent ServaisThomas VoitGisèle BonneDavid IsraeliPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 8, Iss 2, p e55281 (2013) |
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| topic |
Medicine R Science Q |
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Medicine R Science Q Nicolas Vignier Fatima Amor Paul Fogel Angélique Duvallet Jérôme Poupiot Sabine Charrier Michel Arock Marie Montus Isabelle Nelson Isabelle Richard Lucie Carrier Laurent Servais Thomas Voit Gisèle Bonne David Israeli Distinctive serum miRNA profile in mouse models of striated muscular pathologies. |
| description |
Biomarkers are critically important for disease diagnosis and monitoring. In particular, close monitoring of disease evolution is eminently required for the evaluation of therapeutic treatments. Classical monitoring methods in muscular dystrophies are largely based on histological and molecular analyses of muscle biopsies. Such biopsies are invasive and therefore difficult to obtain. The serum protein creatine kinase is a useful biomarker, which is however not specific for a given pathology and correlates poorly with the severity or course of the muscular pathology. The aim of the present study was the systematic evaluation of serum microRNAs (miRNAs) as biomarkers in striated muscle pathologies. Mouse models for five striated muscle pathologies were investigated: Duchenne muscular dystrophy (DMD), limb-girdle muscular dystrophy type 2D (LGMD2D), limb-girdle muscular dystrophy type 2C (LGMD2C), Emery-Dreifuss muscular dystrophy (EDMD) and hypertrophic cardiomyopathy (HCM). Two-step RT-qPCR methodology was elaborated, using two different RT-qPCR miRNA quantification technologies. We identified miRNA modulation in the serum of all the five mouse models. The most highly dysregulated serum miRNAs were found to be commonly upregulated in DMD, LGMD2D and LGMD2C mouse models, which all exhibit massive destruction of striated muscle tissues. Some of these miRNAs were down rather than upregulated in the EDMD mice, a model without massive myofiber destruction. The dysregulated miRNAs identified in the HCM model were different, with the exception of one dysregulated miRNA common to all pathologies. Importantly, a specific and distinctive circulating miRNA profile was identified for each studied pathological mouse model. The differential expression of a few dysregulated miRNAs in the DMD mice was further evaluated in DMD patients, providing new candidates of circulating miRNA biomarkers for DMD. |
| format |
article |
| author |
Nicolas Vignier Fatima Amor Paul Fogel Angélique Duvallet Jérôme Poupiot Sabine Charrier Michel Arock Marie Montus Isabelle Nelson Isabelle Richard Lucie Carrier Laurent Servais Thomas Voit Gisèle Bonne David Israeli |
| author_facet |
Nicolas Vignier Fatima Amor Paul Fogel Angélique Duvallet Jérôme Poupiot Sabine Charrier Michel Arock Marie Montus Isabelle Nelson Isabelle Richard Lucie Carrier Laurent Servais Thomas Voit Gisèle Bonne David Israeli |
| author_sort |
Nicolas Vignier |
| title |
Distinctive serum miRNA profile in mouse models of striated muscular pathologies. |
| title_short |
Distinctive serum miRNA profile in mouse models of striated muscular pathologies. |
| title_full |
Distinctive serum miRNA profile in mouse models of striated muscular pathologies. |
| title_fullStr |
Distinctive serum miRNA profile in mouse models of striated muscular pathologies. |
| title_full_unstemmed |
Distinctive serum miRNA profile in mouse models of striated muscular pathologies. |
| title_sort |
distinctive serum mirna profile in mouse models of striated muscular pathologies. |
| publisher |
Public Library of Science (PLoS) |
| publishDate |
2013 |
| url |
https://doaj.org/article/5331d55271d5433a82a47bf22aaec121 |
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