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(Reference retrieved automatically from Web of Science through information on FAPESP grant and its corresponding number as mentioned in the publication by the authors.)

Changes in calsequestrin, TNF-alpha, TGF-beta and MyoD levels during the progression of skeletal muscle dystrophy in mdx mice: a comparative analysis of the quadriceps, diaphragm and intrinsic laryngeal muscles

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Author(s):
Maranhao, Juliana Barros [1] ; Moreira, Drielen de Oliveira [2] ; Mauricio, Adriana Fogagnolo [2] ; de Carvalho, Samara Camacari [2] ; Ferretti, Renato [3] ; Pereira, Juliano Alves [2] ; Santo Neto, Humberto [2] ; Marques, Maria Julia [2]
Total Authors: 8
Affiliation:
[1] Univ Pernambuco UPE, Recife, PE - Brazil
[2] Univ Estadual Campinas UNICAMP, Inst Biol, Dept Biol Estrut & Func, BR-13083970 Campinas, SP - Brazil
[3] Univ Estadual Paulista, Inst Biociencias Botucatu, Dept Anat, Botucatu, SP - Brazil
Total Affiliations: 3
Document type: Journal article
Source: International Journal of Experimental Pathology; v. 96, n. 5, p. 285-293, OCT 2015.
Web of Science Citations: 7
Abstract

In Duchenne muscular dystrophy (DMD), the search for new biomarkers to follow the evolution of the disease is of fundamental importance in the light of the evolving gene and pharmacological therapies. In addition to the lack of dystrophin, secondary events including changes in calcium levels, inflammation and fibrosis greatly contribute to DMD progression and the molecules involved in these events may represent potential biomarkers. In this study, we performed a comparative evaluation of the progression of dystrophy within muscles that are differently affected by dystrophy (diaphragm; DIA and quadriceps; QDR) or spared (intrinsic laryngeal muscles) using the mdx mice model of DMD. We assessed muscle levels of calsequestrin (calcium-related protein), tumour necrosis factor (TNF-alpha; pro-inflammatory cytokine), tumour growth factor (TGF-beta; pro-fibrotic factor) and MyoD (muscle proliferation) vs. histopathology at early (1 and 4 months of age) and late (9 months of age) stages of dystrophy. Fibrosis was the primary feature in the DIA of mdx mice (9 months: 32% fibrosis), which was greater than in the QDR (9 months: 0.6% fibrosis). Muscle regeneration was the primary feature in the QDR (9 months: 90% of centrally nucleated fibres areas vs. 33% in the DIA). The QDR expressed higher levels of calsequestrin than the DIA. Laryngeal muscles showed normal levels of TNF-alpha, TGF-beta and MyoD. A positive correlation between histopathology and cytokine levels was observed only in the diaphragm, suggesting that TNF-alpha and TGF-beta serve as markers of dystrophy primarily for the diaphragm. (AU)

FAPESP's process: 12/03498-7 - Mechanistic insights on the effects of suramin, on the cardiomyopathy of the mdx mice.
Grantee:Drielen de Oliveira Moreira
Support Opportunities: Scholarships in Brazil - Doctorate
FAPESP's process: 12/13577-1 - Biomarkers of cardiomyopathy in dystrophy: a metabolomic study and pharmacological therapy
Grantee:Adriana Fogagnolo Mauricio
Support Opportunities: Scholarships in Brazil - Doctorate
FAPESP's process: 12/15492-3 - Pharmacological therapy of the dystrophinopathies: fibrosis and muscular regeneration in mdx mice treated with omega-3
Grantee:Samara Camaçarí de Carvalho
Support Opportunities: Scholarships in Brazil - Doctorate
FAPESP's process: 14/04782-6 - Pre-clinical studies in the mdx mouse: metabolomics, biomarkers and omega-3 therapy
Grantee:Maria Julia Marques
Support Opportunities: Regular Research Grants