Reduced mitochondrial respiration and increased calcium deposits in the EDL muscle, but not in soleus, from 12-week-old dystrophic mdx mice

Mitochondria play an important role in providing ATP for muscle contraction. Muscle physiology is compromised in Duchenne muscular dystrophy (DMD) and several studies have shown the involvement of bioenergetics. In this work we investigated the mitochondrial physiology in fibers from fast-twitch mus...

Full description

Saved in:
Bibliographic Details
Published in:Scientific reports 2019-02, Vol.9 (1), p.1986-1986, Article 1986
Main Authors: Gaglianone, Rhayanna B, Santos, Anderson Teixeira, Bloise, Flavia Fonseca, Ortiga-Carvalho, Tania Maria, Costa, Manoel Luis, Quirico-Santos, Thereza, da Silva, Wagner Seixas, Mermelstein, Claudia
Format: Article
Language:English
Subjects:
Bioenergetics
Calcium (mitochondrial)
Deposits
Duchenne's muscular dystrophy
Electron transport chain
Mitochondria
Muscle contraction
Oxygen consumption
Physiology
Respiration
Rodents
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Mitochondria play an important role in providing ATP for muscle contraction. Muscle physiology is compromised in Duchenne muscular dystrophy (DMD) and several studies have shown the involvement of bioenergetics. In this work we investigated the mitochondrial physiology in fibers from fast-twitch muscle (EDL) and slow-twitch muscle (soleus) in the mdx mouse model for DMD and in control C57BL/10J mice. In our study, multiple mitochondrial respiratory parameters were investigated in permeabilized muscle fibers from 12-week-old animals, a critical age where muscle regeneration is observed in the mdx mouse. Using substrates of complex I and complex II from the electron transport chain, ADP and mitochondrial inhibitors, we found in the mdx EDL, but not in the mdx soleus, a reduction in coupled respiration suggesting that ATP synthesis is affected. In addition, the oxygen consumption after addition of complex II substrate is reduced in mdx EDL; the maximal consumption rate (measured in the presence of uncoupler) also seems to be reduced. Mitochondria are involved in calcium regulation and we observed, using alizarin stain, calcium deposits in mdx muscles but not in control muscles. Interestingly, more calcium deposits were found in mdx EDL than in mdx soleus. These data provide evidence that in 12-week-old mdx mice, calcium is accumulated and mitochondrial function is disturbed in the fast-twitch muscle EDL, but not in the slow-twitch muscle soleus.
ISSN:2045-2322
2045-2322
DOI:10.1038/s41598-019-38609-4