Idiopathic inflammatory myopathy human derived cells retain their ability to increase mitochondrial function

PloS One
Carla Basualto-AlarcónJ César Cárdenas

Abstract

Idiopathic Inflammatory Myopathies (IIMs) have been studied within the framework of autoimmune diseases where skeletal muscle appears to have a passive role in the illness. However, persiting weakness even after resolving inflammation raises questions about the role that skeletal muscle plays by itself in these diseases. "Non-immune mediated" hypotheses have arisen to consider inner skeletal muscle cell processes as trigger factors in the clinical manifestations of IIMs. Alterations in oxidative phosphorylation, ATP production, calcium handling, autophagy, endoplasmic reticulum stress, among others, have been proposed as alternative cellular pathophysiological mechanisms. In this study, we used skeletal muscle-derived cells, from healthy controls and IIM patients to determine mitochondrial function and mitochondrial ability to adapt to a metabolic stress when deprived of glucose. We hypothesized that mitochondria would be dysfunctional in IIM samples, which was partially true in normal glucose rich growing medium as determined by oxygen consumption rate. However, in the glucose-free and galactose supplemented condition, a medium that forced mitochondria to function, IIM cells increased their respiration, reaching values matchin...Continue Reading

References

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Jun 1, 1995·Annals of the Rheumatic Diseases·Y CamposJ J Gomez-Reino
Jul 1, 1998·Clinical Immunology and Immunopathology·D S Tews, H H Goebel
Jun 14, 2013·Skeletal Muscle·Sree RayavarapuKanneboyina Nagaraju
Jun 1, 2014·Current Rheumatology Reports·Li Alemo MuntersIngrid E Lundberg
Jun 13, 2015·Annals of the Rheumatic Diseases·Adam P LightfootRobert G Cooper
Apr 14, 2018·Nature Reviews. Rheumatology·Ingrid E LundbergVictoria P Werth

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Methods Mentioned

BETA
biopsies
imaging technique
biopsy
Assay
flow cytometry
FACS

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