Cited by 5Muscle Physiology and DisordersAdipose Tissue and MetabolismMetabolism, Diabetes, and Cancer

Role of adiponectin in the metabolism of skeletal muscles in collagen VI-related myopathies

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Gamberi, Tania, Magherini, Francesca, Mannelli, Michele, Chrisam, Martina, Cescon, Matilde, Castagnaro, Silvia, Modesti, Alessandra, Braghetta, Paola +1 more·2019·Journal of Molecular Medicine

Abstract

The role of adiponectin has been particularly deepened in diabetic muscles while the study of adiponectin in hereditary myopathies has been marginally investigated. Here, we report the study about adiponectin effects in Col6a1-/- (collagen VI-null) mice. Col6a1-/- mice show myophatic phenotype closer to that of patients with Bethlem myopathy, thus representing an excellent animal model for the study of this hereditary disease. Our findings demonstrate that Col6a1-/- mice have decreased plasma adiponectin content and diseased myoblasts have an impaired autocrine secretion of the hormone. Moreover, Col6a1-/- myoblasts show decreased glucose uptake and mitochondria with depolarized membrane potential and impaired functionality, as supported by decreased oxygen consumption. Exogenous addition of globular adiponectin modifies the features of Col6a1-/- myoblasts, becoming closer to that of the healthy myoblasts. Indeed, globular adiponectin enhances glucose uptake in Col6a1-/- myoblasts, modifies mitochondrial membrane potential, and restores oxygen consumption, turning closer to those of wild-type myoblasts. Finally, increase of plasma adiponectin level in Col6a1-/- mice is induced by fasting, a condition that has been previously shown to lead to the amelioration of the dystrophic phenotype. Collectively, our results demonstrate that exogenous replenishment of adiponectin reverses metabolic abnormalities observed in Col6a1-/- myoblasts. KEY MESSAGES: Col6a1-/- mice have decreased level of plasma adiponectin. Myoblasts from Col6a1-/- muscles have impaired local adiponectin secretion. Col6a1-/- myoblasts reveal altered metabolic features. Addition of exogenous adiponectin ameliorates Col6a1-/- metabolic features.

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Authors
Gamberi, T., Magherini, F., Mannelli, M., Chrisam, M., Cescon, M., Castagnaro, S., Modesti, A., Braghetta, P., & Fiaschi, T.
Affiliations
  • University of Florence (IT)
  • University of Padua (IT)
Corresponding author
Fiaschi, Tania
Venue
Journal of Molecular Medicine
Published
2019-03-29
Volume
97
Issue
6
Pages
793-801
ISSN
  • 0946-2716 (ISSN-L)
  • 1432-1440
OpenAlex
W2932543090
Cited by
5
References
34
Citations by year
2023: 1 · 2021: 1 · 2020: 1 · 2019: 2
FWCI
0.33
Type
journal article
Language
en
Open access
Locations
  • Journal of Molecular Medicine (publishedVersion)
  • PubMed (publishedVersion)
  • Florence Research (University of Florence) (submittedVersion, cc-by-nc)
  • Padua Research Archive (University of Padova) (submittedVersion, other-oa)
Funders
Italian Ministry of University and Research
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Muscle Physiology and Disorders
  • Health Sciences › Medicine › Physiology › Adipose Tissue and Metabolism
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Metabolism, Diabetes, and Cancer
Keywords
adiponectin, collagen VI, Bethlem myopathy, skeletal muscle metabolism, myoblasts, glucose uptake, mitochondrial membrane potential, oxygen consumption, fasting, hereditary myopathy
MeSH terms
Animals, Fasting, Glucose, Male, Muscular Diseases, Oxygen Consumption, Muscle, Skeletal, Collagen Type VI, Myoblasts, Glucose Transporter Type 4, Mice, Adiponectin, Membrane Potential, Mitochondrial

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