Hypoxia and Hypoxia-Inducible Factor Signaling in Muscular Dystrophies: Cause and Consequences

Nguyen, Thuy-Hang; Conotte, Stephanie; Belayew, Alexandra; Declèves, Anne-Emilie; Legrand, Alexandre; Tassin, Alexandra

doi:10.3390/ijms22137220

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Hypoxia and Hypoxia-Inducible Factor Signaling in Muscular Dystrophies: Cause and Consequences

by

Thuy-Hang Nguyen

¹,

Stephanie Conotte

¹,

Alexandra Belayew

¹

,

Anne-Emilie Declèves

²

,

Alexandre Legrand

¹ and

Alexandra Tassin

^1,*

¹

Laboratory of Respiratory Physiology, Pathophysiology and Rehabilitation, Research Institute for Health Sciences and Technology, University of Mons, 7000 Mons, Belgium

²

Department of Metabolic and Molecular Biochemistry, Research Institute for Health Sciences and Technology, University of Mons, 7000 Mons, Belgium

^*

Author to whom correspondence should be addressed.

Int. J. Mol. Sci. 2021, 22(13), 7220; https://doi.org/10.3390/ijms22137220

Submission received: 31 May 2021 / Revised: 28 June 2021 / Accepted: 30 June 2021 / Published: 5 July 2021

(This article belongs to the Special Issue Hypoxia Signaling in Human Diseases)

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Abstract

Muscular dystrophies (MDs) are a group of inherited degenerative muscle disorders characterized by a progressive skeletal muscle wasting. Respiratory impairments and subsequent hypoxemia are encountered in a significant subgroup of patients in almost all MD forms. In response to hypoxic stress, compensatory mechanisms are activated especially through Hypoxia-Inducible Factor 1 α (HIF-1α). In healthy muscle, hypoxia and HIF-1α activation are known to affect oxidative stress balance and metabolism. Recent evidence has also highlighted HIF-1α as a regulator of myogenesis and satellite cell function. However, the impact of HIF-1α pathway modifications in MDs remains to be investigated. Multifactorial pathological mechanisms could lead to HIF-1α activation in patient skeletal muscles. In addition to the genetic defect per se, respiratory failure or blood vessel alterations could modify hypoxia response pathways. Here, we will discuss the current knowledge about the hypoxia response pathway alterations in MDs and address whether such changes could influence MD pathophysiology.

Keywords: hypoxia; myopathies; HIF-1α

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MDPI and ACS Style

Nguyen, T.-H.; Conotte, S.; Belayew, A.; Declèves, A.-E.; Legrand, A.; Tassin, A. Hypoxia and Hypoxia-Inducible Factor Signaling in Muscular Dystrophies: Cause and Consequences. Int. J. Mol. Sci. 2021, 22, 7220. https://doi.org/10.3390/ijms22137220

AMA Style

Nguyen T-H, Conotte S, Belayew A, Declèves A-E, Legrand A, Tassin A. Hypoxia and Hypoxia-Inducible Factor Signaling in Muscular Dystrophies: Cause and Consequences. International Journal of Molecular Sciences. 2021; 22(13):7220. https://doi.org/10.3390/ijms22137220

Chicago/Turabian Style

Nguyen, Thuy-Hang, Stephanie Conotte, Alexandra Belayew, Anne-Emilie Declèves, Alexandre Legrand, and Alexandra Tassin. 2021. "Hypoxia and Hypoxia-Inducible Factor Signaling in Muscular Dystrophies: Cause and Consequences" International Journal of Molecular Sciences 22, no. 13: 7220. https://doi.org/10.3390/ijms22137220

APA Style

Nguyen, T.-H., Conotte, S., Belayew, A., Declèves, A.-E., Legrand, A., & Tassin, A. (2021). Hypoxia and Hypoxia-Inducible Factor Signaling in Muscular Dystrophies: Cause and Consequences. International Journal of Molecular Sciences, 22(13), 7220. https://doi.org/10.3390/ijms22137220

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Hypoxia and Hypoxia-Inducible Factor Signaling in Muscular Dystrophies: Cause and Consequences

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