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Related Concept Videos

Muscle Recovery and Fatigue01:24

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Muscle fatigue refers to the decline in a muscle's ability to maintain the force of contraction after prolonged activity. It primarily stems from changes within muscle fibers. Even before experiencing muscle fatigue, one may feel tired and have the urge to stop the activity. This response, known as central fatigue, occurs due to changes in the central nervous system, namely the brain and spinal cord. While there is no single mechanism that induces fatigue, it may serve as a protective...
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Modification of secretory and transmembrane proteins entering the rough ER begins in the ER lumen. These modifications aid in protein folding and stabilize the acquired tertiary structure. Protein modifications in the rough ER co-occur at different stages of protein folding.
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The ER is the hub of protein synthesis in a cell. It has robust systems to quality control protein folding and also for degradation of terminally misfolded proteins. Under normal conditions, a small proportion of misfolded proteins that cannot be salvaged need to be transported to the cytoplasm by the ER-associated degradation or ERAD pathways. However, if the ERAD cannot handle the misfolded proteins, the cell activates the unfolded protein response or UPR to adjust the protein folding...
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The mitochondrial electron transport chain (ETC) is the main energy generation system in the eukaryotic cells. However, mitochondria also produce cytotoxic reactive oxygen species (ROS) due to the large electron flow during oxidative phosphorylation. While Complex I is one of the primary sources of superoxide radicals, ROS production by Complex II is uncommon and may only be observed in cancer cells with mutated complexes.
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Endoplasmic Reticulum Oxidative Stress Triggers Tgf-Beta-Dependent Muscle Dysfunction by Accelerating Ascorbic Acid

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SEPN1 myopathies stem from endoplasmic reticulum (ER) stress and excessive TGF-beta signaling. Restoring TGF-beta balance and managing ascorbic acid levels may treat these skeletal muscle diseases.

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Area of Science:

  • Biochemistry
  • Molecular Biology
  • Cellular Biology

Background:

  • Endoplasmic reticulum (ER) stress and oxidative stress are interconnected, impacting cellular metabolism.
  • Skeletal muscle diseases are often linked to oxidative stress, necessitating investigation into ER-related pathways.

Purpose of the Study:

  • To elucidate the molecular mechanisms connecting ER hyperoxidation, oxidative stress, and skeletal muscle dysfunction.
  • To explore the role of selenoprotein N (SEPN1) and endoplasmic oxidoreductin 1 (ERO1) in SEPN1-related myopathies.

Main Methods:

  • Utilized exon expression arrays to analyze gene expression in response to SEPN1 and ERO1 modulation.
  • Investigated the impact of ascorbic acid turnover and transforming growth factor-beta (TGF-beta) signaling in SEPN1 mutant mice.
  • Assessed the therapeutic potential of TGF-beta antagonism in a mouse model.

Main Results:

  • Genetic modulation of SEPN1 and ERO1 induced an SEPN1-related myopathic phenotype.
  • Excessive TGF-beta signaling, driven by accelerated ascorbic acid turnover, was identified as a key factor.
  • Limited ascorbic acid intake exacerbated myopathy in SEPN1 mutant mice, while TGF-beta antagonism restored muscle function.

Conclusions:

  • The study reveals a novel molecular mechanism for SEPN1-related myopathies involving the TGF-beta/ERO1/ascorbic acid axis.
  • Targeting this axis presents a potential therapeutic strategy for SEPN1-related skeletal muscle diseases.