bims-moremu Biomed News
on Molecular regulators of muscle mass
Issue of 2023–06–04
twenty papers selected by
Anna Vainshtein, Craft Science Inc.



  1. J Appl Physiol (1985). 2023 Jun 01.
      Duchenne muscular dystrophy (DMD) is a severe muscle wasting disease caused by mutations or deletions in the dystrophin gene, for which there remains no cure. As DMD patients also develop bone fragility because of muscle weakness and immobilization, better understanding the pathophysiological mechanisms of dystrophin deficiency will help develop therapies to improve musculoskeletal health. Since alterations in muscle phenotype can influence bone structure, we investigated whether modifying muscle contractile activity through low-frequency stimulation (LFS) could alter bone architecture in mouse models of DMD. We tested the hypothesis that increasing muscle contractile activity could influence bone mass and structure in dystrophin-deficient (mdx) and dystrophin- and utrophin-deficient (dko) dystrophic mice. Tibial bone structure in dko mice was significantly different from that in mdx and wild type (C57BL/10) control mice. Effects of LFS on bone architecture differed between dystrophic and healthy mice, with LFS thinning cortical bone in both dystrophic models. Bone mass was maintained in LFS-treated healthy mice, with a reduced proportion of high-density bone and concomitant increase in low-density bone. LFS-treated dko mice exhibited a net deficit in cortical thickness and reduced high-density bone, but no equivalent increase in low-density bone. These alterations in bone structure and mineral density reduced mechanical strength in mdx and dko mice. The findings reveal muscle activity can regulate bone mass, structure, mineral accrual, and strength, especially in the context of dystrophin and/or utrophin deficiency. The results provide unique insights into the development of bone fragility in DMD and for devising interventions to improve musculoskeletal health.
    Keywords:  bone; exercise; mdx; muscle contraction; muscular dystrophy
    DOI:  https://doi.org/10.1152/japplphysiol.00651.2022
  2. Front Aging. 2023 ;4 1171850
      Age-related loss of skeletal muscle mass leads to a reduction of strength. It is likely due to an inadequate stimulation of muscle protein synthesis (MPS) in response to anabolic stimuli, such as mechanical load. Ribosome biogenesis is a major determinant of translational capacity and is essential for the control of muscle mass. This mini-review aims to put forth the hypothesis that ribosome biogenesis is impaired by aging in response to mechanical load, which could contribute to the age-related anabolic resistance and progressive muscle atrophy. Recent animal studies indicate that aging impedes muscle hypertrophic response to mechanical overload. This is associated with an impaired transcription of ribosomal DNA (rDNA) by RNA polymerase I (Pol I), a limited increase in total RNA concentration, a blunted activation of AKT/mTOR pathway, and an increased phosphorylation of AMPK. In contrast, an age-mediated impairment of ribosome biogenesis is unlikely in response to electrical stimulations. In human, the hypertrophic response to resistance exercise training is diminished with age. This is accompanied by a deficit in long-term MPS and an absence of increased total RNA concentration. The results addressing the acute response to resistance exercise suggest an impaired Pol I-mediated rDNA transcription and attenuated activation/expression of several upstream regulators of ribosome biogenesis in muscles from aged individuals. Altogether, emerging evidence indicates that impaired ribosome biogenesis could partly explain age-related anabolic resistance to mechanical load, which may ultimately contribute to progressive muscle atrophy. Future research should develop more advanced molecular tools to provide in-depth analysis of muscle ribosome biogenesis.
    Keywords:  anabolic resistance; elderly; muscle atrophy; rDNA transcription; resistance exercise; sarcopenia; translational capacity
    DOI:  https://doi.org/10.3389/fragi.2023.1171850
  3. Aging (Albany NY). 2023 May 25. 15
      One of the most pronounced changes in the elderly is loss of strength and mobility due to the decline of skeletal muscle function, resulting in a multifactorial condition termed sarcopenia. Although significant clinical changes begin to manifest at advanced ages, recent studies have shown that changes at the cellular and molecular level precede the symptomatology of sarcopenia. By utilizing a single-cell transcriptomic atlas of mouse skeletal muscle across the lifespan, we identified a clear sign of immune senescence that presents during middle age. More importantly, the change in macrophage phenotype in middle age may explain the changes in extracellular matrix composition, especially collagen synthesis, that contributes to fibrosis and overall muscle weakness with advanced age. Our results show a novel paradigm whereby skeletal muscle dysfunction is driven by alterations in tissue-resident macrophages before the appearance of clinical symptoms in middle-aged mice, providing a new therapeutic approach via regulation of immunometabolism.
    Keywords:  aging; immunometabolism; macrophage; sarcopenia; scRNA-seq
    DOI:  https://doi.org/10.18632/aging.204750
  4. J Neurol. 2023 Jun 01.
      Duchenne muscular dystrophy (DMD) is a severe, progressive, muscle-wasting disease, characterized by progressive deterioration of skeletal muscle that causes rapid loss of mobility. The failure in respiratory and cardiac muscles is the underlying cause of premature death in most patients with DMD. Mutations in the gene encoding dystrophin result in dystrophin deficiency, which is the underlying pathogenesis of DMD. Dystrophin-deficient myocytes are dysfunctional and vulnerable to injury, triggering a series of subsequent pathological changes. In this review, we detail the molecular mechanism of DMD, dystrophin deficiency-induced muscle cell damage (oxidative stress injury, dysregulated calcium homeostasis, and sarcolemma instability) and other cell damage and dysfunction (neuromuscular junction impairment and abnormal differentiation of muscle satellite). We also describe aberrant function of other cells and impaired muscle regeneration due to deterioration of the muscle microenvironment, and dystrophin deficiency-induced multiple organ dysfunction, while summarizing the recent advances in the treatment of DMD.
    Keywords:  Duchenne muscular dystrophy; Dystrophin; Muscle atrophy; Therapies
    DOI:  https://doi.org/10.1007/s00415-023-11796-x
  5. J Biomech. 2023 May 16. pii: S0021-9290(23)00209-9. [Epub ahead of print]155 111640
      Skeletal muscle is the engine that powers what is arguably the most essential and defining feature of human and animal life-locomotion. Muscles function to change length and produce force to enable movement, posture, and balance. Despite this seemingly simple role, skeletal muscle displays a variety of phenomena that still remain poorly understood. These phenomena are complex-the result of interactions between active and passive machinery, as well as mechanical, chemical and electrical processes. The emergence of imaging technologies over the past several decades has led to considerable discoveries regarding how skeletal muscles function in vivo where activation levels are submaximal, and the length and velocity of contracting muscle fibres are transient. However, our knowledge of the mechanisms of muscle behaviour during everyday human movements remains far from complete. In this review, we discuss the principal advancements in imaging technology that have led to discoveries to improve our understanding of in vivo muscle function over the past 50 years. We highlight the knowledge that has emerged from the development and application of various techniques, including ultrasound imaging, magnetic resonance imaging, and elastography to characterise muscle design and mechanical properties. We emphasize that our inability to measure the forces produced by skeletal muscles still poses a significant challenge, and that future developments to accurately and reliably measure individual muscle forces will promote newfrontiers in biomechanics, physiology, motor control, and robotics. Finally, we identify critical gaps in our knowledge and future challenges that we hope can be solved as a biomechanics community in the next 50 years.
    Keywords:  Architecture; Fascicle; Locomotion; Magnetic resonance imaging; Pennation angle; Ultrasound
    DOI:  https://doi.org/10.1016/j.jbiomech.2023.111640
  6. Front Cell Dev Biol. 2023 ;11 1163427
      Introduction: Glycogen storage disease type III (GSDIII) is a rare genetic disease caused by mutations in the AGL gene encoding the glycogen debranching enzyme (GDE). The deficiency of this enzyme, involved in cytosolic glycogen degradation, leads to pathological glycogen accumulation in liver, skeletal muscles and heart. Although the disease manifests with hypoglycemia and liver metabolism impairment, the progressive myopathy is the major disease burden in adult GSDIII patients, without any curative treatment currently available. Methods: Here, we combined the self-renewal and differentiation capabilities of human induced pluripotent stem cells (hiPSCs) with cutting edge CRISPR/Cas9 gene editing technology to establish a stable AGL knockout cell line and to explore glycogen metabolism in GSDIII. Results: Following skeletal muscle cells differentiation of the edited and control hiPSC lines, our study reports that the insertion of a frameshift mutation in AGL gene results in the loss of GDE expression and persistent glycogen accumulation under glucose starvation conditions. Phenotypically, we demonstrated that the edited skeletal muscle cells faithfully recapitulate the phenotype of differentiated skeletal muscle cells of hiPSCs derived from a GSDIII patient. We also demonstrated that treatment with recombinant AAV vectors expressing the human GDE cleared the accumulated glycogen. Discussion: This study describes the first skeletal muscle cell model of GSDIII derived from hiPSCs and establishes a platform to study the mechanisms that contribute to muscle impairments in GSDIII and to assess the therapeutic potential of pharmacological inducers of glycogen degradation or gene therapy approaches.
    Keywords:  CRISPR/Cas9; glycogen storage disease; induced pluripotent stem cell; muscular disorders; skeletal muscle cell
    DOI:  https://doi.org/10.3389/fcell.2023.1163427
  7. J Pharmacol Sci. 2023 Jul;pii: S1347-8613(23)00030-0. [Epub ahead of print]152(3): 167-177
      Cisplatin, a platinum-based anticancer drug used frequently in cancer treatment, causes skeletal muscle atrophy. It was predicted that the proteolytic pathway is enhanced as the mechanism of this atrophy. Therefore, we investigated whether a platinum-based anticancer drug affects the expression of the major proteins of skeletal muscle, myosin heavy chain (MyHC). Mice were injected with cisplatin or oxaliplatin for four consecutive days. C2C12 myotubes were treated using cisplatin and oxaliplatin. Administration of platinum-based anticancer drug reduced quadriceps mass and muscle strength compared to the control group. Protein levels of all MyHC isoforms were reduced in the platinum-based anticancer drug groups. However, only Myh2 (MyHC-IIa) gene expression in skeletal muscle of mice treated with platinum-based anticancer drugs was found to be reduced. Treatment of C2C12 myotubes with platinum-based anticancer drugs reduced the protein levels of all MyHCs, and treatment with the proteasome inhibitor MG-132 restored this reduction. The expression of Mef2c, which was predicted to act upstream of Myh2, was reduced in the skeletal muscle of mice treated systemically with platinum-based anticancer drug. Degradation of skeletal muscle MyHCs by proteasomes may be a factor that plays an important role in muscle mass loss in platinum-based anticancer drug-induced muscle atrophy.
    Keywords:  Cisplatin; Muscle atrophy; Myh2; Myosin heavy chain; Oxaliplatin
    DOI:  https://doi.org/10.1016/j.jphs.2023.04.009
  8. Front Physiol. 2023 ;14 1165811
      Rationale: The anatomical substrate of skeletal muscle autonomic innervation has remained underappreciated since it was described many decades ago. As such, the structural and functional features of muscle sympathetic innervation are largely undetermined in both physiology and pathology, mainly due to methodological limitations in the histopathological analysis of small neuronal fibers in tissue samples. Amyotrophic lateral sclerosis (ALS) is a fatal neuromuscular disease which mainly targets motor neurons, and despite autonomic symptoms occurring in a significant fraction of patients, peripheral sympathetic neurons (SNs) are generally considered unaffected and, as such, poorly studied. Purpose: In this research, we compared sympathetic innervation of normal and ALS muscles, through structural analysis of the sympathetic network in human and murine tissue samples. Methods and Results: We first refined tissue processing to circumvent methodological limitations interfering with the detection of muscle sympathetic innervation. The optimized "Neuro Detection Protocol" (NDP) was validated in human muscle biopsies, demonstrating that SNs innervate, at high density, both blood vessels and skeletal myofibers, independent of the fiber metabolic type. Subsequently, NDP was exploited to analyze sympathetic innervation in muscles of SOD1G93A mice, a preclinical ALS model. Our data show that ALS murine muscles display SN denervation, which has already initiated at the early disease stage and worsened during aging. SN degeneration was also observed in muscles of MLC/SOD1G93A mice, with muscle specific expression of the SOD1G93A mutant gene. Notably, similar alterations in SNs were observed in muscle biopsies from an ALS patient, carrying the SOD1G93A mutation. Conclusion: We set up a protocol for the analysis of murine and, more importantly, human muscle sympathetic innervation. Our results indicate that SNs are additional cell types compromised in ALS and suggest that dysfunctional SOD1G93A muscles affect their sympathetic innervation.
    Keywords:  SOD1G93A mutation; amyotrophic lateral sclerosis; skeletal muscle innervation; sympathetic neurodegeneration; sympathetic neurons
    DOI:  https://doi.org/10.3389/fphys.2023.1165811
  9. Biochem Biophys Res Commun. 2023 May 20. pii: S0006-291X(23)00651-4. [Epub ahead of print]669 30-37
      Vestigial-like family member 3 (VGLL3) is a cofactor for the TEA-domain transcription factor (TEAD) family. Although VGLL3 influences myogenic differentiation, its involvement in slow- and fast-twitch fiber specification remains unknown. In this study, we established a cell line stably overexpressing VGLL3 and analyzed effects of VGLL3 on the myogenic differentiation of murine myoblast C2C12 cells. We found that VGLL3 expression promotes slow-twitch muscle differentiation. Mechanistically, VGLL3 expression induced the expression of peroxisome proliferator-activated receptor-γ coactivator-1α (PGC-1α), a master transcriptional regulator of slow-twitch muscle development. We also found that VGLL3 proteins are degraded by the proteasome, which causes switching of TEAD cofactors from VGLL3 to Yes-associated protein (YAP) and transcriptional coactivator with a PDZ-binding motif (TAZ). These results suggest that the balance between the two kinds of TEAD cofactors VGLL3 and YAP/TAZ controls muscle fiber-type specification.
    DOI:  https://doi.org/10.1016/j.bbrc.2023.05.073
  10. Nat Commun. 2023 May 27. 14(1): 3060
      Formation of oriented myofibrils is a key event in musculoskeletal development. However, the mechanisms that drive myocyte orientation and fusion to control muscle directionality in adults remain enigmatic. Here, we demonstrate that the developing skeleton instructs the directional outgrowth of skeletal muscle and other soft tissues during limb and facial morphogenesis in zebrafish and mouse. Time-lapse live imaging reveals that during early craniofacial development, myoblasts condense into round clusters corresponding to future muscle groups. These clusters undergo oriented stretch and alignment during embryonic growth. Genetic perturbation of cartilage patterning or size disrupts the directionality and number of myofibrils in vivo. Laser ablation of musculoskeletal attachment points reveals tension imposed by cartilage expansion on the forming myofibers. Application of continuous tension using artificial attachment points, or stretchable membrane substrates, is sufficient to drive polarization of myocyte populations in vitro. Overall, this work outlines a biomechanical guidance mechanism that is potentially useful for engineering functional skeletal muscle.
    DOI:  https://doi.org/10.1038/s41467-023-38647-7
  11. iScience. 2023 May 19. 26(5): 106592
      Myoblast determination protein 1 (MyoD) dynamics define the activation status of muscle stem cells (MuSCs), aiding in muscle tissue regeneration after injury. However, the lack of experimental platforms to monitor MyoD dynamics in vitro and in vivo has hampered the investigation of fate determination and heterogeneity of MuSCs. Herein, we report a MyoD knock-in (MyoD-KI) reporter mouse expressing tdTomato at the endogenous MyoD locus. Expression of tdTomato in MyoD-KI mice recapitulated the endogenous MyoD expression dynamics in vitro and during the early phase of regeneration in vivo. Additionally, we showed that tdTomato fluorescence intensity defines MuSC activation status without immunostaining. Based on these features, we developed a high-throughput screening system to assess the effects of drugs on the behavior of MuSCs in vitro. Thus, MyoD-KI mice are an invaluable resource for studying the dynamics of MuSCs, including their fate decisions and heterogeneity, and for drug screening in stem cell therapy.
    Keywords:  Cell biology; Molecular biology; Stem cells research
    DOI:  https://doi.org/10.1016/j.isci.2023.106592
  12. J Appl Physiol (1985). 2023 Jun 01.
      Heat exercise training may increase exercise performance in athletes. The underlying mechanisms remain partly unresolved, and it is unknown if female and male athletes may experience comparable gains. The aims were to investigate whether heat training (HEAT) increases hemoglobin mass (Hbmass), skeletal muscle fiber characteristics and thermoneutral exercise performance in elite female and male endurance athletes. Female (n=20; VO2max = 58.2 ± 6.7 ml.min.kg) and male (n=27; VO2max = 76.4 ± 7.8 ml.min.kg) cyclists were studied before and after five weeks of randomized control or HEAT training consisting of five weekly sessions each of 50 min duration which were included in their normal training regimes. Overall, the observed relative responses to HEAT were largely similar in female and male study participants. HEAT increased (P<0.05) Hbmass in female from 650 ± 77 to 675 ± 76 g (4.0 ± 1.6%) and from 1008 ± 155 to 1041 ± 147 g (3.5 ± 2.3%) in male. In contrast, skeletal muscle citrate synthase activity, fiber type distribution and capillary density remained unchanged with HEAT. Lactate threshold, VO2max and mean power output during 15 min all out testing were all enhanced (P<0.05) following HEAT in female and male study participants. In conclusion, five weeks of HEAT increases Hbmass in female and male elite cyclists and improves exercise performance in a thermoneutral environment. Based on this, heat training may be recommended to elite female and male athletes aiming to perform in a thermoneutral environment.
    Keywords:  exercise; gender; heat; performance
    DOI:  https://doi.org/10.1152/japplphysiol.00115.2023
  13. ACS Appl Mater Interfaces. 2023 May 31.
      Mechanical damages to skeletal muscles could be detrimental to the active work hours and lifestyle of athletes, mountaineers, and security personnel. In this regard, the slowness of conventional treatment strategies and drug-associated side effects greatly demand the design and development of novel biomaterials, which can rescue such mechanically damaged skeletal muscles. To accomplish this demand, we have developed a musculoresponsive polymer-carbon composite for assisting myotubular regeneration (MusCAMLR). The MusCAMLR is enforced to attain anisotropic muscle-like characteristics while incorporating a smartly passivated nanoscale carbon material in the PNIPAM gel under physiological conditions as a stimulus, which is not achieved by the pristine nanocarbon system. The MusCAMLR establishes a specific mechanical interaction with muscle cells, supports myotube regeneration, maintains excellent mechanical similarity with the myotube, and restores the structural integrity and biochemical parameters of mechanically damaged muscles in a delayed onset muscle soreness (DOMS) rat model within a short period of 72 h. Concisely, this study discloses the potential of smartly passivated nanocarbon in generating an advanced biomaterial system, MusCAMLR, from a regularly used polymeric hydrogel system. This engineered polymer-carbon composite reveals its possible potential to be used as a nondrug therapeutic alternative for rescuing mechanically damaged muscles and probably can be extended for therapy of various other diseases including muscular dystrophy.
    Keywords:  PNIPAM hydrogel; carbon nanoparticle; delayed onset muscle soreness; polymer−carbon composite; skeletal muscles
    DOI:  https://doi.org/10.1021/acsami.3c01889
  14. Bio Protoc. 2023 May 20. 13(10): e4678
      Skeletal muscle consists of a mixture of fiber types with different functional and metabolic characteristics. The relative composition of these muscle fiber types has implications for muscle performance, whole-body metabolism, and health. However, analyses of muscle samples in a fiber type-dependent manner are very time consuming. Therefore, these are often neglected in favor of more time-efficient analyses on mixed muscle samples. Methods such as western blot and myosin heavy chain separation by SDS-PAGE have previously been utilized to fiber type-isolated muscle fibers. More recently, the introduction of the dot blot method significantly increased the speed of fiber typing. However, despite recent advancements, none of the current methodologies are feasible for large-scale investigations because of their time requirements. Here, we present the protocol for a new method, which we have named THRIFTY (high-THRoughput Immunofluorescence Fiber TYping), that enables rapid fiber type identification using antibodies towards the different myosin heavy chain (MyHC) isoforms of fast and slow twitch muscle fibers. First, a short segment (<1 mm) is cut off from isolated muscle fibers and mounted on a customized gridded microscope slide holding up to 200 fiber segments. Second, the fiber segments attached to the microscope slide are stained with MyHC-specific antibodies and then visualized using a fluorescence microscope. Lastly, the remaining pieces of the fibers can either be collected individually or pooled together with fibers of the same type for subsequent analyses. The THRIFTY protocol is approximately three times as fast as the dot blot method, which enables not only time-sensitive assays to be performed but also increases the feasibility to conduct large-scale investigations into fiber type specific physiology. Graphical Overview Graphical overview of the THRIFTY workflow. Cut off a small segment (0.5 mm) of an individually dissected muscle fiber and mount it onto the customized microscope slide containing a printed grid system. Using a Hamilton syringe, fixate the fiber segment by applying a small droplet of distilled water on the segment and let it fully dry (1A). The remaining large segment of the fiber should be placed in the corresponding square on a black A4 paper (1B). Once the microscope slide has been fully mounted with fiber segments, submerge the slide in a polypropylene slide mailer (illustrated as a Coplin jar in the figure) containing acetone to permeabilize the fiber segments. Thereafter, incubate the slide with primary antibodies targeting MyHC-I and MyHC-II. Following washes in PBS solution, incubate the slides with fluorescently labeled secondary antibodies, wash again, and mount with a cover glass and antifade reagent (2). Identification of fiber type can be performed using a digital fluorescence microscope (3), whereafter the remaining pieces of the fiber segments (large) are pooled together according to their fiber type or individually collected for experiments on single fibers (4). Image modified from Horwath et al. (2022).
    Keywords:  Fiber type identification; Muscle; Muscle fiber type; Myosin heavy chain
    DOI:  https://doi.org/10.21769/BioProtoc.4678
  15. Redox Biol. 2023 May 20. pii: S2213-2317(23)00149-0. [Epub ahead of print]63 102748
      Exercise physiology has gained increasing interest due to its wide effects to promote health. Recent years have seen a growth in this research field also due to the finding of several circulating factors that mediate the effects of exercise. These factors, termed exerkines, are metabolites, growth factors, and cytokines secreted by main metabolic organs during exercise to regulate exercise systemic and tissue-specific effects. The metabolic effects of exerkines have been broadly explored and entail a promising target to modulate beneficial effects of exercise in health and disease. However, exerkines also have broad effects to modulate redox signaling and homeostasis in several cellular processes to improve stress response. Since redox biology is central to exercise physiology, this review summarizes current evidence for the cross-talk between redox biology and exerkines actions. The role of exerkines in redox biology entails a response to oxidative stress-induced pathological cues to improve health outcomes and to modulate exercise adaptations that integrate redox signaling.
    DOI:  https://doi.org/10.1016/j.redox.2023.102748
  16. J Clin Invest. 2023 Jun 01. pii: e168121. [Epub ahead of print]133(11):
      Exercise confers numerous salutary effects that extend beyond individual organ systems to provide systemic health benefits. Here, we discuss the role of exercise in cardiovascular health. We summarize major findings from human exercise studies in cardiometabolic disease. We next describe our current understanding of cardiac-specific substrate metabolism that occurs with acute exercise and in response to exercise training. We subsequently focus on exercise-stimulated circulating biochemicals ("exerkines") as a paradigm for understanding the global health circuitry of exercise, and discuss important concepts in this emerging field before highlighting exerkines relevant in cardiovascular health and disease. Finally, this Review identifies gaps that remain in the field of exercise science and opportunities that exist to translate biologic insights into human health improvement.
    DOI:  https://doi.org/10.1172/JCI168121
  17. Cell Rep. 2023 Jun 01. pii: S2211-1247(23)00599-5. [Epub ahead of print]42(6): 112588
      Physiology is regulated by interconnected cell and tissue circadian clocks. Disruption of the rhythms generated by the concerted activity of these clocks is associated with metabolic disease. Here we tested the interactions between clocks in two critical components of organismal metabolism, liver and skeletal muscle, by rescuing clock function either in each organ separately or in both organs simultaneously in otherwise clock-less mice. Experiments showed that individual clocks are partially sufficient for tissue glucose metabolism, yet the connections between both tissue clocks coupled to daily feeding rhythms support systemic glucose tolerance. This synergy relies in part on local transcriptional control of the glucose machinery, feeding-responsive signals such as insulin, and metabolic cycles that connect the muscle and liver. We posit that spatiotemporal mechanisms of muscle and liver play an essential role in the maintenance of systemic glucose homeostasis and that disrupting this diurnal coordination can contribute to metabolic disease.
    Keywords:  Bmal1; CP: Metabolism; autonomy; circadian rhythms; endocrinology; glucose; inter-organ crosstalk; liver; metabolism; muscle; systems biology
    DOI:  https://doi.org/10.1016/j.celrep.2023.112588
  18. Front Neurosci. 2023 ;17 1147437
      Sensory neurons embedded in muscle tissue that initiate pain sensations, i.e., nociceptors, are temporarily sensitized by inflammatory mediators during musculoskeletal trauma. These neurons transduce peripheral noxious stimuli into an electrical signal [i.e., an action potential (AP)] and, when sensitized, demonstrate lower activation thresholds and a heightened AP response. We still do not understand the relative contributions of the various transmembrane proteins and intracellular signaling processes that drive the inflammation-induced hyperexcitability of nociceptors. In this study, we used computational analysis to identify key proteins that could regulate the inflammation-induced increase in the magnitude of AP firing in mechanosensitive muscle nociceptors. First, we extended a previously validated model of a mechanosensitive mouse muscle nociceptor to incorporate two inflammation-activated G protein-coupled receptor (GPCR) signaling pathways and validated the model simulations of inflammation-induced nociceptor sensitization using literature data. Then, by performing global sensitivity analyses that simulated thousands of inflammation-induced nociceptor sensitization scenarios, we identified three ion channels and four molecular processes (from the 17 modeled transmembrane proteins and 28 intracellular signaling components) as potential regulators of the inflammation-induced increase in AP firing in response to mechanical forces. Moreover, we found that simulating single knockouts of transient receptor potential ankyrin 1 (TRPA1) and reducing the rates of Gαq-coupled receptor phosphorylation and Gαq subunit activation considerably altered the excitability of nociceptors (i.e., each modification increased or decreased the inflammation-induced fold change in the number of triggered APs compared to when all channels were present). These results suggest that altering the expression of TRPA1 or the concentration of intracellular Gαq might regulate the inflammation-induced increase in AP response of mechanosensitive muscle nociceptors.
    Keywords:  action potential; computational analysis; inflammation; ion channels; musculoskeletal pain; nociceptor; sensitization
    DOI:  https://doi.org/10.3389/fnins.2023.1147437
  19. PLoS Genet. 2023 Jun 02. 19(6): e1010781
      Four SIX homeoproteins display a combinatorial expression throughout embryonic developmental myogenesis and they modulate the expression of the myogenic regulatory factors. Here, we provide a deep characterization of their role in distinct mouse developmental territories. We showed, at the hypaxial level, that the Six1:Six4 double knockout (dKO) somitic precursor cells adopt a smooth muscle fate and lose their myogenic identity. At the epaxial level, we demonstrated by the analysis of Six quadruple KO (qKO) embryos, that SIX are required for fetal myogenesis, and for the maintenance of PAX7+ progenitor cells, which differentiated prematurely and are lost by the end of fetal development in qKO embryos. Finally, we showed that Six1 and Six2 are required to establish craniofacial myogenesis by controlling the expression of Myf5. We have thus described an unknown role for SIX proteins in the control of myogenesis at different embryonic levels and refined their involvement in the genetic cascades operating at the head level and in the genesis of myogenic stem cells.
    DOI:  https://doi.org/10.1371/journal.pgen.1010781
  20. Nat Commun. 2023 Jun 01. 14(1): 3169
      General anesthetics and neuromuscular blockers are used together during surgery to stabilize patients in an unconscious state. Anesthetics act mainly by potentiating inhibitory ion channels and inhibiting excitatory ion channels, with the net effect of dampening nervous system excitability. Neuromuscular blockers act by antagonizing nicotinic acetylcholine receptors at the motor endplate; these excitatory ligand-gated ion channels are also inhibited by general anesthetics. The mechanisms by which anesthetics and neuromuscular blockers inhibit nicotinic receptors are poorly understood but underlie safe and effective surgeries. Here we took a direct structural approach to define how a commonly used anesthetic and two neuromuscular blockers act on a muscle-type nicotinic receptor. We discover that the intravenous anesthetic etomidate binds at an intrasubunit site in the transmembrane domain and stabilizes a non-conducting, desensitized-like state of the channel. The depolarizing neuromuscular blocker succinylcholine also stabilizes a desensitized channel but does so through binding to the classical neurotransmitter site. Rocuronium binds in this same neurotransmitter site but locks the receptor in a resting, non-conducting state. Together, this study reveals a structural mechanism for how general anesthetics work on excitatory nicotinic receptors and further rationalizes clinical observations in how general anesthetics and neuromuscular blockers interact.
    DOI:  https://doi.org/10.1038/s41467-023-38827-5