دورية أكاديمية
Loss of Calpain 3 dysregulates store-operated calcium entry and its exercise response in mice.
العنوان: | Loss of Calpain 3 dysregulates store-operated calcium entry and its exercise response in mice. |
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المؤلفون: | Villani KR; Department of Applied Physiology and Kinesiology, College of Health and Human Performance, University of Florida, Gainesville, Florida, USA., Zhong R; Department of Applied Physiology and Kinesiology, College of Health and Human Performance, University of Florida, Gainesville, Florida, USA.; Department of Emergency Medicine, the First Affiliated Hospital of China Medical University, Shenyang, Liaoning, China., Henley-Beasley CS; Department of Applied Physiology and Kinesiology, College of Health and Human Performance, University of Florida, Gainesville, Florida, USA.; Myology Institute, University of Florida, Gainesville, Florida, USA., Rastelli G; Center for Advanced Studies and Technology, University G. d'Annunzio of Chieti-Pescara, Chieti, Italy.; Department of Neuroscience, Imaging and Clinical Sciences, University G. d'Annunzio of Chieti-Pescara, Chieti, Italy., Harris E; Department of Applied Physiology and Kinesiology, College of Health and Human Performance, University of Florida, Gainesville, Florida, USA., Boncompagni S; Center for Advanced Studies and Technology, University G. d'Annunzio of Chieti-Pescara, Chieti, Italy.; Department of Neuroscience, Imaging and Clinical Sciences, University G. d'Annunzio of Chieti-Pescara, Chieti, Italy., Barton ER; Department of Applied Physiology and Kinesiology, College of Health and Human Performance, University of Florida, Gainesville, Florida, USA.; Myology Institute, University of Florida, Gainesville, Florida, USA., Wei-LaPierre L; Department of Applied Physiology and Kinesiology, College of Health and Human Performance, University of Florida, Gainesville, Florida, USA.; Myology Institute, University of Florida, Gainesville, Florida, USA. |
المصدر: | FASEB journal : official publication of the Federation of American Societies for Experimental Biology [FASEB J] 2024 Jul 31; Vol. 38 (14), pp. e23825. |
نوع المنشور: | Journal Article |
اللغة: | English |
بيانات الدورية: | Publisher: Federation of American Societies for Experimental Biology Country of Publication: United States NLM ID: 8804484 Publication Model: Print Cited Medium: Internet ISSN: 1530-6860 (Electronic) Linking ISSN: 08926638 NLM ISO Abbreviation: FASEB J Subsets: MEDLINE |
أسماء مطبوعة: | Publication: 2020- : [Bethesda, Md.] : Hoboken, NJ : Federation of American Societies for Experimental Biology ; Wiley Original Publication: [Bethesda, Md.] : The Federation, [c1987- |
مواضيع طبية MeSH: | Calpain*/metabolism , Calcium*/metabolism , Physical Conditioning, Animal* , Mice, Knockout* , Muscle Proteins*/metabolism , Muscle Proteins*/genetics , Muscle, Skeletal*/metabolism, Animals ; Mice ; Male ; Mice, Inbred C57BL ; Muscular Dystrophies, Limb-Girdle/metabolism ; Muscular Dystrophies, Limb-Girdle/genetics ; Calcium Signaling |
مستخلص: | Limb-Girdle Muscular Dystrophy R1/2A (LGMD R1/2A) is caused by mutations in the CAPN3 gene encoding Calpain 3, a skeletal-muscle specific, Ca 2+ -dependent protease. Localization of Calpain 3 within the triad suggests it contributes to Ca 2+ homeostasis. Through live-cell Ca 2+ measurements, muscle mechanics, immunofluorescence, and electron microscopy (EM) in Capn3 deficient (C3KO) and wild-type (WT) mice, we determined whether loss of Calpain 3 altered Store-Operated Calcium Entry (SOCE) activity. Direct Ca 2+ influx measurements revealed loss of Capn3 elicits elevated resting SOCE and increased resting cytosolic Ca 2+ , supported by high incidence of calcium entry units (CEUs) observed by EM. C3KO and WT mice were subjected to a single bout of treadmill running to elicit SOCE. Within 1HR post-treadmill running, C3KO mice exhibited diminished force production in extensor digitorum longus muscles and a greater decay of Ca 2+ transients in flexor digitorum brevis muscle fibers during repetitive stimulation. Striking evidence for impaired exercise-induced SOCE activation in C3KO mice included poor colocalization of key SOCE proteins, stromal-interacting molecule 1 (STIM1) and ORAI1, combined with disappearance of CEUs in C3KO muscles. These results demonstrate that Calpain 3 is a key regulator of SOCE in skeletal muscle and identify SOCE dysregulation as a contributing factor to LGMD R1/2A pathology. (© 2024 The Author(s). The FASEB Journal published by Wiley Periodicals LLC on behalf of Federation of American Societies for Experimental Biology.) |
التعليقات: | Update of: bioRxiv. 2024 Jan 15:2024.01.12.575391. doi: 10.1101/2024.01.12.575391. (PMID: 38293127) |
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معلومات مُعتمدة: | P50 AR052646 United States AR NIAMS NIH HHS; AGR00024098 Coalition to Cure Calpain 3 Foundation |
فهرسة مساهمة: | Keywords: Limb Girdle Muscular Dystrophy R1/2A; ORAI1; STIM1; calcium homeostasis; skeletal muscle |
المشرفين على المادة: | EC 3.4.22.- (Calpain) EC 3.4.22.- (Capn3 protein, mouse) SY7Q814VUP (Calcium) 0 (Muscle Proteins) |
SCR Disease Name: | Limb-girdle muscular dystrophy type 2A |
تواريخ الأحداث: | Date Created: 20240720 Date Completed: 20240720 Latest Revision: 20240820 |
رمز التحديث: | 20240820 |
مُعرف محوري في PubMed: | PMC11299996 |
DOI: | 10.1096/fj.202400697R |
PMID: | 39031532 |
قاعدة البيانات: | MEDLINE |
تدمد: | 1530-6860 |
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DOI: | 10.1096/fj.202400697R |