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) |
| References: | Cell. 1995 Apr 7;81(1):27-40. (PMID: 7720071) Eur J Hum Genet. 2002 Dec;10(12):825-32. (PMID: 12461690) FASEB J. 2016 Dec;30(12):4109-4119. (PMID: 27587568) Nat Commun. 2013;4:2805. (PMID: 24241282) Yonsei Med J. 2016 Jan;57(1):173-9. (PMID: 26632398) Neuromuscul Disord. 2007 Feb;17(2):148-56. (PMID: 17236769) J Clin Neurosci. 2018 Jul;53:229-231. (PMID: 29685414) Biochim Biophys Acta. 2007 Feb;1772(2):128-44. (PMID: 16934440) Skelet Muscle. 2016 Feb 24;6:11. (PMID: 26913171) Nat Med. 1999 May;5(5):503-11. (PMID: 10229226) Hum Mol Genet. 2014 Jul 15;23(14):3706-15. (PMID: 24556214) Hum Mol Genet. 2011 Sep 1;20(17):3331-45. (PMID: 21624972) Skelet Muscle. 2020 Jul 29;10(1):22. (PMID: 32727611) Hum Mol Genet. 2008 Nov 1;17(21):3271-80. (PMID: 18676612) Clin Exp Pharmacol Physiol. 2017 Jan;44(1):3-12. (PMID: 27696487) Muscle Nerve. 2015 Aug;52(2):163-73. (PMID: 25900067) Am J Physiol Cell Physiol. 2010 Jul;299(1):C42-50. (PMID: 20427714) Anal Biochem. 2008 Jun 1;377(1):111-3. (PMID: 18358820) Antioxid Redox Signal. 2020 Apr 20;32(12):873-883. (PMID: 31825235) Clin Genet. 2019 Oct;96(4):341-353. (PMID: 31268554) Trends Cardiovasc Med. 1999 Jul;9(5):114-8. (PMID: 10639725) Int J Cell Biol. 2009;2009:340346. (PMID: 20300593) Pediatr Neonatol. 2015 Feb;56(1):62-5. (PMID: 23597518) Hum Mol Genet. 2004 Jul 1;13(13):1373-88. (PMID: 15138196) J Biol Chem. 1998 Jul 3;273(27):17073-8. (PMID: 9642272) J Mol Biol. 2011 Apr 1;407(3):439-49. (PMID: 21295580) Front Physiol. 2020 Dec 21;11:601090. (PMID: 33408641) Expert Rev Mol Med. 2016 Apr 08;18:e7. (PMID: 27055500) J Physiol. 2007 Aug 15;583(Pt 1):81-97. (PMID: 17569733) J Biol Chem. 1989 Nov 25;264(33):20106-11. (PMID: 2555341) J Gen Physiol. 2022 Sep 5;154(9):. (PMID: 35939054) Hum Mol Genet. 2016 Jun 1;25(11):2194-2207. (PMID: 27005420) J Clin Invest. 2020 Mar 2;130(3):1271-1287. (PMID: 32039917) Neurol Res. 2010 Feb;32(1):41-6. (PMID: 20092694) Hum Mol Genet. 2009 Sep 1;18(17):3194-205. (PMID: 19483197) Expert Rev Mol Med. 2014 Oct 08;16:e16. (PMID: 25293420) Hum Mol Genet. 2012 Jul 15;21(14):3193-204. (PMID: 22505582) Cell Calcium. 2024 Jul;121:102892. (PMID: 38735127) Elife. 2019 Oct 28;8:. (PMID: 31657717) J Gen Physiol. 2020 Oct 5;152(10):. (PMID: 32761048) Hum Mol Genet. 2014 Oct 15;23(20):5325-41. (PMID: 24879640) Biochimie. 2016 Mar;122:169-87. (PMID: 26363099) J Physiol. 2008 Oct 15;586(20):4815-24. (PMID: 18772199) Cell Calcium. 2010 May;47(5):458-67. (PMID: 20434768) J Muscle Res Cell Motil. 2021 Jun;42(2):233-249. (PMID: 32812118) Ann Clin Transl Neurol. 2016 Mar 04;3(4):248-65. (PMID: 27081656) Am J Hum Genet. 1999 Jun;64(6):1524-40. (PMID: 10330340) J Gen Physiol. 2022 Dec 5;154(12):. (PMID: 36222861) J Biol Chem. 2006 Jul 7;281(27):18519-31. (PMID: 16627476) Neuromuscul Disord. 2001 Apr;11(3):287-96. (PMID: 11297944) Sci Rep. 2017 Oct 27;7(1):14286. (PMID: 29079778) |
| 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 |