{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Villani KR"],"funding":["National Institute of Arthritis and Musculoskeletal and Skin Diseases","Coalition to Cure Calpain 3 Foundation","NIAMS NIH HHS"],"pagination":["e23825"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC11299996"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["38(14)"],"pubmed_abstract":["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<sup>2+</sup>-dependent protease. Localization of Calpain 3 within the triad suggests it contributes to Ca<sup>2+</sup> homeostasis. Through live-cell Ca<sup>2+</sup> 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<sup>2+</sup> influx measurements revealed loss of Capn3 elicits elevated resting SOCE and increased resting cytosolic Ca<sup>2+</sup>, supported by high incidence of calcium entry units (CEUs) observed by EM. C3KO and WT mice were subjected to a single bou"],"journal":["FASEB journal : official publication of the Federation of American Societies for Experimental Biology"],"pubmed_title":["Loss of Calpain 3 dysregulates store-operated calcium entry and its exercise response in mice."],"pmcid":["PMC11299996"],"funding_grant_id":["P50 AR052646","AGR00024098"],"pubmed_authors":["Barton ER","Boncompagni S","Wei-LaPierre L","Zhong R","Henley-Beasley CS","Rastelli G","Harris E","Villani KR"],"additional_accession":[]},"is_claimable":false,"name":"Loss of Calpain 3 dysregulates store-operated calcium entry and its exercise response in mice.","description":"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<sup>2+</sup>-dependent protease. Localization of Calpain 3 within the triad suggests it contributes to Ca<sup>2+</sup> homeostasis. Through live-cell Ca<sup>2+</sup> 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<sup>2+</sup> influx measurements revealed loss of Capn3 elicits elevated resting SOCE and increased resting cytosolic Ca<sup>2+</sup>, supported by high incidence of calcium entry units (CEUs) observed by EM. C3KO and WT mice were subjected to a single bou","dates":{"release":"2024-01-01T00:00:00Z","publication":"2024 Jul","modification":"2026-03-16T08:59:58.561Z","creation":"2025-08-15T03:06:42.313Z"},"accession":"S-EPMC11299996","cross_references":{"pubmed":["39031532"],"doi":["10.1096/fj.202400697R","10.1096/fj.202400697r"]}}