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http://purl.uniprot.org/citations/31665639http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/31665639http://www.w3.org/2000/01/rdf-schema#comment"Muscle function is regulated by Ca2+, which mediates excitation-contraction coupling, energy metabolism, adaptation to exercise, and sarcolemmal repair. Several of these actions rely on Ca2+ delivery to the mitochondrial matrix via the mitochondrial Ca2+ uniporter, the pore of which is formed by mitochondrial calcium uniporter (MCU). MCU's gatekeeping and cooperative activation are controlled by MICU1. Loss-of-protein mutation in MICU1 causes a neuromuscular disease. To determine the mechanisms underlying the muscle impairments, we used MICU1 patient cells and skeletal muscle-specific MICU1 knockout mice. Both these models show a lower threshold for MCU-mediated Ca2+ uptake. Lack of MICU1 is associated with impaired mitochondrial Ca2+ uptake during excitation-contraction, aerobic metabolism impairment, muscle weakness, fatigue, and myofiber damage during physical activity. MICU1 deficit compromises mitochondrial Ca2+ uptake during sarcolemmal injury, which causes ineffective repair of the damaged myofibers. Thus, dysregulation of mitochondrial Ca2+ uptake hampers myofiber contractile function, likely through energy metabolism and membrane repair."xsd:string
http://purl.uniprot.org/citations/31665639http://purl.org/dc/terms/identifier"doi:10.1016/j.celrep.2019.09.063"xsd:string
http://purl.uniprot.org/citations/31665639http://purl.uniprot.org/core/author"Horn A."xsd:string
http://purl.uniprot.org/citations/31665639http://purl.uniprot.org/core/author"Singh R."xsd:string
http://purl.uniprot.org/citations/31665639http://purl.uniprot.org/core/author"Horvath R."xsd:string
http://purl.uniprot.org/citations/31665639http://purl.uniprot.org/core/author"Seifert E.L."xsd:string
http://purl.uniprot.org/citations/31665639http://purl.uniprot.org/core/author"Jaiswal J.K."xsd:string
http://purl.uniprot.org/citations/31665639http://purl.uniprot.org/core/author"Huang K.T."xsd:string
http://purl.uniprot.org/citations/31665639http://purl.uniprot.org/core/author"Hajnoczky G."xsd:string
http://purl.uniprot.org/citations/31665639http://purl.uniprot.org/core/author"Debattisti V."xsd:string
http://purl.uniprot.org/citations/31665639http://purl.uniprot.org/core/author"Hogarth M.W."xsd:string
http://purl.uniprot.org/citations/31665639http://purl.uniprot.org/core/author"Mazala D.A."xsd:string
http://purl.uniprot.org/citations/31665639http://purl.uniprot.org/core/date"2019"xsd:gYear
http://purl.uniprot.org/citations/31665639http://purl.uniprot.org/core/name"Cell Rep"xsd:string
http://purl.uniprot.org/citations/31665639http://purl.uniprot.org/core/pages"1274-1286.e6"xsd:string
http://purl.uniprot.org/citations/31665639http://purl.uniprot.org/core/title"Dysregulation of Mitochondrial Ca2+ Uptake and Sarcolemma Repair Underlie Muscle Weakness and Wasting in Patients and Mice Lacking MICU1."xsd:string
http://purl.uniprot.org/citations/31665639http://purl.uniprot.org/core/volume"29"xsd:string
http://purl.uniprot.org/citations/31665639http://www.w3.org/2004/02/skos/core#exactMatchhttp://purl.uniprot.org/pubmed/31665639
http://purl.uniprot.org/citations/31665639http://xmlns.com/foaf/0.1/primaryTopicOfhttps://pubmed.ncbi.nlm.nih.gov/31665639
http://purl.uniprot.org/uniprot/#_E9Q9E0-mappedCitation-31665639http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/31665639
http://purl.uniprot.org/uniprot/#_F6Z1Z9-mappedCitation-31665639http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/31665639
http://purl.uniprot.org/uniprot/#_Q9BPX6-mappedCitation-31665639http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/31665639
http://purl.uniprot.org/uniprot/#_Q8VCX5-mappedCitation-31665639http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/31665639
http://purl.uniprot.org/uniprot/F6Z1Z9http://purl.uniprot.org/core/mappedCitationhttp://purl.uniprot.org/citations/31665639