{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Li D"],"funding":["NIDDK NIH HHS","National Institutes of Health","NIAMS NIH HHS","NIH","Muscular Dystrophy Association"],"pagination":["88-98"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC3109084"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["223(1)"],"pubmed_abstract":["The mechanism of force reduction is not completely understood in Duchenne muscular dystrophy (DMD), a dystrophin-deficient lethal disease. Nitric oxide regulates muscle force. Interestingly, neuronal nitric oxide synthase µ (nNOSµ), a major source of muscle nitric oxide, is lost from the sarcolemma in DMD muscle. We hypothesize that nNOSµ delocalization contributes to force reduction in DMD. To test this hypothesis, we generated dystrophin/nNOSµ double knockout mice. Genetic elimination of nNOSµ significantly enhanced force in dystrophin-null mice. Pharmacological inhibition of nNOS yielded similar results. To further test our hypothesis, we studied δ-sarcoglycan-null mice, a model of limb-girdle muscular dystrophy. These mice had minimal sarcolemmal nNOSµ delocalization and muscle force w"],"journal":["The Journal of pathology"],"pubmed_title":["Nitrosative stress elicited by nNOSµ delocalization inhibits muscle force in dystrophin-null mice."],"pmcid":["PMC3109084"],"funding_grant_id":["T90DK70105","R01 AR049419","AR-49419","T90 DK070105","T90 DK070105-05","R01 AR049419-08"],"pubmed_authors":["Lai Y","Li D","Duan D","Yue Y","Hakim CH"],"additional_accession":[]},"is_claimable":false,"name":"Nitrosative stress elicited by nNOSµ delocalization inhibits muscle force in dystrophin-null mice.","description":"The mechanism of force reduction is not completely understood in Duchenne muscular dystrophy (DMD), a dystrophin-deficient lethal disease. Nitric oxide regulates muscle force. Interestingly, neuronal nitric oxide synthase µ (nNOSµ), a major source of muscle nitric oxide, is lost from the sarcolemma in DMD muscle. We hypothesize that nNOSµ delocalization contributes to force reduction in DMD. To test this hypothesis, we generated dystrophin/nNOSµ double knockout mice. Genetic elimination of nNOSµ significantly enhanced force in dystrophin-null mice. Pharmacological inhibition of nNOS yielded similar results. To further test our hypothesis, we studied δ-sarcoglycan-null mice, a model of limb-girdle muscular dystrophy. These mice had minimal sarcolemmal nNOSµ delocalization and muscle force w","dates":{"release":"2011-01-01T00:00:00Z","publication":"2011 Jan","modification":"2025-04-19T11:30:51.295Z","creation":"2019-03-27T00:42:19Z"},"accession":"S-EPMC3109084","cross_references":{"pubmed":["21125668"],"doi":["10.1002/path.2799"]}}