{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Tinklenberg JA"],"funding":["NIAMS NIH HHS","NIH","Muscular Dystrophy Association","A Foundation Building Strength"],"pagination":["130-139"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC6804412"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["78(2)"],"pubmed_abstract":["Mutations in at least 12 genes are responsible for a group of congenital skeletal muscle diseases known as nemaline myopathies (NMs). NMs are associated with a range of clinical symptoms and pathological changes often including the presence of cytoplasmic rod-like structures (nemaline bodies) and myofiber hypotrophy. Our recent work has identified a variable degree of behavioral benefit when treating 2 NM mouse models due to mutations in Acta1 with myostatin inhibition. This study is focused on the effects of delivering ActRIIB-mFc (Acceleron; a myostatin inhibitor) to the nebulin conditional knockout KO (Neb cKO) mouse model of NM. Treatment of Neb cKO mice with ActRIIB-mFc did not produce increases in weight gain, strength, myofiber size, or hypertrophic pathway signaling. Overall, our s"],"journal":["Journal of neuropathology and experimental neurology"],"pubmed_title":["Myostatin Inhibition Using ActRIIB-mFc Does Not Produce Weight Gain or Strength in the Nebulin Conditional KO Mouse."],"pmcid":["PMC6804412"],"funding_grant_id":["MDA 295195","NIH R01AR053897","R01 AR053897"],"pubmed_authors":["Tinklenberg JA","Simpson P","Yang L","Granzier HL","Lawlor MW","Siebers EM","Beatka MJ","Fickau BA","Ayres S","Meng H"],"additional_accession":[]},"is_claimable":false,"name":"Myostatin Inhibition Using ActRIIB-mFc Does Not Produce Weight Gain or Strength in the Nebulin Conditional KO Mouse.","description":"Mutations in at least 12 genes are responsible for a group of congenital skeletal muscle diseases known as nemaline myopathies (NMs). NMs are associated with a range of clinical symptoms and pathological changes often including the presence of cytoplasmic rod-like structures (nemaline bodies) and myofiber hypotrophy. Our recent work has identified a variable degree of behavioral benefit when treating 2 NM mouse models due to mutations in Acta1 with myostatin inhibition. This study is focused on the effects of delivering ActRIIB-mFc (Acceleron; a myostatin inhibitor) to the nebulin conditional knockout KO (Neb cKO) mouse model of NM. Treatment of Neb cKO mice with ActRIIB-mFc did not produce increases in weight gain, strength, myofiber size, or hypertrophic pathway signaling. Overall, our s","dates":{"release":"2019-01-01T00:00:00Z","publication":"2019 Feb","modification":"2025-04-26T22:49:24.366Z","creation":"2020-05-22T08:49:04Z"},"accession":"S-EPMC6804412","cross_references":{"pubmed":["30597051"],"doi":["10.1093/jnen/nly120"]}}