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Myostatin Inhibition Using ActRIIB-mFc Does Not Produce Weight Gain or Strength in the Nebulin Conditional KO Mouse.

Journal of neuropathology and experimental neurology (2019-01-01)
Jennifer A Tinklenberg, Emily M Siebers, Margaret J Beatka, Brittany A Fickau, Samuel Ayres, Hui Meng, Lin Yang, Pippa Simpson, Henk L Granzier, Michael W Lawlor
RESUMEN

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 studies demonstrate a lack of response in Neb cKO mice to myostatin inhibition, which differs from the response observed when treating other NM models.

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Sigma-Aldrich
Anticuerpo anti-GAPDH, monoclonal de ratón antibody produced in mouse, clone GAPDH-71.1, purified from hybridoma cell culture
Sigma-Aldrich
Monoclonal Anti-MYOD1 antibody produced in mouse, clone 5.2F, purified immunoglobulin, buffered aqueous solution