Springer Nature
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Additional file 3 of Nebulin nemaline myopathy recapitulated in a compound heterozygous mouse model with both a missense and a nonsense mutation in Neb

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posted on 2020-02-18, 05:01 authored by Jenni Laitila, Elyshia McNamara, Catherine Wingate, Hayley Goullee, Jacob Ross, Rhonda Taylor, Robbert van der Pijl, Lisa Griffiths, Rachel Harries, Gianina Ravenscroft, Joshua Clayton, Caroline Sewry, Michael Lawlor, Coen Ottenheijm, Anthony Bakker, Julien Ochala, Nigel Laing, Carina Wallgren-Pettersson, Katarina Pelin, Kristen Nowak
Additional file 3. Bodyweight and gross functional changes of NebY2303H,Y935X, NebY935X and NebY2303H mice at 6 months of age. a Female NebY2303H,Y935X and NebY935X mice weighed less (p = 0.017* and p = 0.014*, respectively) than wild-type littermate controls. The functional performance of the mice was tested using whole animal in vivo experiments. b Grip strength testing showed reduced average grip (p = 0.013*) and reduced maximum grip (p = 0.0039**) for the female NebY2303H,Y935X mice. c Female NebY2303H,Y935X mice had impaired performance for three of the four voluntary running wheel measures; distance (p = 0.04*), max speed (p = 0.026*), duration (p = 0.072), and average speed (p = 0.038*). However, no significant differences were demonstrated with the rotarod analyses (data not shown), or for the males in any of the functional tests. Mann-Whitney, *p < 0.05; **p < 0.005, n = 10–22 for each parameter.


A Foundation Building Strength for Nemaline Myopathy