RT Journal Article
SR Electronic
T1 A motor axonopathy in a mouse model of Duchenne Muscular Dystrophy
JF bioRxiv
FD Cold Spring Harbor Laboratory
SP 2020.01.22.914614
DO 10.1101/2020.01.22.914614
A1 Justin S Dhindsa
A1 Angela L McCall
A1 Laura M Strickland
A1 Anna F Fusco
A1 Amanda F Kahn
A1 Mai K Elmallah
YR 2020
UL http://biorxiv.org/content/early/2020/01/23/2020.01.22.914614.1.abstract
AB Skeletal muscle weakness due to loss of dystrophin is a well-documented pathological hallmark of Duchenne muscular dystrophy (DMD). In contrast, the neuropathology of this disease remains understudied. Here, we characterize an axonopathy in the phrenic and hypoglossal (XII) nerves of mdx mice. We observe nerve dysfunction that we propose contributes to respiratory failure, the most common cause of death in DMD.