With increasing life expectancy, comorbidities become overt in Duchenne muscular dystrophy (DMD). Though micturition problems are common, bladder functioning is poorly understood in DMD. We studied dystrophin expression, and multiple isoform involvement in the bladder during maturation to gain insights into their role in micturition.
Dystrophin distribution was evaluated in rat bladders by immunohistochemical co-localization with smooth muscle, interstitial, urothelial, and neuronal markers. Protein levels of Dp140, Dp71 and smooth muscle were quantified by Western blotting neonatal to adult rat bladders.
Dystrophin co-localized with smooth muscle cells and afferent nerve fibers. Dp71 was expressed two to threefold higher than Dp140, independently of age. Age-related muscle mass changes did not influence isoform expression levels.
Dystrophin is expressed in smooth muscle cells and afferent nerve fibers in the urinary bladder, which underscores that micturition problems in DMD may not solely have a myogenic, but also a neurogenic origin. This article is protected by copyright. All rights reserved.
Muscle & nerve. 2019 May 16 [Epub ahead of print]
Judith M Lionarons, Govert Hoogland, Ruben G F Hendriksen, Catharina G Faber, Danique M J Hellebrekers, Gommert A van Koeveringe, Sandra Schipper, Johan S H Vles
Departments of Neurology, Maastricht University Medical Center, Maastricht, the Netherlands., School for Mental Health & Neuroscience, Maastricht University, Maastricht, the Netherlands.