Title: Bladder dysfunction in a transgenic mouse model of multiple system atrophy
Authors: Boudes, Mathieu ×
Uvin, Pieter
Pinto, Silvia
Voets, Thomas
Fowler, Clare J
Wenning, Gregor K
De Ridder, Dirk
Stefanova, Nadia #
Issue Date: Mar-2013
Publisher: John Wiley & Sons
Series Title: Movement disorders : official journal of the Movement Disorder Society vol:28 issue:3 pages:347-55
Article number: 10.1002/mds.25336
Abstract: Multiple system atrophy (MSA) is an adult-onset neurodegenerative disorder presenting with motor impairment and autonomic dysfunction. Urological function is altered in the majority of MSA patients, and urological symptoms often precede the motor syndrome. To date, bladder function and structure have never been investigated in MSA models. We aimed to test bladder function in a transgenic MSA mouse featuring oligodendroglial α-synucleinopathy and define its applicability as a preclinical model to study urological failure in MSA. Experiments were performed in proteolipid protein (PLP)-human α-synuclein (hαSyn) transgenic and control wild-type mice. Diuresis, urodynamics, and detrusor strip contractility were assessed to characterize the urological phenotype. Bladder morphology and neuropathology of the lumbosacral intermediolateral column and the pontine micturition center (PMC) were analyzed in young and aged mice. Urodynamic analysis revealed a less efficient and unstable bladder in MSA mice with increased voiding contraction amplitude, higher frequency of nonvoiding contractions, and increased postvoid residual volume. MSA mice bladder walls showed early detrusor hypertrophy and age-related urothelium hypertrophy. Transgenic hαSyn expression was detected in Schwann cells ensheathing the local nerve fibers in the lamina propria and muscularis of MSA bladders. Early loss of parasympathetic outflow neurons and delayed degeneration of the PMC accompanied the urological deficits in MSA mice. PLP-hαSyn mice recapitulate major urological symptoms of human MSA that may be linked to αSyn-related central and peripheral neuropathology and can be further used as a preclinical model to decipher pathomechanisms of MSA. © 2013 Movement Disorder Society.
ISSN: 0885-3185
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Screening, Diagnostics and Biomarkers (-)
× corresponding author
# (joint) last author

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