Exploratory Profiling of Urine MicroRNAs in the dy2J/dy2J Mouse Model of LAMA2-CMD: Relation to Disease Progression

In this study we aimed at profiling miRNA expression in urine from dy2J/dy2J mice to assess their potential for monitoring disease progression. Three distinct time points (three, four and six weeks of age) were chosen to represent asymptomatic, initial symptoms and established disease, respectively. Here we show that distinct sets of miRNA characterise each time point whilst CK fails to differentiate between them. MATERIALS AND METHODS Ethics Statement Wild-type and dy2J/dy2J (B6.WK-Lama2dy-2J/J) mice were purchased from Jackson laboratory and bred in the Biomedical Center according to institutional animal care guidelines. Permission was given by the Malmö/Lund (Sweden) ethical committee for animal research (ethical permit number M152-14). Tissue collection Three-, four- and six-week-old control and dy2J/dy2J mice (n = 5 per group) were sacrificed by cervical dislocation. Quadriceps muscles were dissected for histology and embedded in paraffin. Histology and morphometric analyses Muscle sections were stained with haematoxylin & eosin 12 or picro sirius red/fast green 11. Stained cross-sections were scanned using Aperio’s Scanscope CS2 (with Scanscope console v. 8.2.0.1263) and representative images were created using Aperio software. Centrally nucleated muscle fibres representing regenerating muscle cells and peripherally nucleated non-regenerating muscle cells were counted in quadriceps femoris using ImageJ software version 1.45i (NIH, Bethesd...
Source: PLOS Currents Muscular Dystrophy - Category: Neurology Authors: Source Type: research