Age-related changes and sialylation pattern in the skeletal muscle of C57Bl/6 GNE±and C57Bl/6 wildtype mice

Aims: Reduced sialic acid might have an impact on the ageing skeletal muscle in the heterozygous UDP-N-Acetylglucosamine2-Epimerase/N-Acetylmannosamine kinase GNE knock out mouse model (C57Bl/6 GNE±) compared to wildtype (C57Bl/6 GNE+/+), mimicking a carrier status of autosomal-recessive hereditary inclusion body myositis. Methods: Mice were analysed after 24 weeks (n=9 C57Bl/6 GNE+/+, n=7 C57Bl/6 GNE±) and after 80 weeks (n=10 C57Bl/6 GNE+/+, n=12 C57Bl/6 GNE±) for clinical phenotype, creatine kinase (CK) levels, histopathological changes by light and electron microscopy, and membrane bound sialic acid concentr…