Analysis of autophagic dysfunction in skeletal muscles from Pompe disease mice by using blood ubiquitin-protein conjugates

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In this study, we tried to evaluate the autophagic dysfunction in skeletal muscle from Pompe disease mice by using blood ubiquitin-protein conjugates.(1) We found that the levels of 58 kDa ubiquitin-protein conjugates in plasma from Pompe disease mice were increased prior to pathological accumulation of autophagosomes in skeletal muscles.(2) We found that levels of 58 kDa proteins were increased in skeletal muscles as well as plasma from Pompe disease mice, and these proteins were localized to autophagosomes accumulated in skeletal muscle fibers from Pompe disease mice.(3) These findings suggested that analysis of 58 kDa ubiquitin-protein conjugates in plasma may be able to detect the autophagic dysfunction in skeletal muscles and be more sensitive than immunohistochemical analysis of skeletal muscles.