Suppression of autophagy in skeletal muscle uncovers the accumulation of ubiquitinated proteins and their potential role in muscle damage in Pompe disease.

@article{Raben2008SuppressionOA,
  title={Suppression of autophagy in skeletal muscle uncovers the accumulation of ubiquitinated proteins and their potential role in muscle damage in Pompe disease.},
  author={Nina Raben and Victoria Hill and Lauren E. Shea and Shoichi Takikita and Rebecca Baum and Noboru Mizushima and Evelyn Ralston and Paul H. Plotz},
  journal={Human molecular genetics},
  year={2008},
  volume={17 24},
  pages={
          3897-908
        }
}
The role of autophagy, a catabolic lysosome-dependent pathway, has recently been recognized in a variety of disorders, including Pompe disease, the genetic deficiency of the glycogen-degrading lysosomal enzyme acid-alpha glucosidase. Accumulation of lysosomal glycogen, presumably transported from the cytoplasm by the autophagic pathway, occurs in multiple tissues, but pathology is most severe in skeletal and cardiac muscle. Skeletal muscle pathology also involves massive autophagic buildup in… CONTINUE READING
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