Pathology of skeletal muscle cells in adult-onset glycogenosis type II (Pompe disease): ultrastructural study

Eliza Lewandowska; Teresa Wierzba-Bobrowicz; Rafał Rola; Joanna Modzelewska; Tomasz Stępień; Agnieszka Ługowska; Elżbieta Pasennik; Danuta Ryglewicz

eISSN: 1509-572x
ISSN: 1641-4640

Folia Neuropathologica

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2/2008
vol. 46

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abstract:

Pathology of skeletal muscle cells in adult-onset glycogenosis type II (Pompe disease): ultrastructural study

Eliza Lewandowska

,

Teresa Wierzba-Bobrowicz

,

Rafał Rola

,

Joanna Modzelewska

,

Tomasz Stępień

,

Agnieszka Ługowska

,

Elżbieta Pasennik

,

Danuta Ryglewicz

Folia Neuropathol 2008; 46 (2): 123-133

Online publish date: 2008/06/24

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Ultrastructural analysis of the skeletal muscle cells in adult-onset Pompe disease revealed lysosomal and cytoplasmic glycogen storage, autophagic vacuoles and abnormal mitochondria. Significant glycogen accumulation within lysosomes causes their rupture and release of glycogen into the cytoplasm. Excess cytoplasmic glycogen could lead to damage of the structure of muscle cells including myofibrils. In consequence, parts of the sarcoplasm and damaged organelles were sequestered within membrane-limited vacuoles. We suppose that massive accumulation of autophagic vacuoles results from the inability of destroyed lysosomes to fuse with vacuoles. Autophagic vacuoles may be a prominent feature of muscle cells in adult glycogenosis type II.

keywords:

Pathology of skeletal muscle cells in adult-onset glycogenosis type II (Pompe disease): ultrastructural study

Eliza Lewandowska , Teresa Wierzba-Bobrowicz , Rafał Rola , Joanna Modzelewska , Tomasz Stępień , Agnieszka Ługowska , Elżbieta Pasennik , Danuta Ryglewicz

adult-onset Pompe disease, skeletal muscle cells, ultrastructure, lysosomes, autophagic vacuoles

Eliza Lewandowska

,

Teresa Wierzba-Bobrowicz

,

Rafał Rola

,

Joanna Modzelewska

,

Tomasz Stępień

,

Agnieszka Ługowska

,

Elżbieta Pasennik

,

Danuta Ryglewicz