Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
2
pubmed:dateCreated
2008-6-30
pubmed:abstractText
Ultrastructural analysis of the skeletal muscle 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.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:issn
1641-4640
pubmed:author
pubmed:issnType
Print
pubmed:volume
46
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
123-33
pubmed:meshHeading
pubmed:year
2008
pubmed:articleTitle
Pathology of skeletal muscle cells in adult-onset glycogenosis type II (Pompe disease): ultrastructural study.
pubmed:affiliation
Department of Neuropathology, Institute of Psychiatry and Neurology, Warsaw, Poland. lewandow@ipin.edu.pl
pubmed:publicationType
Journal Article