Abstract:
:The muscle biopsy from a 12-year-old boy with chronic creatine kinase elevation was studied by electron microscopy. At the age of 7 years the patient had a possible abortive attack of malignant hyperthermia. The biopsy specimen contained many fibres with segmental contracture and necrosis; thin sections showed defects of the plasma membrane with loss of glycogen granules into the interstitium. Freeze-fracturing of the plasma membrane showed clustering of intramembranous particles and some membrane areas were devoid of particles and of pinocytotic caveolae. The E-face showed irregular elevations and the P-face corresponding defects. These deviations of the fracture-plane were due to manifest membrane openings, to abnormalities of the structure of the lipid bilayer, to clustering of membrane particles or to the fact that intracellular membranes often were abnormally close to the plasma membrane. The findings suggest that a disintegration of the lipid-protein system of the membrane precedes the formation of manifest defects. The manifest defects resembled those in Duchenne muscular dystrophy. It is unknown whether the changes seen by freeze-fracturing were specific for a myogenic disorder and whether they were due to a basic membrane abnormality or to defects in other systems of the muscle cell.
journal_name
J Neuropathol Exp Neuroljournal_title
Journal of neuropathology and experimental neurologyauthors
Schmalbruch Hdoi
10.1097/00005072-197907000-00006subject
Has Abstractpub_date
1979-07-01 00:00:00pages
407-18issue
4eissn
0022-3069issn
1554-6578journal_volume
38pub_type
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journal_title:Journal of neuropathology and experimental neurology
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journal_title:Journal of neuropathology and experimental neurology
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journal_title:Journal of neuropathology and experimental neurology
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journal_title:Journal of neuropathology and experimental neurology
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journal_title:Journal of neuropathology and experimental neurology
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journal_title:Journal of neuropathology and experimental neurology
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journal_title:Journal of neuropathology and experimental neurology
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journal_title:Journal of neuropathology and experimental neurology
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