title: Remodeling of mitochondrial interior in cardiac lipofuscinosis.
abstract: Ultrastructural analysis was performed in cardiac ceroidlipofuscinosis to confirm the presence and the nature of storage material. Granular osmophilic deposits characteristic of GROD structures coincidented with particularly aberrant mitochondria. Remodeling of mitochondrial interior with the appearance of several form of abnormal inclusions was never observed in cardiac ceroidlipofuscinosis. The presence of dense osmophilic bodies, glycogen conglomerates, balloon-like and onion-like structures in mitochondrial interior seem to be early events of this storage process.
authors: Neuromuscular Unit, Mossakowski Medical Research Centre, Polish Academy of Sciences, Warsaw, Poland.
authors: Anna
authors: A
authors: Fidziańska
authors: Ewa
authors: E
authors: Walczak
authors: Małgorzata
authors: M
authors: Szwoch
mesh_terms: D001706:Biopsy; D009202:Cardiomyopathies / Q000473:pathology*; D006801:Humans; D002479:Inclusion Bodies / Q000648:ultrastructure; D008854:Microscopy, Electron; D008929:Mitochondria, Heart / Q000648:ultrastructure*; D009206:Myocardium / Q000648:ultrastructure*; D009472:Neuronal Ceroid-Lipofuscinoses / Q000473:pathology*