title: Multiple mitochondrial alterations in a case of myopathy.
abstract: Mitochondrial alterations are the most common feature of human myopathies. A biopsy of quadriceps muscle from a 50-year-old woman exhibiting myopathic symptoms was examined by transmission electron microscopy. Biopsied fibers from quadriceps muscle displayed numerous subsarcolemmal mitochondria that contained crystalloids. Numbering 1-6 per organelle, these consisted of rows of punctuate densities measuring ∼0.34 nm; the parallel rows of these dots had a periodicity of ∼0.8 nm. The crystalloids were ensconced within cristae or in the outer compartment. Some mitochondria without crystalloids had circumferential cristae, leaving a membrane-free center that was filled with a farinaceous material. Other scattered fibrocyte defects included disruption of the contractile apparatus or its sporadic replacement by a finely punctuate material in some myofibers. Intramitochondrial crystalloids, although morphologically striking, do not impair organelle physiology to a significant degree, so the muscle weakness of the patient must originate elsewhere.
authors.affiliation: Electron Microscopy Facility and.
authors.forename: H
authors.initials: H
authors.lastname: Fujioka
authors.forename: B
authors.initials: B
authors.lastname: Tandler
authors.forename: M
authors.initials: M
authors.lastname: Cohen
authors.forename: D
authors.initials: D
authors.lastname: Koontz
authors.forename: C L
authors.initials: CL
authors.lastname: Hoppel
mesh_terms: D001706:Biopsy; D005260:Female; D006801:Humans; D046529:Microscopy, Electron, Transmission; D008875:Middle Aged; D008931:Mitochondria, Muscle / Q000737:chemistry / Q000648:ultrastructure*; D018485:Muscle Fibers, Skeletal / Q000737:chemistry / Q000648:ultrastructure*; D009135:Muscular Diseases / Q000378:metabolism / Q000473:pathology*; D052097:Quadriceps Muscle / Q000737:chemistry / Q000648:ultrastructure*