Multiple Mitochondrial Alterations in a Case of Myopathy

作者：Fujioka H^*; Tandler B; Cohen M; Koontz D; Hoppel C L

来源：Ultrastructural Pathology, 2014, 38(3): 204-210.

DOI：10.3109/01913123.2014.888114

• Crystalloids
• electron microscopy
• mitochondria
• myopathy
• skeletal muscle

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 similar to 0.34 nm; the parallel rows of these dots had a periodicity of similar to 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.

• 出版日期2014-5