The fine structure of the intramitochondrial crystalloids found in mitochondrial myopathies was investigated using high angle tilting of ultrathin sections and freeze fracture replicas. Observations show the crystalloids to be composed of 8 nm granules in a unique array. Analysis of the arrangement seen in three mutually perpendicular planes has permitted a model of the crystalloid and its relationship to the mitochondrial membranes to be proposed. The biochemical nature and pathophysiological significance of the crystalloids is still undetermined.
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