Morphological observations in skeletal muscle from patients with a mitochondrial myopathy.

摘要: Mitochondrial metabolic dysfunction is considered to be the cause of certain congenital myopathies and a number multisystem disorders in humans. The morphological hallmark these diseases ‘ragged red’ fibre, which shows abnormally intensive oxidative enzyme reactions. Electron microscopy reveals that numerically increased mitochondria fibres are often markedly enlarged possess aberrant configurations cristae. mitochondrial matrix contains lipid-like inclusions or vacuolation. most characteristic abnormality occurrence highly ordered intracristal intermembrane space. These appear true crystals, composed proteinaceous material. It argued activity accumulation proteins related nuclear nucleolar hypertrophy noticeable ragged red fibres. Since protein crystals particular occur when an capillary density around present, it suggested oxygen free radicals lipid peroxidation processes involved fibre pathology.