Ultrastructural changes of mitochondria in the skeletal muscle of patients with amyotrophic lateral sclerosis.
Functional defects and morphological changes of mitochondria have been reported to be in the skeletal muscle of patients with amyotrophic lateral sclerosis (ALS). Recent studies suggested that mitochondrial abnormalities are related to the pathogenesis of ALS. The purpose of this study is to evaluate the ultrastructural changes of muscle mitochondria in ALS patients. The authors examined 49 cases of diagnostic muscle biopsy samples with definite or probable ALS by electron microscopy. Of the 49 cases, 5 (10%) had ultrastructural abnormalities of muscle mitochondria, including giant mitochondria, paracrystalline inclusions, and abnormal cristae. These abnormal mitochondria were mainly observed among subsarcolemmal mitochondrial aggregates.
Chung
Myoung Ja
MJ
Department of Pathology, Chonbuk National University, Medical School, Chonju, Korea.
Suh
Yeon-Lim
YL
D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000209:etiology* / Q000473:pathology*; D001706:Biopsy; D006801:Humans; D008875:Middle Aged; D008931:Mitochondria, Muscle / Q000648:ultrastructure*; D017240:Mitochondrial Myopathies / Q000150:complications* / Q000473:pathology*; D018482:Muscle, Skeletal / Q000648:ultrastructure*