Ben Hamida C, Soussi-Yanicostas N, Butler-Browne G S, Bejaoui K, Hentati F, Ben Hamida M
Institut National de Neurologie, Laboratoire de Neuropathologie et de Neurobiologie Moléculaire, La Rabta, Tunis, Tunisia.
Muscle Nerve. 1994 Apr;17(4):400-10. doi: 10.1002/mus.880170407.
Immunocytochemical and biochemical analyses were carried out on patients affected by chronic SMA. Three groups of patients were identified. In group I, the muscle presented a fascicular atrophy; a high percentage of atrophic type II fibers; and fibers expressing fast, slow, embryonic, and fetal myosin isoforms. In group II, the muscle was characterized by atrophic fibers and normal/hypertrophic fibers expressing only slow myosin isoforms. In group III, the muscle was characterized by fiber type grouping and fibers coexpressing fast and slow myosin isoforms but never embryonic or fetal MHC isoforms. The muscles of groups I and III contained both fast and slow myosins whereas group II muscles were predominantly slow by immunocytochemical analysis or only slow by biochemical analysis. In view of these results, immunocytochemical and histochemical analyses could help to classify chronic SMA and help to understand the different pathogenic processes which seem to be related to the maturational stage of the muscle at the age of onset of the disease.
对慢性脊髓性肌萎缩症患者进行了免疫细胞化学和生化分析。确定了三组患者。在第一组中，肌肉呈现束状萎缩；高比例的萎缩II型纤维；以及表达快、慢、胚胎和胎儿肌球蛋白异构体的纤维。在第二组中，肌肉的特征是萎缩纤维以及仅表达慢肌球蛋白异构体的正常/肥大纤维。在第三组中，肌肉的特征是纤维类型分组以及同时表达快和慢肌球蛋白异构体但从不表达胚胎或胎儿MHC异构体的纤维。第一组和第三组的肌肉中同时含有快肌球蛋白和慢肌球蛋白，而通过免疫细胞化学分析，第二组肌肉主要是慢肌球蛋白，或通过生化分析仅为慢肌球蛋白。鉴于这些结果，免疫细胞化学和组织化学分析有助于对慢性脊髓性肌萎缩症进行分类，并有助于了解不同的致病过程，这些过程似乎与疾病发病年龄时肌肉的成熟阶段有关。
