Biochemical and hislogical studies of nitric oxide (NO) in neuromuscular disorders.

[1]肌肉组织中一氧化氮合酶（NOS）和超氧化物歧化酶（SOD）的组织学测定：对肌肉组织中Mn-SOD进行免疫组织化学分析。Mn-SOD在线粒体脑肌病中主要分布于肌膜下的红色碎布纤维区。Mn-SOD阳性纤维可用于诊断线粒体脑肌病。此外，为了确定病变肌肉中一氧化氮合酶的定位，进行了神经元型NOS（nNOS）和内皮型NOS（ec-NOS）的免疫组织化学分析。nNOS的免疫组化染色在参差不齐的红色纤维的肌膜区是突出的。EC-NOS在红色破碎纤维的肌原纤维中呈强阳性。结果表明，SOD和NOS是重要的线粒体脑肌病患者的肌肉。在多发性肌炎中，坏死或再生的纤维显示nNOS和NADH-黄递酶染色较差。[2]NOS和d的组织学定位 ...更多信息 骨骼肌中坏死或再生纤维中的肌萎缩蛋白相关蛋白：通过在大鼠骨骼肌中注射心脏毒素制备骨骼肌纤维坏死或再生的大鼠模型。肌营养不良蛋白、α肌聚糖和α 1促突触蛋白的免疫染色在坏死的肌纤维中消失，并在几天后在再生的肌纤维中重新出现。肌营养不良蛋白和α肌聚糖的表达在再生过程中往往早于。在坏死或再生肌纤维的多发性肌炎，肌营养不良蛋白和α肌聚糖的免疫染色是明显阳性的肌膜区域，而α 1 syntrophin和NOS染色不佳或没有染色。[3]细胞因子诱导的C2 C12骨骼肌肌细胞中NOS的表达：为了确定骨骼肌中是否可以诱导NOS的产生，用细胞因子刺激C2 C12小鼠骨骼肌肌细胞。干扰素-γ、白细胞介素-1 α和肿瘤坏死因子-α的组合导致亚硝酸盐的产生。亚硝酸盐的产生显着减少细胞与L-NAME在细胞因子刺激的共孵育。Cu/Zn-SOD、Mn-SOD或过氧化氢酶共孵育也抑制了亚硝酸盐的产生。少

[1] Histological determination of nitric oxide synthase (NOS) and superoxide dismutase (SOD) in biopsied human muscle : Immunohistochemical analyzes were made of Mn-SOD in biopsied muscles. Mn-SOD was mainly present in subsarcolmmal region of ragged-red fibers in mitochondrial encephalomyopathies. Mn-SOD positive fibers may be of use for diagnosing mitochondrial encephalomyopathies. In addition, to determine localization of nitric oxide synthase in diseased muscle, immunohistochemical analyzes of neuronal-type NOS (nNOS) and endothelial-type NOS (ec-NOS) were performed. Immunostaining of nNOS was prominent in the sarcolemmal region of the ragged-red fibers. Ec-NOS was strongly positive in the myofibrils of ragged-red fibers. Findings suggest that SOD and NOS are important in the muscles of patients with mitochondrial encephalomyopaties. In polymyositis, necrotic or regenerating fibers showed poorly staining of nNOS and NADH-diaphorase staining.[2] Histological localization of NOS and d … More ystrophin related proteins in necrotic or regenerating fibers in skeletal muscle : A rat model for necrosis or regeneration of skeletal muscle fibers was prepared by cardiotoxin injection in rat skeletal muscles. Immunostaining of dystrophin, alpha sarcoglycan and alpha 1 syntrophin disappeared in necrotic muscle fibers, and reappeared in regenerating fibers after several days. Expression of dystrophin and alpha sarcoglycan tended to be earlier than that of in the regenerating process. In necrotic or regenerating muscle fibers of polymyostitis, immunostaining of dystrophin and alpha sarcoglycan was distinctly positive in the sarcolemmal region, while alpha 1 syntrophin and NOS poorly stained or did not stain.[3] Cytokine-induced expression of NOS in C2C12 skeletal muscle myocytes : To determine whether NOS production can be induced in skeletal muscle, i stimulated C2C12 mouse skeletal muscle myocytes with cytokines. Combinations of interferon-gamma, interleukin-1alpha, and tumor necrotic facter-alpha resulted nitrite production. Nitrite production was significantly reduced by the coincubation of cells with L-NAME during cytokine stimulations. The coincubation Cu/Zn-SOD,Mn-SOD or catalase also inhibited nitrite production with cytokine stimulations. Less