OXIDATIVE METABOLISM IN INHERITED NEUROLOGICAL DISEASES AND MYCOPLASMAS

越来越多的证据表明，氧化可能存在缺陷。 某些遗传性神经疾病患者的代谢。 因此，丙酮酸氧化系统的缺陷已经在一些 患有乳酸血症和弥漫性神经系统疾病的患者 Friedreich‘s共济失调患者的线粒体苹果酸酶和 部分慢性阻塞性肺疾病患者谷氨酸脱氢酶部分缺乏 橄榄桥-小脑变性。然而，有很多争议。 关于确切的酶缺陷(S)。这个项目的目标是 其中一些患者或皮肤成纤维细胞中的缺陷的阐明 从这样的病人中衍生出来的。为此，我们正在分析一些 成纤维细胞或白细胞中的线粒体和非线粒体酶 我们已经开始了对线粒体的电子显微镜研究。我们 开始对支原体的氧化代谢感兴趣是因为 成纤维细胞培养中的支原体污染干扰了测定 这些细胞中的丙酮酸脱氢酶复合体。氧化代谢 人们对支原体的了解很少。希望，对这一点的澄清 这些疾病的缺陷将有助于诊断和治疗。 对患者进行干预。支原体生理学知识 可能有助于了解这些生物的致病性。

An increasing amount of evidence points to a possible defect in oxidative metabolism in patients with certain inherited neurological disorders. Thus, a defect in the pyruvate oxidation system has been shown in some patients with lactic acidemia and diffuse neurologic disease, of the mitochondrial malic enzyme in patients with Friedreich's ataxia, and a partial deficiency of glutamate dehydrogenase in some patients with olivopontocerebellar degeneration. However, there is much controversy about the exact enzymatic defect(s). The objective of this project is the elucidation of the defect in some of these patients or in skin fibroblasts derived from such patients. For this purpose we are assaying a number of mitochondrial and non-mitochondrial enzymes in fibroblasts or leukocytes and we have initiated electron microscopic studies of the mitochondria. We became interested in the oxidative metabolism of mycoplasmas because mycoplasma contamination of fibroblast cultures interfered with the assay of pyruvate dehydrogenase complex in these cells. The oxidative metabolism of mycoplasmas is poorly understood. Hopefully, the elucidation of the defect in these diseases will help in the diagnosis and therapeutic intervention in the patients. Knowledge of the physiology of mycoplasmas may help in understanding the pathogenicity of these organisms.