Connolly G P
Purine Neuroscience Laboratory, Division of Chemical Pathology, Guy's Hospital, United Medical School, London, UK.
Trends Pharmacol Sci. 1998 May;19(5):171-7. doi: 10.1016/s0165-6147(98)01202-4.
Biochemical studies of human fibroblasts from patients with neurological disorders have revealed a wealth of information on how such disorders occur. In this review, Gerald Connolly describes how recently developed fluorescence video imaging techniques have been used to study the physiology of skin fibroblasts isolated from patients with certain neurological disorders, including those produced by Alzheimer's disease, Lesch-Nyhan syndrome, mitochondrial disorders, amyotrophic lateral sclerosis and lysosomal disorders. The results of these studies indicate disruptions in cell homeostasis, particularly specific changes in Ca2+ homeostasis and autofluorescence, which mirror changes thought to occur in the CNS of neurologically impaired patients. More extensive studies of these 'systemic changes' using new fluorescent indicators, combined with advances in imaging techniques, are predicted to increase the potential usefulness of human skin fibroblasts as experimental models and to help diagnose and treat neurological disorders.
对患有神经系统疾病患者的人成纤维细胞进行的生化研究揭示了大量有关此类疾病如何发生的信息。在这篇综述中，杰拉尔德·康诺利描述了最近开发的荧光视频成像技术如何用于研究从患有某些神经系统疾病的患者中分离出的皮肤成纤维细胞的生理学，这些疾病包括由阿尔茨海默病、莱施-尼汉综合征、线粒体疾病、肌萎缩侧索硬化症和溶酶体疾病引起的疾病。这些研究结果表明细胞稳态受到破坏，特别是Ca2+稳态和自发荧光的特定变化，这反映了在神经受损患者的中枢神经系统中被认为会发生的变化。预计使用新的荧光指示剂对这些“全身变化”进行更广泛的研究，再结合成像技术的进步，将增加人类皮肤成纤维细胞作为实验模型的潜在用途，并有助于诊断和治疗神经系统疾病。
