Fibroblast models of neurological disorders: fluorescence measurement studies.
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.
Connolly
G P
GP
Purine Neuroscience Laboratory, Division of Chemical Pathology, Guy's Hospital, United Medical School, London, UK.
D000544:Alzheimer Disease / Q000503:physiopathology; D000690:Amyotrophic Lateral Sclerosis / Q000503:physiopathology; D002118:Calcium / Q000378:metabolism; D002478:Cells, Cultured; D002493:Central Nervous System Diseases / Q000175:diagnosis / Q000503:physiopathology*; D005347:Fibroblasts / Q000473:pathology / Q000502:physiology*; D006801:Humans; D007926:Lesch-Nyhan Syndrome / Q000503:physiopathology; D016464:Lysosomal Storage Diseases / Q000503:physiopathology; D017240:Mitochondrial Myopathies / Q000503:physiopathology; D015221:Potassium Channels / Q000502:physiology; D012867:Skin / Q000503:physiopathology*; D013050:Spectrometry, Fluorescence / Q000379:methods
D015221:Potassium Channels; D002118:Calcium