Аннотация:FEATURESNo mechanism-based cures are available for neurodegenerative disorders such as amyotrophic lateral sclerosis (ALS), Alzheimer's disease and Parkinson's disease.This is despite extensive characterization of many disease-associated mutations and the development of excellent animal models.The fact that the expression of disease-causative genes and proteins starts during embryonic stages raises the intriguing possibility that adult pathology in these devastating disorders is the result of abnormalities initiated during earlier development.Symptoms onset and disease occurs decades later only after compensatory mechanisms can no longer mask the primary insult.In favour of this idea, van Zundert et al., outline in detail pathological changes that have been detected in developing transgenic ALS mouse models expressing mutant superoxide dismutase 1 (SOD1) and discuss the changes in motor neuron physiology that occur months before the onset of behavioral symptoms.A series of critical issues are discussed highlighting the importance of determining the earliest cellular pathophysiology to identify the primary target(s) of SOD1.This effort promises to uncover novel approaches for developing pre-symptomatic diagnostic tools and for the design of new therapies that enhance the nervous system own mechanisms for protection to effectively mitigate or delay the onset of irreversible neuronal damage.
Год издания:2012
Издательство:Wiley
Источник:Journal of Cellular Biochemistry
Ключевые слова:Biotin and Related Studies, Biochemical and biochemical processes
