Abstract

This chapter focuses on neurotoxins and neurodegenerative diseases and demonstrates how clinical observations led to the study of toxic compounds in model systems. It is generally accepted that the study of selective neurotoxins plays a significant role in the improvement of understanding of the pathogenesis of neurodegeneration in various diseases of the nervous system. It is expected that the present and other future model systems would reveal further insights into the pathogenesis of sporadically occurring neurodegenerative diseases. This chapter demonstrates that there is no present evidence that supports the assumption that exogenous chemicals play an etiological role in progressive sporadic neurodegenerative diseases. The chapter focuses on compounds, mechanisms, and diseases that seem to be of major relevance for understanding of human neurological diseases. The chapter discusses the possible impact of neurotoxic model systems for sporadic neurodegenerative diseases, such as Parkinsonism, hyperkinetic extrapyramidal movement disorders, and motor neuron diseases. The chapter also elaborates some inherited metabolic diseases, which are presumably related to an endogenous neurotoxic mechanism as part of their pathogenesis.

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