Mortalin in neurological diseases

Abstract

Mitochondria play critical roles in neuronal function and emerging evidence shows that mitochondrial dysfunction is essential in the pathogenesis of several major neurological diseases, including neurodegenerative disorders and cerebral ischemia. The present chapter centers on the roles of the functionally diverse mitochondrial protein, mortalin. Since its initial discovery in mouse embryonic fibroblasts and identification of its association with cellular mortality, subsequent research efforts have recognized mortalin as having potential roles in the maintenance of mitochondrial homeostasis, energy generation, mitochondrial import of nuclear-encoded proteins, and chaperoning of misfolded proteins. Dysfunction of mortalin has been implicated in a variety of neurological disorders, including Alzheimer's disease, Parkinson's disease, and brain tumors in addition to brain ischemia. This chapter will present the associative evidence implicating the protein as being involved in neurological diseases, as well as attempt to synthesize potential mechanisms by which mortalin participates in the processes of these conditions.