Pathological aggregation of endogenous proteins is a common feature of many neurodegenerative
diseases. This is generally accompanied by elevated levels of oxidative stress associated
with transition metal dyshomeostasis. As such, strategies targeted toward rectifying metal imbalance
are increasingly becoming an attractive therapeutic option. One class of compound showing such
therapeutic potential are the bis(thiosemicarbazone) metal complexes. These are small, orally
bioavailable compounds capable of crossing the blood brain barrier and capable of delivering
bioavailable metal intracellularly. Members of this family of compounds have been shown to successfully
treat animal models of several neurodegenerative diseases such as Alzheimer’s disease, Parkinson’s
disease and amyotrophic lateral sclerosis. Here we review the current evidence for the efficacy
of bis(thiosemicarbazone) metal complexes in treating these diseases and discuss the implications for
future development of these compounds.
Keywords: bis(thiosemicarbazone), BTSC, ALS, CuII(atsm), CUII(gtsm), AD.
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