ABSTRACT
Monogenic conditions are responsible for only a minority of cases of dementia. However, they may provide important insights into the more common sporadic forms. Thus, for example, cloning of the genes for familial Alzheimer’s disease and cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) has revealed pathways involved in the degeneration of neurons and vascular smooth muscle cells (VSMC), respectively (Sisodia and George-Hyslop, 2002; Wang et al, 2002a). Studies with transgenic animals and cells overexpressing the mutant proteins have led to new therapeutic strategies (Fassbender et al, 2001; Schenk, 2002; Wang et al, 2002a). Some of these strategies are currently tested in sporadic disease (Simons et al, 2002).
