Recent efforts—including our MitoCarta work—have provided a map of mitochondrial proteins and their sub-mitochondrial locations. Yet, hundreds of these proteins remain poorly characterized, limiting our understanding of basic mitochondrial function and pathophysiology. To address this, we are blending modern large-scale and classical reductionist approaches—a process we refer to as ‘‘systems biochemistry’’—to link these mitochondrial uncharacterized (x) proteins (MXPs) to specific pathways and processes. This work is helping to identify new disease-related mitochondrial proteins and long-sought ‘‘missing’’ proteins that fulfill key functions. Collectively, these studies are moving us toward a more complete understanding of mitochondrial activities and providing a molecular framework for the investigation of mitochondrial pathogenesis.
Selected Recent Publications