Mitochondrial proteome design: From molecular identity to pathophysiological regulation

Introduction The essence of the mitochondria lies within their ability to generate energy, maintain metabolisms, and regulate signaling cascades. As fundamental constituents of numerous rudimentary cellular processes, the molecular architecture and the proteome of the mitochondria are critical to intracellular biochemistry. The behavior of the mitochondria is subjugated to the environment of this organelle; perturbation of mitochondrial homeostasis can prompt pathophysiological conditions to arise. The history of mitochondrial research can be traced back to Rudolf Albrecht von Kolliker in 1857 when he first described the mitochondrion. In 1890, Richard Altmann termed the mitochondria “bioblasts,” noted their ubiquitous nature, and showed exceptional foresight when he explained that these bioblasts were living inside cells and were responsible for “elementary functions.” The endosymbiotic theory was first articulated by Konstantin Mereschkowski in 1905. Although several hypotheses were put forth to explain the merger between mitochondria and cells, it was Lynn Margulis who tied the endosymbiotic theory to biochemical and cytological evidence. Mitochondria are of -protobacterial origin, engulfed by cells through endosymbiosis about two billion years ago. The name “mitochondrion” was first introduced in 1898 by Carl Benda from the Greek words “mitos” (thread) and “chondros” (granule). The first