Cardiac Myopathy In Conditional Hsp60 Transgenic Mice

The mitochondrial chaperonin Hsp60 (Hspd1) plays important roles in sustaining cellular viability, regulate cellular functions and maintain homeostasis. Mutations in the Hsp60 gene or erratic expression has been frequently observed in wide-ranging human diseases. Targeting Hsp60 to ameliorate the prognosis of mitochondrial dysfunction-related diseases were proposed in the past. Genetically engineered mice provide a compelling tool to investigate the aetiology and pathogenesis of these diseases. Eventually, this will benefit the development of therapeutics towards these physiological complications. Conventional Hsp60 transgenic mice are often neonatally lethal. We've generated a unique conditional Hsp60 transgenic (Tg) mouse model to investigate the mitochondrial activities and demonstrated that ubiquitous expression of human Hsp60 protein in mice leads to neonatal death due to septum defect (ASD) and cardiac myopathy. This chapter concisely reviews recent advances regarding manipulating Hsp60 levels in cells and mouse models along with depicts our quest to develop transgenic mice to study Hsp60-related human diseases.