Copper (Cu) is essential in the biological redox reaction for the normal function in a body, and its deficiency is fatal to a body. At the same time Cu is extremely toxic when it exists in excess. Thus, a body has to regulate the concentration of Cu within a certain range by several groups of Cu-regulating proteins. If the strict regulation of Cu in a body is disordered, the clinical symptoms are manifested. The knowledge for the mechanisms of Cu disorder in Wilson disease, Menkes disease and their animal models largely contributed to reveal the mechanisms of normal Cu homeostasis and the roles of Cu-regulating protein in the recent two decades. However, entire mechanisms underlying the maintenance of Cu homeostasis in a body and cells have not fully understood. Analytical techniques such as speciation and imaging of Cu also contributed to obtain unique knowledge for the Cu metabolisms. In this presentation, recent advances in the Cu metabolism and pathogenic progressions of Cu-relating disorders obtained by the analytical techniques will be highlighted.
Contact local IBMM : Prof. Christine Enjalbal (DAPP)