The Functions of Mitochondrial 2',3'-Cyclic Nucleotide-3'-Phosphodiesterase and Prospects for Its Future.

2',3'-cyclic nucleotide-3'-phosphodiesterase (CNPase) is a myelin-associated enzyme that catalyzes the phosphodiester hydrolysis of 2',3'-cyclic nucleotides to 2'-nucleotides. However, its presence is also found in unmyelinated cells and other cellular structures. Understanding of its specific physiological functions, particularly in unmyelinated cells, is still incomplete. This review concentrates on the role of mitochondrial CNPase (mtCNPase), independent of myelin. mtCNPase is able to regulate the functioning of the mitochondrial permeability transition pore (mPTP), and thus is involved in the mechanisms of cell death, both apoptosis and necrosis. Its participation in the development of various diseases and pathological conditions, such as aging, heart disease and alcohol dependence, is also reviewed. As such, mtCNPase can be considered as a potential target for the development of therapeutic strategies in the treatment of mitochondria-related diseases.