Mitochondrial complex I deficiency leads to the retardation of early embryonic development in knockout mice.

Background: The gene encodes an 18-kD subunit of mitochondria complex I, and mutations in this gene lead to the development of a severe neurodegenerative disease called Leigh syndrome (LS) in humans. To investigate the disease phenotypes and molecular mechanisms of Leigh syndrome, the knockout (KO) mouse has been widely used as a novel animal model. Because the homozygotes cannot survive beyond child-bearing age, whether and mitochondrial complex I influence early embryonic development remains unknown.