First, in 2005, a novel mutation of MGP associated with high level of inactive dp-ucMGP is described and associated with neurological manifestations, abnormalities of brain’s white matter and optic nerve atrophy, in addition to typical manifestations of Keutel syndrome [13, 14], suggesting a link between MGP activity and nervous system pathophysiology. The gene discussed is MGP; the disease is Keutel syndrome.