The Tunisian family pedigree shows cosegregation of the 3 carboxylase mutations with VKD coagulation factor deficiency. Affected and unaffected subjects are indicated by solid or open symbols, respectively. The arrow points to the proposita. The female indicated by the hatched circle was dead in early infancy for unknown reasons. Mutated residues are in red, and the chromosomal colors indicate maternal (blue) or paternal (black) inheritance.