Conversely, the heterozygote knockout mice for the Prkar1a gene (Prkar1a+/− mice), which mimic the Prkar1a subunit haploinsufficiency of the CNC, developed, in about 80% of cases, tumoral lesions on the tail vertebrae and the sacroiliac region, which arise from cells of the osteoblast lineages [39] and present a histological and cytological similarity to the osteochondromyxomas that develop in human CNC patients. Here, PRKAR1A is linked to Carney complex.