Mutations in the zinc finger protein 711 gene (ZNF11) have been shown to result in nonspecific mental retardation (MRXZ). Located at Xq21.1-q21.3, the ZNF11 gene is comprised of 7 coding exons in which both point transitions and small deletions have been observed. Mutations in ZNF11 demonstrate X-linked dominant inheritance with variable penetrance in females. Patients with ZNF11 muations present with moderate mental retardation and lack of additional consisten distinctive features.