Among these cofactors, PBX1 is of great interest in regards to malformations found in MRKH syndrome: it is required for skeletal development and patterning [27], kidney morphogenesis [28] and especially, its gene inactivation leads to absence of Müllerian structures [29]. The gene discussed is PBX1; the disease is Mayer-Rokitansky-Kuster-Hauser syndrome.