Genomic Organization of the Mottled Gene, the Mouse Homologue of the Human Menkes Disease Gene

The mouse homologue of the Menkes gene has been shown to span 120 kb of genomic DNA and to be similar in structure to both its human MNK homologue (ATP7A) and the Wilson disease gene (WD;ATP7B). Conservation of the majority of intron/exon boundaries among the three genes was also observed. The high overall conservation of both theAtp7agene and the direction of transcription of theAtp7a, Pgk1,andXnpgenes between human and mouse is compatible with the evolution of an ancestral gene subject to strong evolutionary constraints lying within a locally relatively conserved region of the X chromosome.