Incontinentia pigmenti (IP) also known as Bloch-Sulzberger syndrome is an X-chromosome-linked genodermatosis caused by mutations of nuclear factor (NF)-kB essential modulator (NEMO) gene at Xq28. The vast majority of NEMO mutations involve identical deletion of exons 4-10 (ANEMO) causing IP [Aradhya et al., 2001]. The NEMO locus contains an inversely oriented, truncated and nonfunctional NEMO pseudogene that plays an essential role in de novo recurrent formation of the ANEMO mutation [Bardaro et al., 20031
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