Reduced Stability and Bi-Allelic, Coequal Expression of Profilaggrin mRNA in Keratinocytes Cultured From Subjects With Ichthyosis Vulgaris

Wilas Nirunsuksiri, She-Hui Zhang, Philip Fleckman
1998 Journal of Investigative Dermatology  
Ichthyosis vulgaris (IV) is an inherited scaling skin disorder in which expression of profilaggrin is reduced. Previous studies have indicated that the reduction is caused by defective post-transcriptional control of gene expression. Here we present evidence that profilaggrin mRNA in keratinocytes cultured from subjects with IV is intrinsically unstable and has a shorter half-life compared with that in normal cells. When IV-affected keratinocytes were treated with the protein synthesis
more » ... synthesis inhibitor cycloheximide, the steady-state level of profilaggrin mRNA was increased due to stabilization of the transcript. In addi-
doi:10.1046/j.1523-1747.1998.00208.x pmid:9620289 fatcat:rjyv33xwzra7ffjof2oxtos6k4