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A Decade of Progress

Arch Dermatol. 2003;139:74-77.

	Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings.

OUR UNDERSTANDING of the molecular basis of genetic disorders of skin and mosaic conditions has progressed tremendously in the past 100 years and particularly during the past decade. Approximately 10 years ago, a transgenic mouse model of a genodermatosis was engineered in the Chicago area with a keratin 14 deletion,¹ which was noted to resemble that of patients with the Dowling-Meara form of epidermolysis bullosa (EB).² Through the collaboration of basic scientists, physician-scientists, and clinicians, the underlying basis of most genodermatoses has now been determined. These subsequent discoveries have been based on the mapping of genes to specific chromosomal loci, other transgenic and knockout mouse models, and direct sequencing of genomic DNA from affected patients.

Based on the understanding that keratin gene expression is site specific and that the sites of phenotypic expression should mirror the pattern of gene expression, the molecular basis of several other genodermatoses due to keratin . . . [Full Text of this Article]