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RESEARCH COMMUNICATION

Epidermal detachment, desmosomal dissociation, and destabilization of corneodesmosin in Spink5^-/- mice

¹ Department of Molecular and Cellular Biology, ² Department of Molecular and Human Genetics, ³ Department of Dermatology, ⁴ Biology of Inflammation Center and Department of Medicine, Baylor College of Medicine, Houston, Texas 77030, USA; ⁵ Laboratory for Cutaneous Biology, Dermatology Unit, Beaumont Hospital, CHUV, Lausanne CH-1011, Switzerland

Netherton syndrome (NS) is a human autosomal recessive skin disease caused by mutations in the SPINK5 gene, which encodes the putative proteinase inhibitor LEKTI. We have generated a transgenic mouse line with an insertional mutation that inactivated the mouse SPINK5 ortholog. Mutant mice exhibit fragile stratum corneum and perinatal death due to dehydration. Our analysis suggests that the phenotype is a consequence of desmosomal fragility associated with premature proteolysis of corneodesmosin, an extracellular desmosomal component. Our mouse mutant provides a model system for molecular studies of desmosomal stability and keratinocyte adhesion, and for designing therapeutic strategies to treat NS.

[Keywords: Spink5; LektI; proteinase inhibitor; Netherton Syndrome; desmosomes; Cdsn]

Received June 17, 2004; revised version accepted August 6, 2004.

Article and publication are at http://www.genesdev.org/cgi/doi/10.1101/gad.1232104.

Corresponding authors.

⁶ E-MAIL taoy{at}bcm.tmc.edu; FAX (713) 798-5168.

⁷ E-MAIL overbeek{at}bcm.tmc.edu; FAX (713) 798-7819.

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