Loss of proteolytically processed filaggrin caused by epidermal deletion of Matriptase/MT-SP1

doi:10.1083/jcb.200304161

Published 24 November 2003. doi:10.1083/jcb.200304161

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© The Rockefeller University Press, 0021-9525/2003/11/901 $8.00
The Journal of Cell Biology, Volume 163, Number 4, 901-910

Article

Loss of proteolytically processed filaggrin caused by epidermal deletion of Matriptase/MT-SP1

Karin List¹^,6, Roman Szabo¹, Philip W. Wertz³, Julie Segre², Christian C. Haudenschild⁴, Soo-Youl Kim⁵ and Thomas H. Bugge¹

¹ Proteases and Tissue Remodeling Unit, National Institute of Dental and Craniofacial Research
² National Human Genome Research Institute, National Institutes of Health, Bethesda, MD 20892
³ Dows Institute, University of Iowa College of Dentistry, Iowa City, IA 52242
⁴ Department of Experimental Pathology, American Red Cross, Rockville, MD 20855
⁵ Department of Neuroscience, Weill Medical College of Cornell University and Burke Medical Research Institute, White Plains, NY 10605
⁶ Finsen Laboratory, DK-2100 Copenhagen, Denmark

Address correspondence to Thomas H. Bugge, Proteases and Tissue Remodeling Unit, Oral and Pharyngeal Cancer Branch, National Institute of Dental and Craniofacial Research, National Institutes of Health, 30 Convent Drive, Room 211, Bethesda, MD 20892. Tel.: (301) 435-1840. Fax: (301) 402-0823. email: thomas.bugge{at}nih.gov

Profilaggrin is a large epidermal polyprotein that is proteolyticallyprocessed during keratinocyte differentiation to release multiplefilaggrin monomer units as well as a calcium-binding regulatoryNH₂-terminal filaggrin S-100 protein. We show that epidermaldeficiency of the transmembrane serine protease Matriptase/MT-SP1perturbs lipid matrix formation, cornified envelope morphogenesis,and stratum corneum desquamation. Surprisingly, proteomic analysisof Matriptase/MT-SP1–deficient epidermis revealed theselective loss of both proteolytically processed filaggrin monomerunits and the NH₂-terminal filaggrin S-100 regulatory protein.This was associated with a profound accumulation of profilaggrinand aberrant profilaggrin-processing products in the stratumcorneum. The data identify keratinocyte Matriptase/MT-SP1 asan essential component of the profilaggrin-processing pathwayand a key regulator of terminal epidermal differentiation.

Key Words: barrier function; lipid lamellar bodies; membrane serine protease; profilaggrin; stratum corneum

K. List and R. Szabo contributed equally to this paper.

Abbreviations used in this paper: CE, cornified envelope; E,embryonic day.

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