101. ARS Component B: structural characterization, tissue expression and regulation of the gene and protein (SLURP-1) associated with Mal de Meleda
- Author
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Mastrangeli, R., Donini, S., Kelton, C. A., C. M., He, Bressan, A., Milazzo, F., Ciolli, V., Borrelli, F., Martelli, F., Biffoni, M., Serlupi Crescenzi, O., Serani, S., Micangeli, E., El Tayar, N., Vaccaro, Rosa, Renda, Tindaro Giuseppe, Lisciani, R., Rossi, M., and Papoian, R.
- Subjects
Keratinocytes ,mal de meleda ,Mice, Inbred BALB C ,hyperkeratosis ,Molecular Structure ,Reverse Transcriptase Polymerase Chain Reaction ,slurp-1 ,Molecular Sequence Data ,ars component b ,keratinocytes ,Immunohistochemistry ,Urokinase-Type Plasminogen Activator ,Mice ,Keratoderma, Palmoplantar ,Animals ,Antigens, Ly ,Humans ,Amino Acid Sequence ,RNA, Messenger ,Cells, Cultured - Abstract
The ARS Component B gene (EMBL ID: HSARS81S, AC: X99977) encodes a 9 kD non-glycosylated polypeptide (also known as SLURP-1, SwissProt/TrEMBL: P55000), a soluble member of the human Ly6/uPAR superfamily. ARS Component B gene mutations have been implicated in Mal de Meleda. In this study we show by immunohistochemistry that SLURP-1 (secreted Ly-6/uPAR related protein, the protein product of the ARS Component B gene) is localized to human skin, exocervix, gums, stomach and esophagus. In the epidermis, keratinocytes underlying the stratum corneum are highly positive for SLURP1 immunostaining and cultured keratinocytes secrete the expected 9 kD protein. Circulating SLURP1 is detected in human plasma and urine. In the mouse, expression is evident in skin, eye, whole lung, trachea, esophagus and stomach. Human ARS Component B mRNA expression is regulated by retinoic acid, epidermal growth factor and interferon-gamma. The tissue localization and the association with Mal de Meleda suggest that ARS Component B and its protein product SLURP1 are implicated in maintaining the physiological and structural integrity of the keratinocyte layers of the skin.
- Published
- 2004