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 Printable version |
ARS Component B: structural characterization, tissue expression and regulation of the gene and protein (SLURP‐1) associated with Mal de Meleda |
European Journal of Dermatology. Volume 13, Number 6, 560-70, November - December 2003, Investigative report
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 Free Article
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Author(s) : Renato MASTRANGELI, Silvia DONINI, Christie A. KELTON, Chaomei HE, Alessandro BRESSAN, Ferdinando MILAZZO, Veniero CIOLLI, Francesco BORRELLI, Fabrizio MARTELLI, Mauro BIFFONI, Ottaviano SERLUPI‐CRESCENZI, Serenella SERANI, Emilia MICANGELI, Nabil EL TAYAR, Rosa VACCARO, Tindaro RENDA, Romeo LISCIANI, Mara ROSSI, Ruben PAPOIAN |
Summary : 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 Ly‐6\\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 SLURP‐1 immunostaining and cultured keratinocytes secrete the expected 9kD protein. Circulating SLURP‐1 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‐γ. The tissue localization and the association with Mal de Meleda suggest that
ARS Component B and its protein product SLURP‐1 are implicated in maintaining the physiological and structural integrity of the keratinocyte layers of the skin. |
Keywords : ARS Component B,
hyperkeratosis, keratinocytes, Mal de Meleda, SLURP‐1 |
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