title: ARS Component B: structural characterization, tissue expression and regulation of the gene and protein (SLURP-1) associated with Mal de Meleda.
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.
authors.affiliation: Industria Farmaceutica Serono S.p.A Ardea site, 00040, Rome, Italy.
authors.forename: Renato
authors.initials: R
authors.lastname: Mastrangeli
authors.forename: Silvia
authors.initials: S
authors.lastname: Donini
authors.forename: Christie A
authors.initials: CA
authors.lastname: Kelton
authors.forename: Chaomei
authors.initials: C
authors.lastname: He
authors.forename: Alessandro
authors.initials: A
authors.lastname: Bressan
authors.forename: Ferdinando
authors.initials: F
authors.lastname: Milazzo
authors.forename: Veniero
authors.initials: V
authors.lastname: Ciolli
authors.forename: Francesco
authors.initials: F
authors.lastname: Borrelli
authors.forename: Fabrizio
authors.initials: F
authors.lastname: Martelli
authors.forename: Mauro
authors.initials: M
authors.lastname: Biffoni
authors.forename: Ottaviano
authors.initials: O
authors.lastname: Serlupi-Crescenzi
authors.forename: Serenella
authors.initials: S
authors.lastname: Serani
authors.forename: Emilia
authors.initials: E
authors.lastname: Micangeli
authors.forename: Nabil
authors.initials: N
authors.lastname: El Tayar
authors.forename: Rosa
authors.initials: R
authors.lastname: Vaccaro
authors.forename: Tindaro
authors.initials: T
authors.lastname: Renda
authors.forename: Romeo
authors.initials: R
authors.lastname: Lisciani
authors.forename: Mara
authors.initials: M
authors.lastname: Rossi
authors.forename: Ruben
authors.initials: R
authors.lastname: Papoian
mesh_terms: D000595:Amino Acid Sequence; D000818:Animals; D000950:Antigens, Ly / Q000235:genetics*; D002478:Cells, Cultured; D006801:Humans; D007150:Immunohistochemistry; D015603:Keratinocytes / Q000378:metabolism*; D007645:Keratoderma, Palmoplantar / Q000235:genetics* / Q000378:metabolism; D051379:Mice; D008807:Mice, Inbred BALB C; D008969:Molecular Sequence Data; D015394:Molecular Structure; D012333:RNA, Messenger / Q000032:analysis; D020133:Reverse Transcriptase Polymerase Chain Reaction; D014568:Urokinase-Type Plasminogen Activator / Q000235:genetics*
chemical_list: D000950:Antigens, Ly; D012333:RNA, Messenger; C048963:SLURP1 protein, human; D014568:Urokinase-Type Plasminogen Activator