ARS Component B: structural characterization, tissue expression and regulation of the gene and protein (SLURP-1) associated with Mal de Meleda

Eur J Dermatol. 2003 Nov-Dec;13(6):560-70.

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.

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Antigens, Ly / genetics*
  • Cells, Cultured
  • Humans
  • Immunohistochemistry
  • Keratinocytes / metabolism*
  • Keratoderma, Palmoplantar / genetics*
  • Keratoderma, Palmoplantar / metabolism
  • Mice
  • Mice, Inbred BALB C
  • Molecular Sequence Data
  • Molecular Structure
  • RNA, Messenger / analysis
  • Reverse Transcriptase Polymerase Chain Reaction
  • Urokinase-Type Plasminogen Activator / genetics*

Substances

  • Antigens, Ly
  • RNA, Messenger
  • SLURP1 protein, human
  • Urokinase-Type Plasminogen Activator