Identification of SLURP-1 as an epidermal neuromodulator explains the clinical phenotype of Mal de Meleda

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Serval ID
serval:BIB_42136D8EED7F
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Identification of SLURP-1 as an epidermal neuromodulator explains the clinical phenotype of Mal de Meleda
Journal
Human Molecular Genetics
Author(s)
Chimienti  F., Hogg  R. C., Plantard  L., Lehmann  C., Brakch  N., Fischer  J., Huber  M., Bertrand  D., Hohl  D.
ISSN
0964-6906 (Print)
Publication state
Published
Issued date
11/2003
Volume
12
Number
22
Pages
3017-24
Notes
Journal Article
Research Support, Non-U.S. Gov't --- Old month value: Nov 15
Abstract
Mal de Meleda is an autosomal recessive inflammatory and keratotic palmoplantar skin disorder due to mutations in the ARS B gene, encoding for SLURP-1 (secreted mammalian Ly-6/uPAR-related protein 1). SLURP-1 belongs to the Ly-6/uPAR superfamily of receptor and secreted proteins, which participate in signal transduction, immune cell activation or cellular adhesion. The high degree of structural similarity between SLURP-1 and the three fingers motif of snake neurotoxins and Lynx1 suggests that this protein interacts with the neuronal acetylcholine receptors. We found that SLURP-1 potentiates the human alpha 7 nicotinic acetylcholine receptors that are present in keratinocytes. These results identify SLURP-1 as a secreted epidermal neuromodulator which is likely to be essential for both epidermal homeostasis and inhibition of TNF-alpha release by macrophages during wound healing. This explains both the hyperproliferative as well as the inflammatory clinical phenotype of Mal de Meleda.
Keywords
Acetylcholine/metabolism Amino Acid Sequence Animals Antigens, Ly/chemistry/*genetics/isolation & purification/pharmacology Cell Line Cell Nucleus/metabolism Clone Cells DNA, Complementary/administration & dosage/metabolism Dose-Response Relationship, Drug Epidermis/*metabolism Female Genes, Recessive Humans Keratoderma, Palmoplantar/*genetics/metabolism/pathology Microinjections Models, Molecular Moths/cytology Mutation Neurotransmitter Agents/*metabolism Oocytes/metabolism Patch-Clamp Techniques Peptides/chemistry/genetics/metabolism Phenotype Protein Structure, Tertiary Receptors, Cholinergic/drug effects/metabolism Recombinant Proteins/isolation & purification/metabolism Urinary Plasminogen Activator/chemistry/*genetics/isolation & purification/pharmacology Xenopus laevis/physiology
Pubmed
Web of science
Open Access
Yes
Create date
28/01/2008 11:34
Last modification date
25/09/2019 7:09
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