Chimienti_2003_Hum.Mol.Genet_12_3017

Reference

Title : Identification of SLURP-1 as an epidermal neuromodulator explains the clinical phenotype of Mal de Meleda - Chimienti_2003_Hum.Mol.Genet_12_3017
Author(s) : Chimienti F , Hogg RC , Plantard L , Lehmann C , Brakch N , Fischer J , Huber M , Bertrand D , Hohl D
Ref : Hum Mol Genet , 12 :3017 , 2003
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.

PubMedSearch : Chimienti_2003_Hum.Mol.Genet_12_3017
PubMedID: 14506129

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Citations formats

Chimienti F, Hogg RC, Plantard L, Lehmann C, Brakch N, Fischer J, Huber M, Bertrand D, Hohl D (2003)
Identification of SLURP-1 as an epidermal neuromodulator explains the clinical phenotype of Mal de Meleda
Hum Mol Genet 12 :3017

Chimienti F, Hogg RC, Plantard L, Lehmann C, Brakch N, Fischer J, Huber M, Bertrand D, Hohl D (2003)
Hum Mol Genet 12 :3017