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Title:

rAAV2-mediated restoration of LEKTI in LEKTI-deficient cells from Netherton patients.

Document type:
Journal Article; Research Support, Non-U.S. Gov't; Article
Author(s):
Roedl, D; Oji, V; Buters, JT; Behrendt, H; Braun-Falco, M
Abstract:
Netherton syndrome (NS, MIM 256500) is a potential live threatening autosomal-recessive skin disorder clinically characterized by the trias of congenital erythroderma, hair shaft anomalies and atopic diathesis. It is caused by mutations in the gene SPINK5 resulting in a deficiency of its processed protein named lympho-epithelial Kazal-type related inhibitor (LEKTI). LEKTI controls the activity of several serine proteases in the skin that are involved in terminal differentiation. Loss of LEKTI re...     »
Journal title abbreviation:
J Dermatol Sci
Year:
2011
Journal volume:
61
Journal issue:
3
Pages contribution:
194-8
Language:
eng
Fulltext / DOI:
doi:10.1016/j.jdermsci.2010.12.004
Pubmed ID:
http://view.ncbi.nlm.nih.gov/pubmed/21251800
Print-ISSN:
0923-1811
TUM Institution:
Molekulare Allergologie (Prof. Schmidt-Weber)
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