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Document type:
journal article 
Author(s):
Roedl, D; Oji, V; Buters, JT; Behrendt, H; Braun-Falco, M 
Title:
rAAV2-mediated restoration of LEKTI in LEKTI-deficient cells from Netherton patients. 
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:
Pages contribution:
194-8 
Language:
eng 
Print-ISSN:
0923-1811 
TUM Institution:
Institut für Molekulare Allergologie und Umweltforschung