Netherton syndrome is caused by homozygous or compound heterozygous mutations in the SPINK5 gene, which encodes the serine protease inhibitor LEKTI
(lymphoepithelial Kazal-type-related inhibitor), on chromosome 5q32 (2).
Immunohistochemistry using specific antibodies showed complete absence of lymphoepithelial Kazal-type-related inhibitor (LEKTI) in the biopsy specimen.
The gene encodes a serine protein kinase known as lymphoepithelial Kazal-type inhibitor (LEKTI), expressed in mucosal and epithelial surface [2-4].
The proteolytic activity is dependent on pH in the SC and is regulated by a cocktail of protease inhibitors, including lymphoepithelial Kazal-type 5 serine protease inhibitor (LEKTI) encoded by the serine protease inhibitor Kazal-type 5 (SPINK5) .
Lopez et al., "LEKTI fragments specifically inhibit KLK5, KLK7, and KLK14 and control desquamation through a pH-dependent interaction," Molecular Biology of the Cell, vol.
FLG, its major processing proteases and key protease inhibitor lymphoepithelial Kazal-type-related inhibitor (LEKTI) were measured by both real-time quantitative polymerase chain reaction and Western blotting.
FLG has several site-specific functions governed by the program of epidermal terminal differentiation in which a number of proteases are involved, such as corneum trypsin- and chymotrypsin-like enzymes (stratum corneum tryptic enzyme/kallikrein 5 [KLK5] and stratum corneum chymotryptic enzyme/KLK7),[sup],, channel-activating serine protease1 (CAP1),[sup] matriptase (coded by ST14),[sup] and cysteine protease caspase-14[sup] and their key protease inhibitor lymphoepithelial Kazal-type-related inhibitor (LEKTI) (encoded by serine protease inhibitor Kazal-type 5 gene/ SPINK5 ).[sup],, All of the proteases can enhance FLG degradation processing.
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In Netherton syndrome, mutations occur in SPINK5, a serine protease inhibitor that encodes a critical serine protease inhibitor, lymphoepithelial-Kazal-type 5 inhibitor (LEKTI
In Netherton syndrome, mutations occur in 5PINK5, a serine protease inhibitor that encodes a critical serine protease inhibitor, lymphoepithelial-Kazal-type 5 inhibitor (LEKTI
However, other studies, using appropriate controls, have found no relation between diversity and trust, either at the cross-national level (Green, Preston and Janmaat, 2006; Hooghe et al., 2009) or in cross-area analyses in Canada (Johnson and Soroka, 1999) and the UK (Lekti