Document Detail

LEKTI is localized in lamellar granules, separated from KLK5 and KLK7, and is secreted in the extracellular spaces of the superficial stratum granulosum.
MedLine Citation:
PMID:  15675955     Owner:  NLM     Status:  MEDLINE    
Lympho-epithelial Kazal-type-related inhibitor (LEKTI) is a putative serine protease inhibitor encoded by serine protease inhibitor Kazal-type 5 (SPINK5). It is strongly expressed in differentiated keratinocytes in normal skin but expression is markedly reduced or absent in Netherton syndrome (NS), a severe ichthyosis caused by SPINK5 mutations. At present, however, both the precise intracellular localization and biological roles of LEKTI are not known. To understand the functional role of LEKTI, we examined the localization of LEKTI together with kallikrein (KLK)7 and KLK5, possible targets of LEKTI, in the human epidermis, by confocal laser scanning microscopy and immunoelectron microscopy. In normal skin, LEKTI, KLK7, and KLK5 were all found in the lamellar granule (LG) system, but were separately localized. LEKTI was expressed earlier than KLK7 and KLK5. In NS skin, LEKTI was absent and an abnormal split in the superficial stratum granulosum was seen in three of four cases. Collectively, these results suggest that in normal skin the LG system transports and secretes LEKTI earlier than KLK7 and KLK5 preventing premature loss of stratum corneum integrity/cohesion. Our data provide new insights into the biological functions of LG and the pathogenesis of NS.
Akemi Ishida-Yamamoto; Céline Deraison; Chrystelle Bonnart; Emmanuelle Bitoun; Ross Robinson; Timothy J O'Brien; Kotaro Wakamatsu; Sawa Ohtsubo; Hidetoshi Takahashi; Yoshio Hashimoto; Patricia J C Dopping-Hepenstal; John A McGrath; Hajime Iizuka; Gabriele Richard; Alain Hovnanian
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  The Journal of investigative dermatology     Volume:  124     ISSN:  0022-202X     ISO Abbreviation:  J. Invest. Dermatol.     Publication Date:  2005 Feb 
Date Detail:
Created Date:  2005-01-28     Completed Date:  2005-03-21     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  0426720     Medline TA:  J Invest Dermatol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  360-6     Citation Subset:  IM    
Department of Dermatology, Asahikawa Medical College, Midorigaoka-Higashi 2-1-1-1, Asahikawa 078-8510, Japan.
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MeSH Terms
Carrier Proteins / genetics,  metabolism*,  secretion
Desmosomes / enzymology,  pathology,  ultrastructure
Epidermis / metabolism,  pathology
Extracellular Space / metabolism
Ichthyosis / genetics*,  metabolism*,  pathology
Keratinocytes / enzymology,  pathology
Microscopy, Electron, Transmission
Proteinase Inhibitory Proteins, Secretory
Serine Endopeptidases / metabolism*,  secretion
Grant Support
Reg. No./Substance:
0/Carrier Proteins; 0/Proteinase Inhibitory Proteins, Secretory; 0/SPINK5 protein, human; EC 3.4.21.-/KLK5 protein, human; EC 3.4.21.-/KLK7 protein, human; EC 3.4.21.-/Kallikreins; EC 3.4.21.-/Serine Endopeptidases

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