Syndecan-1 interaction with the LG4/5 domain in laminin-332 is essential for keratinocyte migration

2007 ◽  
Vol 214 (1) ◽  
pp. 238-249 ◽  
Author(s):  
Sophie Bachy ◽  
François Letourneur ◽  
Patricia Rousselle
2008 ◽  
Vol 121 (18) ◽  
pp. 2992-3001 ◽  
Author(s):  
G. Fitsialos ◽  
I. Bourget ◽  
S. Augier ◽  
A. Ginouves ◽  
R. Rezzonico ◽  
...  

Genes ◽  
2021 ◽  
Vol 12 (5) ◽  
pp. 716
Author(s):  
Daniele Castiglia ◽  
Paola Fortugno ◽  
Angelo Giuseppe Condorelli ◽  
Sabina Barresi ◽  
Naomi De Luca ◽  
...  

Junctional epidermolysis bullosa (JEB) is a clinically and genetically heterogeneous skin fragility disorder frequently caused by mutations in genes encoding the epithelial laminin isoform, laminin-332. JEB patients also present mucosal involvement, including painful corneal lesions. Recurrent corneal abrasions may lead to corneal opacities and visual impairment. Current treatments are merely supportive. We report a novel JEB phenotype distinguished by the complete resolution of skin fragility in infancy and persistent ocular involvement with unremitting and painful corneal abrasions. Biallelic LAMB3 mutations c.3052-5C>G and c.3492_3493delCG were identified as the molecular basis for this phenotype, with one mutation being a hypomorphic splice variant that allows residual wild-type laminin-332 production. The reduced laminin-332 level was associated with impaired keratinocyte adhesion. Then, we also investigated the therapeutic power of a human amniotic membrane (AM) eyedrop preparation for corneal lesions. AM were isolated from placenta donors, according to a procedure preserving the AM biological characteristics as a tissue, and confirmed to contain laminin-332. We found that AM eyedrop preparation could restore keratinocyte adhesion in an in vitro assay. Of note, AM eyedrop administration to the patient resulted in long-lasting remission of her ocular manifestations. Our findings suggest that AM eyedrops could represent an effective, non-invasive, simple-to-handle treatment for corneal lesions in patients with JEB and possibly other EB forms.


2008 ◽  
Vol 104 (3) ◽  
pp. 934-941 ◽  
Author(s):  
Young Seomun ◽  
Jong-Tak Kim ◽  
Choun-Ki Joo

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