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• W2896667967 endingPage "84" @default.
• W2896667967 startingPage "72" @default.
• W2896667967 abstract "The behavior of a cell depends on how its adhesion molecules interact with the cellular microenvironment. Hemidesmosomal collagen XVII essentially contributes to cell adhesion and modulates keratinocyte directionality and proliferation during skin regeneration, however only little is known about the involved interactions. Here, we used keratinocytes from patients with junctional epidermolysis bullosa with late onset, which exclusively produce a collagen XVII mutant with the p.R1303Q mutation within its extracellular C-terminus. Although this mutant was normally expressed and targeted to the membrane and the expression of integrins α3β1, α6β4 and of laminin-332 was unchanged, the keratinocytes were less adhesive, showed migratory defects and decreased clonogenic growth. Since the p.R1303Q substitution is located within the predicted laminin-332 binding site of collagen XVII, we anticipated an altered collagen XVII-laminin-332 interaction. Indeed, the pR1303Q collagen XVII ectodomain showed decreased binding capability to laminin-332 and was less co-localized with pericellular laminin-332 molecules in cell culture. Thus, aberrant collagen XVII-laminin-332 interaction results in reduced cell adhesion, destabilized cell motility and decreased clonogenicity, which in turn lead to blister formation, delayed wound healing and skin atrophy." @default.
• W2896667967 created "2018-10-26" @default.
• W2896667967 creator A5036244835 @default.
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• W2896667967 creator A5088114081 @default.
• W2896667967 date "2019-07-01" @default.
• W2896667967 modified "2023-10-16" @default.
• W2896667967 title "Amino acid substitution in the C-terminal domain of collagen XVII reduces laminin-332 interaction causing mild skin fragility with atrophic scarring" @default.
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• W2896667967 doi "https://doi.org/10.1016/j.matbio.2018.10.003" @default.
• W2896667967 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/30316981" @default.
• W2896667967 hasPublicationYear "2019" @default.
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