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Article

Pathomechanisms of epidermolysis bullosa: Beyond structural proteins.

Matrix biology : journal of the International Society for Matrix Biology - 1 Jun 2022

Harvey Nailah, Youssefian Leila, Saeidian Amir Hossein, Vahidnezhad Hassan, Uitto Jouni

Abstract excerpt

Epidermolysis bullosa (EB), a phenotypically and genetically heterogeneous disorder, has been linked to mutations in the genes encoding structural proteins that reinforce skin integrity via dermal-epidermal adhesion. Breakdowns in these adhesion mechanisms result in four different subtypes of EB classified on the basis of the level of tissue separation within the cutaneous basement membrane zone (BMZ). Mutations...

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PMID: 35504439
DOI: 10.1016/j.matbio.2022.04.007

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