The Fras1/Frem gene family encodes for structurally similar, developmentally regulated extracellular matrix proteins. Mutations in Fras1, Frem1 and Frem2 have been identified in different classes of mouse bleb mutants, while defects in the human orthologs FRAS1 and FREM2 are causative for Fraser syndrome. The hallmark phenotypic feature of bleb mice is embryonic skin blistering due to dermal-epidermal detachment. The similarity of the phenotypic characteristics among the bleb mouse mutants, together with the fact that Fras1/Frem proteins are co-localized in embryonic epithelial basement membranes, suggest that they operate in a common pathway. Here, we report for the first time the immunofluorescence pattern of Frem3 and provide a comparative analysis of the spatiotemporal localization of all Fras1/Frem proteins during mouse embryonic development. We demonstrate their overall co-localization in embryonic epithelial basement membranes, with emphasis on areas of phenotypic interest such as eyelids, limbs, kidneys, lungs and organs of the gastrointestinal tract and the central nervous system. We further studied collagen VII, impairment of which produces dystrophic epidermolysis bullosa, a postnatal skin blistering disorder. We show that basement membrane levels of collagen VII rise at late embryonic life, concomitant with descending Fras1/Frem immunolabeling. © 2006 Elsevier B.V. All rights reserved.
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