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Article

The Fras1/Frem family of extracellular matrix proteins: structure, function, and association with Fraser syndrome and the mouse bleb phenotype.

Connective tissue research - 1 Jan 2008

Petrou Petros, Makrygiannis Apostolos K, Chalepakis Georges

Abstract excerpt

Fras1 and the structurally related proteins Frem1, Frem2, and Frem3, comprise a novel family of extracellular matrix proteins, which localize in a similar fashion underneath the lamina densa of epithelial basement membranes. They are involved in the structural adhesion of the skin epithelium to its underlying mesenchyme. Deficiency in the individual murine Fras1/Frem genes gives rise to the bleb phenotype, which...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 18661360
DOI: 10.1080/03008200802148025

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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The Fras1/Frem family of extracellular matrix proteins: structure, function, and association with Fraser syndrome and the mouse bleb phenotype.PMID 18661360Read ↗

The role of Fras1/Frem proteins in the structure and function of basement membrane.Semantic proximity Read ↗

Let's stick together: The role of the Fras1 and Frem proteins in epidermal adhesionSemantic proximity Read ↗

Spatiotemporal distribution of Fras1/Frem proteins during mouse embryonic development.Semantic proximity Read ↗

Breakdown of the reciprocal stabilization of QBRICK/Frem1, Fras1, and Frem2 at the basement membrane provokes Fraser syndrome-like defects.Semantic proximity Read ↗

The extracellular matrix gene Frem1 is essential for the normal adhesion of the embryonic epidermis.Semantic proximity Read ↗

Fraser syndrome and mouse blebbed phenotype caused by mutations in FRAS1/Fras1 encoding a putative extracellular matrix protein.Semantic proximity Read ↗

Fras1 deficiency results in cryptophthalmos, renal agenesis and blebbed phenotype in mice.Semantic proximity Read ↗

The genetics of Fraser syndrome and the blebs mouse mutantsSemantic proximity Read ↗

bfb, a novel ENU-induced blebs mutant resulting from a missense mutation in Fras1.Semantic proximity Read ↗

Basement membrane distortions impair lung lobation and capillary organization in the mouse model for fraser syndrome.Semantic proximity Read ↗

AMACO is a component of the basement membrane-associated Fraser complex.Semantic proximity Read ↗

<i>Frem2</i> Knockout Mice Exhibit Fraser Syndrome Phenotypes and Neonatal Lethality Due to Bilateral Renal AgenesisSemantic proximity Read ↗

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