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Article

The extracellular matrix gene Frem1 is essential for the normal adhesion of the embryonic epidermis.

Proceedings of the National Academy of Sciences of the United States of America - 14 Sept 2004

Smyth Ian, Du Xin, Taylor Martin S, Justice Monica J, Beutler Bruce, Jackson Ian J

Abstract excerpt

Fraser syndrome is a rare recessive disorder characterized by cryptophthalmos, syndactyly, renal defects, and a range of other developmental abnormalities. Because of their extensive phenotypic overlap, the mouse blebbing mutants have been considered models of this disorder, and the recent isolat...

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Share to Basement Membrane
Share to Cell Adhesion
Share to Collagen Type VI

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Embryo, Mammalian

Share to Epidermal Cells

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Share to Extracellular Matrix Proteins

Share to Gene Expression Regulation, Developmental

Source

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Identifiers

PMID: 15345741
DOI: 10.1073/pnas.0402760101
PMCID: PMC518794

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 2 Sept 2026. Prepared record publication-15345741-2026-09-02.2. Manifest publication-graph-cohort-2026-09-02.2.

Source receipt: PubMed via Helena Literature Corpus, retrieved 2 Sept 2026.

Related research

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The extracellular matrix gene Frem1 is essential for the normal adhesion of the embryonic epidermis.PMID 15345741Read ↗

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

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

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

Novel frem1-related mouse phenotypes and evidence of genetic interactions with gata4 and slit3.Semantic proximity Read ↗

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

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

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

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

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

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