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Article

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

The international journal of biochemistry & cell biology - 1 Apr 2011

Pavlakis Evangelos, Chiotaki Rena, Chalepakis Georges

Abstract excerpt

Basement membranes constitute architecturally complex extracellular matrix (ECM) protein networks of great structural and regulatory importance. Recently, a novel group of basement membrane proteins, Fras1 (Fraser syndrome protein (1) and the Fras1-related extracellular matrix proteins Frem1, Frem2 and Frem3, has emerged. They comprise components of the sublamina densa region and contribute to embryonic...

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Source

Identifiers

PMID: 21182980
DOI: 10.1016/j.biocel.2010.12.016

Genes

FRAS1
FREM2

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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The role of Fras1/Frem proteins in the structure and function of basement membrane.PMID 21182980Read ↗

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

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

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

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

Basement membrane localization of Frem3 is independent of the Fras1/Frem1/Frem2 protein complex within the sublamina densa.Semantic proximity Read ↗

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

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

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

The genetics of Fraser syndrome and the blebs mouse mutantsSemantic 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 ↗

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