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Article

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

The Journal of biological chemistry - 18 Mar 2005

Petrou Petros, Pavlakis Evangelos, Dalezios Yannis, Galanopoulos Vassilis K, Chalepakis Georges

Abstract excerpt

Fras1 is a putative extracellular matrix protein that has been implicated in the structural adhesion of embryonic epidermis to dermis. Moreover, mutations in Fras1/FRAS1 have been associated with the mouse blebbed phenotype and the human rare genetic disorder Fraser syndrome, respectively. Here we report the mapping of Fras1 within the extracellular space and evaluate the effects of Fras1 deficiency on lung...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

Identifiers

PMID: 15623520
DOI: 10.1074/jbc.M412368200

Genes

FRAS1

Provenance

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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Basement membrane distortions impair lung lobation and capillary organization in the mouse model for fraser syndrome.PMID 15623520Read ↗

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

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Generation of mice with a conditional null Fraser syndrome 1 (Fras1) allele.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 ↗

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

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

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