| Behavioural effects of extracellular matrix protein Fras1 depletion in the mouse. | Behavioural effects of extracellular matrix protein Fras1 depletion in the mouse.
Kalpachidou T, Makrygiannis AK, Pavlakis E, Stylianopoulou F, Chalepakis G, Stamatakis A. | 08/21/2021 |
| The close correlation between limb bud epidermal blistering, decreased Msx2 expression, and reduced ICD in the Fras1(rdf) hindlimb buds suggests that epithelium detachment from the mesenchyme may create a physical gap that interrupts the transmission of BMP, among other signals, resulting in soft tissue syndactyly. | Syndactyly in a novel Fras1(rdf) mutant results from interruption of signals for interdigital apoptosis.
Hines EA, Verheyden JM, Lashua AJ, Larson SC, Branchfield K, Domyan ET, Gao J, Harvey JF, Herriges JC, Hu L, Mcculley DJ, Throckmorton K, Yokoyama S, Ikeda A, Xu G, Sun X., Free PMC Article | 12/17/2016 |
| the role of Fras1 in development | bfb, a novel ENU-induced blebs mutant resulting from a missense mutation in Fras1.
Miller KA, Gordon CT, Welfare MF, Caruana G, Bertram JF, Bateman JF, Farlie PG., Free PMC Article | 06/21/2014 |
| Targeted deletion of Fras1 in kidney podocytes circumvented skin blistering, renal agenesis, and early death. | Generation of mice with a conditional null Fraser syndrome 1 (Fras1) allele.
Pitera JE, Turmaine M, Woolf AS, Scambler PJ. | 06/22/2013 |
| Report Fras1 up-regulation/Frem down-regulation in nephrons from mice with polycystic kidney disease and Frem2 mutations. | Expression of Fraser syndrome genes in normal and polycystic murine kidneys.
Kerecuk L, Long DA, Ali Z, Anders C, Kolatsi-Joannou M, Scambler PJ, Woolf AS., Free PMC Article | 08/25/2012 |
| The QBRICK, together with Fras1, defines the ability of BMs to bind integrin 81 through modulating the BM assembly of nephronectin, thereby regulating the integrin 81-mediated signals transmitted from the ureteric buds to the metanephric mesenchyme. | Basement membrane assembly of the integrin α8β1 ligand nephronectin requires Fraser syndrome-associated proteins.
Kiyozumi D, Takeichi M, Nakano I, Sato Y, Fukuda T, Sekiguchi K., Free PMC Article | 08/25/2012 |
| Fras1 is expressed in the branching ureteric bud; renal agenesis occurs in homozygous Fras1 null mutant blebbed (bl) mice on a C57BL6J background. | Fras1, a basement membrane-associated protein mutated in Fraser syndrome, mediates both the initiation of the mammalian kidney and the integrity of renal glomeruli.
Pitera JE, Scambler PJ, Woolf AS., Free PMC Article | 01/21/2010 |
| The localization pattern of Fras1 and Frem2 was indistinguishable, while both proteins along with Frem3 could be detected even in the absence of Frem1. | Differential localization profile of Fras1/Frem proteins in epithelial basement membranes of newborn and adult mice.
Pavlakis E, Makrygiannis AK, Chiotaki R, Chalepakis G. | 01/21/2010 |
| Fras1 is not only essential as a component of a macromolecular complex for the extracellular stabilization of Frem2 but it is also required for its proper intracellular trafficking and export from embryonic epithelial cells. | Basement membrane localization of Frem3 is independent of the Fras1/Frem1/Frem2 protein complex within the sublamina densa.
Petrou P, Pavlakis E, Dalezios Y, Chalepakis G. | 01/21/2010 |
| Taken together, our findings indicate that besides a cooperative function with Fras1 in embryonic basement membranes, Frem1 can also act independently in processes related to epidermal differentiation. | Overlapping and divergent localization of Frem1 and Fras1 and its functional implications during mouse embryonic development.
Petrou P, Chiotaki R, Dalezios Y, Chalepakis G. | 01/21/2010 |
| Study reports the immunofluorescence pattern of Frem3 and a comparative analysis of the spatiotemporal localization of all Fras1/Frem proteins during mouse embryonic development. | Spatiotemporal distribution of Fras1/Frem proteins during mouse embryonic development.
Chiotaki R, Petrou P, Giakoumaki E, Pavlakis E, Sitaru C, Chalepakis G. | 01/21/2010 |
| QBRICK/Frem1, Fras1, and Frem2 interactions at the basement membrane have roles in preventing Fraser syndrome-like defects | Breakdown of the reciprocal stabilization of QBRICK/Frem1, Fras1, and Frem2 at the basement membrane provokes Fraser syndrome-like defects.
Kiyozumi D, Sugimoto N, Sekiguchi K., Free PMC Article | 01/21/2010 |
| Screened DNA from bl/bl mice and identified a premature termination of mouse Fras1. Thus, the bl mouse is a model for Fraser syndrome in humans. | Fraser syndrome and mouse blebbed phenotype caused by mutations in FRAS1/Fras1 encoding a putative extracellular matrix protein.
McGregor L, Makela V, Darling SM, Vrontou S, Chalepakis G, Roberts C, Smart N, Rutland P, Prescott N, Hopkins J, Bentley E, Shaw A, Roberts E, Mueller R, Jadeja S, Philip N, Nelson J, Francannet C, Perez-Aytes A, Megarbane A, Kerr B, Wainwright B, Woolf AS, Winter RM, Scambler PJ. | 01/21/2010 |