Spatiotemporal distribution of Fras1/Frem proteins during mouse embryonic development

Chiotaki, Rena; Petrou, Petros; Giakoumaki, Elsa; Pavlakis, Evangelos; Sitaru, Cassian; Chalepakis, Georges

doi:10.1016/j.modgep.2006.12.001

Cited by 36 publications

(50 citation statements)

References 19 publications

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“…In the majority of developmental contexts Fras1 and Frem2 are expressed in the epidermis whereas Frem1 is expressed in the underlying mesenchyme or dermis (Smyth et al, 2004), although this is often tissue specific and for the most part the proteins all localize to the basement membrane. Intracellular epidermal Frem1 is noted in some contexts (Chiotaki et al, 2007;Petrou et al, 2007a). These observations, along with the domain structure of the proteins, led us to speculate that the proteins interact directly to mediate epidermal adhesion (Smyth et al, 2004).…”

Section: Introductionmentioning

confidence: 96%

“…Elegant biochemical studies have subsequently shown that Frem1, Frem2, and Fras1 form a ternary complex in vitro and that this complex is most probably required for normal epidermal adhesion in utero (Kiyozumi et al, 2006). It is unclear whether Frem3 is involved in this complex, but the gene's divergent expression and the normal localization of the protein in mice carrying mutations in the other three proteins suggest that it acts in an independent manner (Chiotaki et al, 2007;Petrou et al, 2007b). One way in which we can study the nature of the relationships between these genes and the pathology resulting from their loss is by comparing gene expression in mammals with other vertebrates.…”

Section: Introductionmentioning

confidence: 99%

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Expression of the fras1/frem gene family during zebrafish development and fin morphogenesis

Gautier

Naranjo-Golborne

Taylor

et al. 2008

Developmental Dynamics

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Mouse studies have highlighted the requirement of the extracellular matrix Fras and Frem proteins for embryonic epidermal adhesion. Mutations of the genes encoding some of these proteins underlie the blebs mouse mutants, whereas mutations in human FRAS1 and FREM2 cause Fraser syndrome, a congenital disorder characterized by embryonic blistering and renal defects. We have cloned the zebrafish homologues of these genes and characterized their evolutionary diversification and expression during development. The fish gene complement includes fras1, frem1a, frem1b, frem2a, frem2b, and frem3, which display complex overlapping and complementary expression patterns in developing tissues including the pharyngeal arches, hypochord, musculature, and otic vesicle. Expression during fin development delineates distinct populations of epidermal cells which have previously only been described at a morphological level. We detect relatively little gene expression in epidermis or pronephros, suggesting that the essential role of these proteins in mediating their development in humans and mice is recently evolved. Developmental Dynamics 237:3295-3304, 2008.

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Section: Introductionmentioning

confidence: 96%

Section: Introductionmentioning

confidence: 99%

Expression of the fras1/frem gene family during zebrafish development and fin morphogenesis

Gautier

Naranjo-Golborne

Taylor

et al. 2008

Developmental Dynamics

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“…Fras1 encodes a transmembrane protein containing motifs implicated in signaling and adhesion (Gautier et al, 2008;McGregor et al, 2003). The large extracellular portion of Fras1 is cleaved and released into basal lamina underlying epithelia (Carney et al, 2010;Chiotaki et al, 2007;Kiyozumi et al, 2006;. Hence, a layer of Fras1 protein surrounds the neural crest-derived skeletogenic portion of pharyngeal arches.…”

Section: Introductionmentioning

confidence: 99%

fras1shapes endodermal pouch 1 and stabilizes zebrafish pharyngeal skeletal development

et al. 2012

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SUMMARYLesions in the epithelially expressed human gene FRAS1 cause Fraser syndrome, a complex disease with variable symptoms, including facial deformities and conductive hearing loss. The developmental basis of facial defects in Fraser syndrome has not been elucidated. Here we show that zebrafish fras1 mutants exhibit defects in facial epithelia and facial skeleton. Specifically, fras1 mutants fail to generate a late-forming portion of pharyngeal pouch 1 (termed late-p1) and skeletal elements adjacent to late-p1 are disrupted. Transplantation studies indicate that fras1 acts in endoderm to ensure normal morphology of both skeleton and endoderm, consistent with well-established epithelial expression of fras1. Late-p1 formation is concurrent with facial skeletal morphogenesis, and some skeletal defects in fras1 mutants arise during late-p1 morphogenesis, indicating a temporal connection between late-p1 and skeletal morphogenesis. Furthermore, fras1 mutants often show prominent second arch skeletal fusions through space occupied by late-p1 in wild type. Whereas every fras1 mutant shows defects in late-p1 formation, skeletal defects are less penetrant and often vary in severity, even between the left and right sides of the same individual. We interpret the fluctuating asymmetry in fras1 mutant skeleton and the changes in fras1 mutant skeletal defects through time as indicators that skeletal formation is destabilized. We propose a model wherein fras1 prompts late-p1 formation and thereby stabilizes skeletal formation during zebrafish facial development. Similar mechanisms of stochastic developmental instability might also account for the high phenotypic variation observed in human FRAS1 patients.

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“…In the present study, the following rabbit polyclonal antibodies were used: Fras1 , Frem1 (Petrou et al 2007a), Frem2 and Frem3 (Chiotaki et al 2007), collagen VII (kindly provided by Dr C. Sitaru et al 2005).…”

Section: Antibodiesmentioning

confidence: 99%

“…Recent comparative immunohistochemical studies reported the colocalization of the Fras1/Frem proteins in all epithelial basement membranes, throughout embryonic development (Chiotaki et al 2007). Furthermore, utilization of transmission electron microscopy to investigate the exact location of these proteins, revealed their colocalization in the sublamina densa of various embryonic epithelial basement membranes (Kiyozumi et al 2006;Petrou et al 2007b).…”

Section: Introductionmentioning

confidence: 99%

Differential localization profile of Fras1/Frem proteins in epithelial basement membranes of newborn and adult mice

Pavlakis

Makrygiannis

Chiotaki

et al. 2008

Histochem Cell Biol

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The Fras1/Frem gene family encodes for structurally similar proteins of the extracellular matrix, functionally correlated with embryonic dermal-epidermal adhesion as deduced from the appearance of sub-epidermal blisters in mouse mutants compromising the function of Fras1, Frem1 and Frem2 proteins. Mutations in the human counterparts FRAS1 and FREM2 have been detected in patients suffering from Fraser syndrome. So far, Fras1/Frem proteins have been shown to be strictly colocalized in the sublamina densa of mouse epithelial basement membranes during development. Here, we focused on the characterization of the localization pattern of the aforementioned proteins, in various parts of the adult mouse skin as well as a range of organs and tissues. Frem3 was present in a broad range of epithelial basement membranes where Fras1, Frem1 and Frem2 were missing. The localization profile of Frem3 coincided with that of collagen VII in all skin basement membranes but differed in that Frem3 was additionally found in the basement membrane of several internal epithelia, where collagen VII was absent. Fras1 and Frem2 were colocalized with Frem3 in the basement membrane of certain skin parts, underlying the thin-layer, of rapidly proliferating keratinocytes, whereas Frem1 was detected only in the basement membrane of the tail. The localization pattern of Fras1 and Frem2 was indistinguishable, while both proteins along with Frem3 could be detected even in the absence of Frem1.

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Spatiotemporal distribution of Fras1/Frem proteins during mouse embryonic development

Cited by 36 publications

References 19 publications

Expression of the fras1/frem gene family during zebrafish development and fin morphogenesis

Expression of the fras1/frem gene family during zebrafish development and fin morphogenesis

fras1shapes endodermal pouch 1 and stabilizes zebrafish pharyngeal skeletal development

Differential localization profile of Fras1/Frem proteins in epithelial basement membranes of newborn and adult mice

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