Lrp4 regulates initiation of ureteric budding and is crucial for kidney formation--a mouse model for Cenani-Lenz syndrome.
Development of the kidney is initiated when the ureteric bud (UB) branches from the Wolffian duct and invades the overlying metanephric mesenchyme (MM) triggering the mesenchymal/epithelial interactions that are the basis of organ formation. Multiple signaling pathways must be integrated to ensure p...
| Main Authors: | , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Public Library of Science (PLoS)
2010-04-01
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| Series: | PLoS ONE |
| Online Access: | http://europepmc.org/articles/PMC2861670?pdf=render |
| _version_ | 1818178283832868864 |
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| author | Courtney M Karner Martin F Dietrich Eric B Johnson Natalie Kappesser Christian Tennert Ferda Percin Bernd Wollnik Thomas J Carroll Joachim Herz |
| author_facet | Courtney M Karner Martin F Dietrich Eric B Johnson Natalie Kappesser Christian Tennert Ferda Percin Bernd Wollnik Thomas J Carroll Joachim Herz |
| author_sort | Courtney M Karner |
| collection | DOAJ |
| description | Development of the kidney is initiated when the ureteric bud (UB) branches from the Wolffian duct and invades the overlying metanephric mesenchyme (MM) triggering the mesenchymal/epithelial interactions that are the basis of organ formation. Multiple signaling pathways must be integrated to ensure proper timing and location of the ureteric bud formation.We have used gene targeting to create an Lrp4 null mouse line. The mutation results in early embryonic lethality with a subpenetrant phenotype of kidney agenesis. Ureteric budding is delayed with a failure to stimulate the metanephric mesenchyme in a timely manner, resulting in failure of cellular differentiation and resulting absence of kidney formation in the mouse as well as comparable malformations in humans with Cenani-Lenz syndrome.Lrp4 is a multi-functional receptor implicated in the regulation of several molecular pathways, including Wnt and Bmp signaling. Lrp4(-/-) mice show a delay in ureteric bud formation that results in unilateral or bilateral kidney agenesis. These data indicate that Lrp4 is a critical regulator of UB branching and lack of Lrp4 results in congenital kidney malformations in humans and mice. |
| first_indexed | 2024-12-11T20:45:31Z |
| format | Article |
| id | doaj.art-6ab02f7eb2bc43e296e04a2b70fc7946 |
| institution | Directory Open Access Journal |
| issn | 1932-6203 |
| language | English |
| last_indexed | 2024-12-11T20:45:31Z |
| publishDate | 2010-04-01 |
| publisher | Public Library of Science (PLoS) |
| record_format | Article |
| series | PLoS ONE |
| spelling | doaj.art-6ab02f7eb2bc43e296e04a2b70fc79462022-12-22T00:51:23ZengPublic Library of Science (PLoS)PLoS ONE1932-62032010-04-0154e1041810.1371/journal.pone.0010418Lrp4 regulates initiation of ureteric budding and is crucial for kidney formation--a mouse model for Cenani-Lenz syndrome.Courtney M KarnerMartin F DietrichEric B JohnsonNatalie KappesserChristian TennertFerda PercinBernd WollnikThomas J CarrollJoachim HerzDevelopment of the kidney is initiated when the ureteric bud (UB) branches from the Wolffian duct and invades the overlying metanephric mesenchyme (MM) triggering the mesenchymal/epithelial interactions that are the basis of organ formation. Multiple signaling pathways must be integrated to ensure proper timing and location of the ureteric bud formation.We have used gene targeting to create an Lrp4 null mouse line. The mutation results in early embryonic lethality with a subpenetrant phenotype of kidney agenesis. Ureteric budding is delayed with a failure to stimulate the metanephric mesenchyme in a timely manner, resulting in failure of cellular differentiation and resulting absence of kidney formation in the mouse as well as comparable malformations in humans with Cenani-Lenz syndrome.Lrp4 is a multi-functional receptor implicated in the regulation of several molecular pathways, including Wnt and Bmp signaling. Lrp4(-/-) mice show a delay in ureteric bud formation that results in unilateral or bilateral kidney agenesis. These data indicate that Lrp4 is a critical regulator of UB branching and lack of Lrp4 results in congenital kidney malformations in humans and mice.http://europepmc.org/articles/PMC2861670?pdf=render |
| spellingShingle | Courtney M Karner Martin F Dietrich Eric B Johnson Natalie Kappesser Christian Tennert Ferda Percin Bernd Wollnik Thomas J Carroll Joachim Herz Lrp4 regulates initiation of ureteric budding and is crucial for kidney formation--a mouse model for Cenani-Lenz syndrome. PLoS ONE |
| title | Lrp4 regulates initiation of ureteric budding and is crucial for kidney formation--a mouse model for Cenani-Lenz syndrome. |
| title_full | Lrp4 regulates initiation of ureteric budding and is crucial for kidney formation--a mouse model for Cenani-Lenz syndrome. |
| title_fullStr | Lrp4 regulates initiation of ureteric budding and is crucial for kidney formation--a mouse model for Cenani-Lenz syndrome. |
| title_full_unstemmed | Lrp4 regulates initiation of ureteric budding and is crucial for kidney formation--a mouse model for Cenani-Lenz syndrome. |
| title_short | Lrp4 regulates initiation of ureteric budding and is crucial for kidney formation--a mouse model for Cenani-Lenz syndrome. |
| title_sort | lrp4 regulates initiation of ureteric budding and is crucial for kidney formation a mouse model for cenani lenz syndrome |
| url | http://europepmc.org/articles/PMC2861670?pdf=render |
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