Deficiency of Sprouty Proteins in Mice Resemble Ciliopathic Phenotype

Kód výsledku v IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216305%3A26620%2F19%3APU132816" target="_blank" >RIV/00216305:26620/19:PU132816 - isvavai.cz</a>

Nalezeny alternativní kódy

RIV/00216224:14110/19:00112474

Výsledek na webu

<a href="https://onlinelibrary.wiley.com/doi/abs/10.1002/jmor.21003" target="_blank" >https://onlinelibrary.wiley.com/doi/abs/10.1002/jmor.21003</a>

DOI - Digital Object Identifier

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Jazyk výsledku

angličtina

Název v původním jazyce

Deficiency of Sprouty Proteins in Mice Resemble Ciliopathic Phenotype

Popis výsledku v původním jazyce

The Sprouty family of proteins is a highly conserved group of intracellular modulators of receptor tyrosine kinase signaling, including the fibroblast growth factor receptor (FGFR) pathway. There are four orthologs (Sprouty1-4) in mammals. During embryonic development and postnatal bone remodeling, tight regulation of FGFR signaling is essential in endochondral ossification and in bone and mineral homeostasis. Sprouty2 was shown to be implicated in endochondral bone formation in Sprouty2-/- mice with reduced postnatal skeletal size and trabecular bone mass. Nevertheless, the underlying mechanism of its action at the molecular level remains to be elucidated. Moreover, long bones of Sprouty4-/- display limb malformations, such as chondroma-like lesions in locations adjacent to growth plate, or fused and/or supernumerary digits on front limbs. Our skeletal and other phenotypic findings on Sprouty2- and 4-deficient mice and Sprouty2/4 double knockouts (DKOs) strikingly resemble the phenotype of model mice

Název v anglickém jazyce

Deficiency of Sprouty Proteins in Mice Resemble Ciliopathic Phenotype

Popis výsledku anglicky

The Sprouty family of proteins is a highly conserved group of intracellular modulators of receptor tyrosine kinase signaling, including the fibroblast growth factor receptor (FGFR) pathway. There are four orthologs (Sprouty1-4) in mammals. During embryonic development and postnatal bone remodeling, tight regulation of FGFR signaling is essential in endochondral ossification and in bone and mineral homeostasis. Sprouty2 was shown to be implicated in endochondral bone formation in Sprouty2-/- mice with reduced postnatal skeletal size and trabecular bone mass. Nevertheless, the underlying mechanism of its action at the molecular level remains to be elucidated. Moreover, long bones of Sprouty4-/- display limb malformations, such as chondroma-like lesions in locations adjacent to growth plate, or fused and/or supernumerary digits on front limbs. Our skeletal and other phenotypic findings on Sprouty2- and 4-deficient mice and Sprouty2/4 double knockouts (DKOs) strikingly resemble the phenotype of model mice

Druh

O - Ostatní výsledky

CEP obor

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OECD FORD obor

10605 - Developmental biology

Projekt

Výsledek vznikl pri realizaci vícero projektů. Více informací v záložce Projekty.

Návaznosti

P - Projekt vyzkumu a vyvoje financovany z verejnych zdroju (s odkazem do CEP)

Rok uplatnění

2019

Kód důvěrnosti údajů

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů