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Literature summary for 2.4.1.224 extracted from

  • Zhang, R.; Cao, P.; Yang, Z.; Wang, Z.; Wu, J.L.; Chen, Y.; Pan, Y.
    Heparan sulfate biosynthesis enzyme, Ext1, contributes to outflow tract development of mouse heart via modulation of FGF signaling (2015), PLoS ONE, 10, e0136518.
    View publication on PubMedView publication on EuropePMC
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Organism

Organism UniProt Comment Textmining
Mus musculus P97464 gene EXT1
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Source Tissue

Source Tissue Comment Organism Textmining
embryo
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 Mus musculus
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heart
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 Mus musculus
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mesoderm
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 Mus musculus
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neural crest cell
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 Mus musculus
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pharynx
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 Mus musculus
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Synonyms

Synonyms Comment Organism
exostosin-1
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 Mus musculus
EXT1
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 Mus musculus

General Information

General Information Comment Organism
malfunction deletion of Ext1 in the mesoderm induces a cardiac phenotype similar to that of a mutant with conditional deletion of UDP-glucose dehydrogenase, a key enzyme responsible for synthesis of all glycosaminoglycans. The outflow tract defect in conditional Ext1 knockout (Ext1f/f:Mesp1Cre) mice is attributable to the reduced contribution of second heart field and neural crest cells. Ext1 deletion leads to downregulation of FGF signaling in the pharyngeal mesoderm. Exogenous FGF8 ameliorates the defects in the outflow tract and pharyngeal explants. Phenotype, detailed overview Mus musculus
metabolism Exostosin glycosyltransferases exclusively catalyze heparan sulfate polymerization. Heparan sulfate/heparin, chondroitin sulfate, dermatan sulfate, and keratan sulfate form glycosaminoglycans, long linear polysaccharide chains consisting of repeat disaccharide units. Glycosaminoglycans are the major components of the extracellular matrix and play critical roles in regulating transport and signaling of numerous growth factors during embryonic development Mus musculus
physiological function Ext1 is a glycosyltransferase responsible for heparan sulfate synthesis. Function of Ext1 in heart development, overview. Ext1 expression in second heart field and neural crest cells is required for outflow tract remodeling. Ext1 is crucial for outflow tract formation in distinct progenitor cells, and heparan sulfate modulates FGF signaling during early heart development. Proper expression of Ext1 is required for cardiogenesis, heparan sulfate is required for heart development Mus musculus