| http://purl.uniprot.org/citations/33784257 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/33784257 | http://www.w3.org/2000/01/rdf-schema#comment | "Activating mutations in the fibroblast growth factor receptor 3 (FGFR3) or inactivating mutations in guanylyl cyclase-B (GC-B), also known as NPR-B or Npr2, cause short-limbed dwarfism. FGFR3 activation causes dephosphorylation and inactivation of GC-B, but the contribution of GC-B dephosphorylation to achondroplasia (ACH) is unknown. GC-B7E/7E mice that express a glutamate-substituted version of GC-B that cannot be inactivated by dephosphorylation were bred with mice expressing FGFR3-G380R, the most common human ACH mutation, to determine if GC-B dephosphorylation is required for ACH. Crossing GC-B7E/7E mice with FGFR3G380R/G380R mice increased naso-anal and long (tibia and femur), but not cranial, bone length twice as much as crossing GC-B7E/7E mice with FGFR3WT/WT mice from 4 to 16 weeks of age. Consistent with increased GC-B activity rescuing ACH, long bones from the GC-B7E/7E/FGFR3G380R/G380R mice were not shorter than those from GC-BWT/WT/FGFR3WT/WT mice. At 2 weeks of age, male but not female FGFR3G380R/G380R mice had shorter long bones and smaller growth plate hypertrophic zones, whereas female but not male GC-B7E/7E mice had longer bones and larger hypertrophic zones. In 2-week-old males, crossing FGFR3G380R/G380R mice with GC-B7E/7E mice increased long bone length and hypertrophic zone area to levels observed in mice expressing WT versions of both receptors. We conclude that preventing GC-B dephosphorylation rescues reduced axial and appendicular skeleton growth in a mouse model of achondroplasia."xsd:string |
| http://purl.uniprot.org/citations/33784257 | http://purl.org/dc/terms/identifier | "doi:10.1172/jci.insight.147832"xsd:string |
| http://purl.uniprot.org/citations/33784257 | http://purl.uniprot.org/core/author | "Lee Y.C."xsd:string |
| http://purl.uniprot.org/citations/33784257 | http://purl.uniprot.org/core/author | "Potter L.R."xsd:string |
| http://purl.uniprot.org/citations/33784257 | http://purl.uniprot.org/core/author | "Robinson J.W."xsd:string |
| http://purl.uniprot.org/citations/33784257 | http://purl.uniprot.org/core/author | "Legeai-Mallet L."xsd:string |
| http://purl.uniprot.org/citations/33784257 | http://purl.uniprot.org/core/author | "Lin Y.W."xsd:string |
| http://purl.uniprot.org/citations/33784257 | http://purl.uniprot.org/core/author | "Kaci N."xsd:string |
| http://purl.uniprot.org/citations/33784257 | http://purl.uniprot.org/core/author | "Wagner B.M."xsd:string |
| http://purl.uniprot.org/citations/33784257 | http://purl.uniprot.org/core/date | "2021"xsd:gYear |
| http://purl.uniprot.org/citations/33784257 | http://purl.uniprot.org/core/name | "JCI Insight"xsd:string |
| http://purl.uniprot.org/citations/33784257 | http://purl.uniprot.org/core/pages | "147832"xsd:string |
| http://purl.uniprot.org/citations/33784257 | http://purl.uniprot.org/core/title | "Prevention of guanylyl cyclase-B dephosphorylation rescues achondroplastic dwarfism."xsd:string |
| http://purl.uniprot.org/citations/33784257 | http://purl.uniprot.org/core/volume | "6"xsd:string |
| http://purl.uniprot.org/citations/33784257 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/33784257 |
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