Matches in SemOpenAlex for { <https://semopenalex.org/work/W2004313215> ?p ?o ?g. }
- W2004313215 endingPage "915" @default.
- W2004313215 startingPage "905" @default.
- W2004313215 abstract "Achondroplasia (ACH), the most common form of human dwarfism is caused by a mutation in the Fibroblast Growth Factor Receptor 3 (FGFR3) gene, resulting in constitutive activation of the receptor. Typical radiological features include shortening of the tubular bones and macrocephaly, due to disruption of endochondral ossification. Consequently, FGFR3 has been described as a negative regulator of bone growth. Studying a large cohort of ACH patients, a delay in bone age was observed shortly after birth (for boys p = 2.6 × 10−9 and for girls p = 1.2 × 10−8). This delay was no longer apparent during adolescence. In order to gain further insight into bone formation, bone development was studied in a murine model of chondrodysplasia (Fgfr3Y367C/+) from birth to 6 weeks of age. Delayed bone age was also observed in Fgfr3Y367C/+ mice at 1 week of age followed by an accelerated secondary ossification center formation. A low level of chondrocyte proliferation was observed in the normal growth plate at birth, which increased with bone growth. In the pathological condition, a significantly high level of proliferative cells was present at birth, but exhibited a transient decrease only to rise again subsequently. Histological and in situ analyses suggested the altered endochondral ossification process may result from delayed chondrocyte differentiation, disruption of vascularization and osteoblast invasion of the femur. All these data provide evidence that FGFR3 regulates normal chondrocyte proliferation and differentiation during bone growth and suggest that constitutive activation of the receptor disrupts both processes. Therefore, the consequences of FGFR3 activation on the physiological process of bone development appear to be dependent on spatial and temporal occurrence. In conclusion, these observations support the notion that FGFR3 has a dual effect, as both a negative and a positive regulator of the endochondral ossification process during post-natal bone development." @default.
- W2004313215 created "2016-06-24" @default.
- W2004313215 creator A5012453905 @default.
- W2004313215 creator A5012488478 @default.
- W2004313215 creator A5029002217 @default.
- W2004313215 creator A5029418581 @default.
- W2004313215 creator A5064294296 @default.
- W2004313215 creator A5082891546 @default.
- W2004313215 creator A5085291692 @default.
- W2004313215 creator A5089962133 @default.
- W2004313215 date "2010-11-01" @default.
- W2004313215 modified "2023-10-01" @default.
- W2004313215 title "Delayed bone age due to a dual effect of FGFR3 mutation in Achondroplasia" @default.
- W2004313215 cites W1558075619 @default.
- W2004313215 cites W1972979483 @default.
- W2004313215 cites W1974142764 @default.
- W2004313215 cites W1977343110 @default.
- W2004313215 cites W1977595953 @default.
- W2004313215 cites W1978217317 @default.
- W2004313215 cites W1978635256 @default.
- W2004313215 cites W1979326294 @default.
- W2004313215 cites W1981856729 @default.
- W2004313215 cites W1988790505 @default.
- W2004313215 cites W1994172438 @default.
- W2004313215 cites W1996195979 @default.
- W2004313215 cites W1997941225 @default.
- W2004313215 cites W2005259839 @default.
- W2004313215 cites W2005453160 @default.
- W2004313215 cites W2006078771 @default.
- W2004313215 cites W2016411107 @default.
- W2004313215 cites W2030409202 @default.
- W2004313215 cites W2036851890 @default.
- W2004313215 cites W2046159839 @default.
- W2004313215 cites W2047727235 @default.
- W2004313215 cites W2055935604 @default.
- W2004313215 cites W2060029591 @default.
- W2004313215 cites W2064623632 @default.
- W2004313215 cites W2086964102 @default.
- W2004313215 cites W2092503452 @default.
- W2004313215 cites W2101469591 @default.
- W2004313215 cites W2105419881 @default.
- W2004313215 cites W2107044385 @default.
- W2004313215 cites W2108698814 @default.
- W2004313215 cites W2113988225 @default.
- W2004313215 cites W2114529565 @default.
- W2004313215 cites W2121266463 @default.
- W2004313215 cites W2121360268 @default.
- W2004313215 cites W2122164545 @default.
- W2004313215 cites W2127056741 @default.
- W2004313215 cites W2132345567 @default.
- W2004313215 cites W2135369311 @default.
- W2004313215 cites W2136379850 @default.
- W2004313215 cites W2139318098 @default.
- W2004313215 cites W2150666277 @default.
- W2004313215 cites W2153039235 @default.
- W2004313215 cites W2154917734 @default.
- W2004313215 cites W2156221138 @default.
- W2004313215 cites W2160088835 @default.
- W2004313215 cites W2163961175 @default.
- W2004313215 cites W2165216233 @default.
- W2004313215 cites W2167488706 @default.
- W2004313215 cites W2168876853 @default.
- W2004313215 cites W2172197499 @default.
- W2004313215 cites W4230848225 @default.
- W2004313215 cites W4239370680 @default.
- W2004313215 cites W4248318320 @default.
- W2004313215 cites W4319054333 @default.
- W2004313215 doi "https://doi.org/10.1016/j.bone.2010.07.020" @default.
- W2004313215 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/20673820" @default.
- W2004313215 hasPublicationYear "2010" @default.
- W2004313215 type Work @default.
- W2004313215 sameAs 2004313215 @default.
- W2004313215 citedByCount "23" @default.
- W2004313215 countsByYear W20043132152012 @default.
- W2004313215 countsByYear W20043132152014 @default.
- W2004313215 countsByYear W20043132152015 @default.
- W2004313215 countsByYear W20043132152016 @default.
- W2004313215 countsByYear W20043132152017 @default.
- W2004313215 countsByYear W20043132152018 @default.
- W2004313215 countsByYear W20043132152020 @default.
- W2004313215 countsByYear W20043132152021 @default.
- W2004313215 countsByYear W20043132152023 @default.
- W2004313215 crossrefType "journal-article" @default.
- W2004313215 hasAuthorship W2004313215A5012453905 @default.
- W2004313215 hasAuthorship W2004313215A5012488478 @default.
- W2004313215 hasAuthorship W2004313215A5029002217 @default.
- W2004313215 hasAuthorship W2004313215A5029418581 @default.
- W2004313215 hasAuthorship W2004313215A5064294296 @default.
- W2004313215 hasAuthorship W2004313215A5082891546 @default.
- W2004313215 hasAuthorship W2004313215A5085291692 @default.
- W2004313215 hasAuthorship W2004313215A5089962133 @default.
- W2004313215 hasConcept C105702510 @default.
- W2004313215 hasConcept C126322002 @default.
- W2004313215 hasConcept C134018914 @default.
- W2004313215 hasConcept C147708747 @default.
- W2004313215 hasConcept C170493617 @default.
- W2004313215 hasConcept C17938293 @default.
- W2004313215 hasConcept C202751555 @default.