The paradox of FGFR3 signaling in skeletal dysplasia: Why
chondrocytes growth arrest while other cells over proliferate

J 2014

The paradox of FGFR3 signaling in skeletal dysplasia: Why chondrocytes growth arrest while other cells over proliferate

KREJČÍ, Pavel

Základní údaje

Originální název

The paradox of FGFR3 signaling in skeletal dysplasia: Why chondrocytes growth arrest while other cells over proliferate

Autoři

KREJČÍ, Pavel

Vydání

Mutation Research - Reviews in Mutation Research, Amsterdam, Elsevier Science, 2014, 1383-5742

Další údaje

Jazyk

angličtina

Typ výsledku

Článek v odborném periodiku

Obor

10600 1.6 Biological sciences

Stát vydavatele

Nizozemské království

Utajení

není předmětem státního či obchodního tajemství

Impakt faktor

Impact factor: 6.213

Označené pro přenos do RIV

Ano

Kód RIV

RIV/00216224:14110/14:00073450

Organizační jednotka

Lékařská fakulta

Klíčová slova anglicky

FGFR3; RASopathies; ERK; Achondroplasia; Skeletal dyplasia; Cancer

Štítky

EL OK

Příznaky

Mezinárodní význam, Recenzováno

Změněno: 27. 5. 2014 11:46, Ing. Mgr. Věra Pospíšilíková

Anotace

V originále

Somatic mutations in receptor tyrosine kinase FGFR3 cause excessive cell proliferation, leading to cancer or skin overgrowth. Remarkably, the same mutations inhibit chondrocyte proliferation and differentiation in developing bones, resulting in skeletal dysplasias, such as hypochondroplasia, achondroplasia, SADDAN and thanatophoric dysplasia. A similar phenotype is observed in Noonan syndrome, Leopard syndrome, hereditary gingival fibromatosis, neurofibromatosis type 1, Costello syndrome, Legius syndrome and cardiofaciocutaneous syndrome. Collectively termed RASopathies, the latter syndromes are caused by germline mutations in components of the RAS/ERK MAP kinase signaling pathway. This article considers the evidence suggesting that FGFR3 activation in chondrocytes mimics the activation of major oncogenes signaling via the ERK pathway. Subsequent inhibition of chondrocyte proliferation in FGFR3-related skeletal dysplasias and RASopathies is proposed to result from activation of defense mechanisms that originally evolved to safeguard mammalian organisms against cancer.

Návaznosti

GAP305/11/0752, projekt VaV

Název: Molekulární základy FGFR3 signalingu v kostní dysplázii

Investor: Grantová agentura ČR, Molekulární základy FGFR3 signalingu v kostní dysplázii

LH12004, projekt VaV

Název: FGFR3-specifický adaptérom a jeho role v patologické FGFR3 signalizaci v nemoci (Akronym: AMVIS-FGFR3)

Investor: Ministerstvo školství, mládeže a tělovýchovy ČR, FGFR3-specifický adaptérom a jeho role v patologické FGFR3 signalizaci v nemoci

MUNI/M/0071/2013, interní kód MU

Název: High-throughput screening of compound libraries aimed on discovery of novel inhibitors of the FGFR/ERK MAP kinase signaling (Akronym: HTS-FGFR)

Investor: Masarykova univerzita, High-throughput screening of compound libraries aimed on discovery of novel inhibitors of the FGFR/ERK MAP kinase signaling, INTERDISCIPLINARY - Mezioborové výzkumné projekty

Citovat

KREJČÍ, Pavel. The paradox of FGFR3 signaling in skeletal dysplasia: Why chondrocytes growth arrest while other cells over proliferate. Mutation Research - Reviews in Mutation Research. Amsterdam: Elsevier Science, 2014, roč. 759, January–March, s. 40-48. ISSN 1383-5742. Dostupné z: https://doi.org/10.1016/j.mrrev.2013.11.001.

@article{1139209,
   author = {Krejčí, Pavel},
   article_location = {Amsterdam},
   article_number = {January–March},
   doi = {https://doi.org/10.1016/j.mrrev.2013.11.001},
   keywords = {FGFR3; RASopathies; ERK; Achondroplasia; Skeletal dyplasia; Cancer},
   language = {eng},
   issn = {1383-5742},
   journal = {Mutation Research - Reviews in Mutation Research},
   title = {The paradox of FGFR3 signaling in skeletal dysplasia: Why chondrocytes growth arrest while other cells over proliferate},
   volume = {759},
   year = {2014}
}

TY  - JOUR
ID  - 1139209
AU  - Krejčí, Pavel
PY  - 2014
TI  - The paradox of FGFR3 signaling in skeletal dysplasia: Why chondrocytes growth arrest while other cells over proliferate
JF  - Mutation Research - Reviews in Mutation Research
VL  - 759
IS  - January–March
SP  - 40-48
EP  - 40-48
PB  - Elsevier Science
SN  - 13835742
KW  - FGFR3
KW  - RASopathies
KW  - ERK
KW  - Achondroplasia
KW  - Skeletal dyplasia
KW  - Cancer
N2  - Somatic mutations in receptor tyrosine kinase FGFR3 cause excessive cell proliferation, leading to cancer or skin overgrowth. Remarkably, the same mutations inhibit chondrocyte proliferation and differentiation in developing bones, resulting in skeletal dysplasias, such as hypochondroplasia, achondroplasia, SADDAN and thanatophoric dysplasia. A similar phenotype is observed in Noonan syndrome, Leopard syndrome, hereditary gingival fibromatosis, neurofibromatosis type 1, Costello syndrome, Legius syndrome and cardiofaciocutaneous syndrome. Collectively termed RASopathies, the latter syndromes are caused by germline mutations in components of the RAS/ERK MAP kinase signaling pathway. This article considers the evidence suggesting that FGFR3 activation in chondrocytes mimics the activation of major oncogenes signaling via the ERK pathway. Subsequent inhibition of chondrocyte proliferation in FGFR3-related skeletal dysplasias and RASopathies is proposed to result from activation of defense mechanisms that originally evolved to safeguard mammalian organisms against cancer.
ER  -

KREJČÍ, Pavel. The paradox of FGFR3 signaling in skeletal dysplasia: Why chondrocytes growth arrest while other cells over proliferate. \textit{Mutation Research - Reviews in Mutation Research}. Amsterdam: Elsevier Science, 2014, roč.~759, January–March, s.~40-48. ISSN~1383-5742. Dostupné z: https://doi.org/10.1016/j.mrrev.2013.11.001.

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