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Distinct missense mutations of the FGFR3 lys650 codon modulate receptor kinase activation and the severity of the skeletal dysplasia phenotype.

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Am J Hum Genet2000PMID 11055896PMC1287918stubpubmedProvenance
Source
PubMed
Retrieved
Sep 8, 2026
Layer
normalized (units and labels harmonized; values unchanged)
Run
ING-CIVIC-20260908-000001
Published

Abstract

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Linked entities

Linked entities (6)

How each link was made (MeSH, dictionary, registry reference, curation…) and whether it has been validated. Candidate links are not counted in entity statistics.

Validated 6

Curated evidence

Evidence citing this paper (5)

civicProvenance
Source
CIViC — Clinical Interpretation of Variants in Cancer
Dataset
CIViC evidence items
Version
civic-2026-09-08
Retrieved
Sep 8, 2026
Layer
normalized (units and labels harmonized; values unchanged)
Evidence
expert curation
License
CC0 1.0
PMID
11055896
Run
ING-CIVIC-20260908-000001
Open at source
CuratedShowing 1–5 of 5 evidence items · levels, directions and significance as curated at the source; each row links to its CIViC record.
TherapyCancerTypeLevelDirection · significanceRating (1–5)StatusEvidenceSource
FGFR3 K650E1
(functional)—UNRESOLVEDFunctionalDSupports Gain Of Function1accepted
EID10381

The FGFR3 variant K650E is known to be responsible for thanatophoric dysplasia type 2 (TDII), a severe form of skeletal dysplasia. The mutant receptor was tested for comparison between other variants … (full text at CIViC)

PMID 11055896 · Bellus et al., 2000 · Open in CIViC

civic
FGFR3 K650M1
(functional)—UNRESOLVEDFunctionalDSupports Gain Of Function2accepted
EID10380

The K650M variant in FGFR3 is known to be responsible for the severe skeletal dysplasia forms seen in thanatophoric dysplasia type 1 / severe achondroplasia with developmental delay and acanthosis nig… (full text at CIViC)

PMID 11055896 · Bellus et al., 2000 · Open in CIViC

civic
FGFR3 K650N1
(functional)—UNRESOLVEDFunctionalDSupports Gain Of Function2accepted
EID10377

Five patients from four families suffering from skeletal dysplasia were found to harbor a novel heterozygous K650N variant in FGFR3. The variant cosegregated in two patients from one family (a father … (full text at CIViC)

PMID 11055896 · Bellus et al., 2000 · Open in CIViC

civic
FGFR3 K650Q1
(functional)—UNRESOLVEDFunctionalDSupports Gain Of Function2accepted
EID10378

A patient suffering from skeletal dysplasia was found to harbor a novel heterozygous K650Q variant in FGFR3. NIH3T3 cells were transfected with the variant construct followed by kinase activity assay … (full text at CIViC)

PMID 11055896 · Bellus et al., 2000 · Open in CIViC

civic
FGFR3 K650T1
(functional)—UNRESOLVEDFunctionalDSupports Gain Of Function2accepted
EID10382

FGFR3 cDNA construct with the K650T variant was synthesized and transfected into NIH3T3 cells. The K650T variant receptor had a 3.1-fold greater kinase activity compared to the wild type receptor in a… (full text at CIViC)

PMID 11055896 · Bellus et al., 2000 · Open in CIViC

civic