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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID 		Fgfr3tm4Cxd
targeted mutation 4, Chu-Xia Deng
MGI:2135675
Summary 4 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cn1
 		Fgfr3tm4Cxd/Fgfr3+
Krastm4Tyj/Kras+
Tg(Upk2-cre)6Xrw/0 		involves: 129S4/SvJae * 129S6/SvEvTac * FVB/N MGI:5141739
cn2
 		Ctnnb1tm1Mmt/Ctnnb1+
Fgfr3tm4Cxd/Fgfr3+
Tg(Upk2-cre)6Xrw/0 		involves: 129S6/SvEvTac * 129X1/SvJ * FVB/N MGI:5141741
cn3
 		Fgfr3tm4Cxd/Fgfr3+
Tg(Col2a1-cre)1Bhr/0 		involves: 129S6/SvEvTac * C57BL/6 * NIH Black Swiss * SJL MGI:3640323
cn4
 		Fgfr3tm4Cxd/Fgfr3+
Tg(Upk2-cre)6Xrw/0 		involves: 129S6/SvEvTac * FVB/N MGI:5141738


Genotype
MGI:5141739
cn1
Allelic
Composition		 Fgfr3tm4Cxd/Fgfr3+
Krastm4Tyj/Kras+
Tg(Upk2-cre)6Xrw/0
Genetic
Background		 involves: 129S4/SvJae * 129S6/SvEvTac * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fgfr3tm4Cxd mutation (0 available); any Fgfr3 mutation (52 available)
Krastm4Tyj mutation (9 available); any Kras mutation (76 available)
Tg(Upk2-cre)6Xrw mutation (0 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
integument
increased skin papilloma incidence ( J:174242 )
• by 1 year of age, 44% of mutants develop skin papilloma, with tumors reaching 1 cm in diameter by a median of 220 days

mortality/aging
premature death ( J:174242 )
• shorter survival due to skin papillomas, with a survival time between 100-400 days

neoplasm
neoplasm ( J:174242 )
N
• mice aged to 12 month do not develop urothelial hyperplasia or urothelial carcinoma or lung tumors
increased skin papilloma incidence ( J:174242 )
• by 1 year of age, 44% of mutants develop skin papilloma, with tumors reaching 1 cm in diameter by a median of 220 days




Genotype
MGI:5141741
cn2
Allelic
Composition		 Ctnnb1tm1Mmt/Ctnnb1+
Fgfr3tm4Cxd/Fgfr3+
Tg(Upk2-cre)6Xrw/0
Genetic
Background		 involves: 129S6/SvEvTac * 129X1/SvJ * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ctnnb1tm1Mmt mutation (0 available); any Ctnnb1 mutation (49 available)
Fgfr3tm4Cxd mutation (0 available); any Fgfr3 mutation (52 available)
Tg(Upk2-cre)6Xrw mutation (0 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
mortality/aging
premature death ( J:174242 )
• shorter survival due to lung cancer, with a survival time between 200-400 days

renal/urinary system
abnormal urinary bladder urothelium morphology ( J:174242 )
• 100% of mutants exhibit areas of hyperproliferation in the bladder urothelium from about 3 months of age, however these lesions do not progress further when examined at 12 months of age

neoplasm
neoplasm ( J:174242 )
N
• despite hyperproliferation in the bladder, mutants do not develop urothelial carcinoma by 12 months of age
• mutants do not develop skin papillomas
increased lung tumor incidence ( J:174242 )
• 36% of mutants develop lung tumors by 1 year of age
• lung tumors resemble solitary fibrous tumors of the lugs (hemangiopericytomas)

respiratory system
increased lung tumor incidence ( J:174242 )
• 36% of mutants develop lung tumors by 1 year of age
• lung tumors resemble solitary fibrous tumors of the lugs (hemangiopericytomas)

Mouse Models of Human Disease
 DO ID OMIM ID(s) Ref(s)
lung cancer DOID:1324 OMIM:211980
OMIM:608935
OMIM:612571
OMIM:612593
OMIM:614210
 J:174242




Genotype
MGI:3640323
cn3
Allelic
Composition		 Fgfr3tm4Cxd/Fgfr3+
Tg(Col2a1-cre)1Bhr/0
Genetic
Background		 involves: 129S6/SvEvTac * C57BL/6 * NIH Black Swiss * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fgfr3tm4Cxd mutation (0 available); any Fgfr3 mutation (52 available)
Tg(Col2a1-cre)1Bhr mutation (3 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
mortality/aging
neonatal lethality, incomplete penetrance ( J:63198 )
• majority die on the first day after birth

skeleton
abnormal skeleton morphology ( J:63198 )
• exhibit skeletal phenotypes similar to heterozygous Fgfr3tm4.1Cxd mice

respiratory system
impaired lung alveolus development ( J:63198 )
• P1 lungs show reduced alveoli formation similar to that seen in heterozygous Fgfr3tm4.1Cxd mice

Mouse Models of Human Disease
 DO ID OMIM ID(s) Ref(s)
thanatophoric dysplasia DOID:13481 OMIM:187600
OMIM:187601
OMIM:273680
 J:63198




Genotype
MGI:5141738
cn4
Allelic
Composition		 Fgfr3tm4Cxd/Fgfr3+
Tg(Upk2-cre)6Xrw/0
Genetic
Background		 involves: 129S6/SvEvTac * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fgfr3tm4Cxd mutation (0 available); any Fgfr3 mutation (52 available)
Tg(Upk2-cre)6Xrw mutation (0 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
neoplasm
neoplasm ( J:174242 )
N
• mutants do not develop skin or lung tumors, or urothelial hyperplasia, dysplasia or carcinoma




Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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Send questions and comments to User Support. 		last database update
04/09/2024
MGI 6.23 		The Jackson Laboratory