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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Frem1tm1Ksek
targeted mutation 1, Kiyotoshi Sekiguchi
MGI:3665235
Summary 3 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
Frem1tm1Ksek/Frem1tm1Ksek involves: 129 MGI:5427912
hm2
Frem1tm1Ksek/Frem1tm1Ksek involves: 129 * C57BL/6 MGI:3665237
ht3
Frem1tm1Ksek/Frem1+ involves: 129 * C57BL/6 MGI:5296001


Genotype
MGI:5427912
hm1
Allelic
Composition
Frem1tm1Ksek/Frem1tm1Ksek
Genetic
Background
involves: 129
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Frem1tm1Ksek mutation (1 available); any Frem1 mutation (117 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
renal/urinary system
• most mice exhibit renal dysmorphogenesis (10 of 13 mice unilateral renal agenesis and 2 of 13 unlilateral or bilateral renal dysgenesis at E15.5)
• unilaterally in 10 of 13 mice




Genotype
MGI:3665237
hm2
Allelic
Composition
Frem1tm1Ksek/Frem1tm1Ksek
Genetic
Background
involves: 129 * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Frem1tm1Ksek mutation (1 available); any Frem1 mutation (117 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Phenotypes and electron microscopy of Frem1tm1Ksek/Frem1tm1Ksek mice

limbs/digits/tail

vision/eye

renal/urinary system

integument
• exhibit subepidermal blistering occurring between the lamina densa of the basement membrane and the underlying dermis at E14.5

skeleton
• mutants exhibit complete fusion of the posterior frontal suture at postnatal day 28 compared to day 45 in controls

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Fraser syndrome DOID:0090001 OMIM:PS219000
J:111788




Genotype
MGI:5296001
ht3
Allelic
Composition
Frem1tm1Ksek/Frem1+
Genetic
Background
involves: 129 * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Frem1tm1Ksek mutation (1 available); any Frem1 mutation (117 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
craniofacial
• mutants exhibit variable suture abnormalities similar to Frem1bat heterozygotes

skeleton
• mutants exhibit variable suture abnormalities similar to Frem1bat heterozygotes





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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last database update
12/10/2024
MGI 6.24
The Jackson Laboratory