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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
FuzGt(OSTGST001398)Lex
gene trap OSTGST001398, Lexicon Genetics
MGI:3531090
Summary 6 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
FuzGt(OSTGST001398)Lex/FuzGt(OSTGST001398)Lex involves: 129S5/SvEvBrd MGI:7764025
hm2
FuzGt(OSTGST001398)Lex/FuzGt(OSTGST001398)Lex involves: 129S5/SvEvBrd * C57BL/6 MGI:5297069
hm3
FuzGt(OSTGST001398)Lex/FuzGt(OSTGST001398)Lex involves: 129S5/SvEvBrd * C57BL/6N MGI:4438900
cn4
FuzGt(OSTGST001398)Lex/FuzGt(OSTGST001398)Lex
Gt(ROSA)26Sortm4(ACTB-tdTomato,-EGFP)Luo/Gt(ROSA)26Sor+
H2az2Tg(Wnt1-cre)11Rth/H2az2+
involves: 129S1/Sv * 129S5/SvEvBrd * 129X1/SvJ * C57BL/6J * CBA/J MGI:8266923
cn5
FuzGt(OSTGST001398)Lex/FuzGt(OSTGST001398)Lex
H2az2Tg(Wnt1-cre)11Rth/H2az2+
involves: 129S5/SvEvBrd * C57BL/6J * CBA/J MGI:8266921
cx6
Fgf8tm2Mrt/Fgf8+
FuzGt(OSTGST001398)Lex/FuzGt(OSTGST001398)Lex
involves: 129 * 129S5/SvEvBrd MGI:8266924


Genotype
MGI:7764025
hm1
Allelic
Composition
FuzGt(OSTGST001398)Lex/FuzGt(OSTGST001398)Lex
Genetic
Background
involves: 129S5/SvEvBrd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
FuzGt(OSTGST001398)Lex mutation (0 available); any Fuz mutation (23 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
craniofacial
• cell numbers are increased in the maxilla, but no increase in mitotic cells or change in apoptosis is seen
• palatine bone defects are seen throughout the anterior-posterior extent of the secondary palate
• palatine bones are displaced medially
• palatine bones are small and constrained but roughly normal in shape
• mice exhibit enlarged maxillary processes as early as E9.0 and by E9.5, the maxillary domain is larger, indicating maxillary hyperplasia
• palatal primordia are evident at the appropriate stage but are displaced medially compared to controls and do not extend ventrally at E13.5
• at E14.5, the palatal condensations do not extend and appear as one contiguous domain by E16
• however, a recognizable palate can form despite an initial failure of shelf outgrowth and the majority of midfacial bones are present and ossifying
• mice show expanded mesenchyme within the oral cavity
• palatal narrowing and palatine bone defects are seen throughout the anterior-posterior extent of the secondary palate
• mice appear to have a cleft secondary palate but frontal sections show that palatal shelves are not clefted and instead, palatine bones show the classic inverted-V shape typical of high arched palate, commonly referred to as pseudo-cleft

digestive/alimentary system
• palatal primordia are evident at the appropriate stage but are displaced medially compared to controls and do not extend ventrally at E13.5
• at E14.5, the palatal condensations do not extend and appear as one contiguous domain by E16
• however, a recognizable palate can form despite an initial failure of shelf outgrowth and the majority of midfacial bones are present and ossifying
• palatal narrowing and palatine bone defects are seen throughout the anterior-posterior extent of the secondary palate
• mice appear to have a cleft secondary palate but frontal sections show that palatal shelves are not clefted and instead, palatine bones show the classic inverted-V shape typical of high arched palate, commonly referred to as pseudo-cleft

growth/size/body
• cell numbers are increased in the maxilla, but no increase in mitotic cells or change in apoptosis is seen
• palatine bone defects are seen throughout the anterior-posterior extent of the secondary palate
• palatine bones are displaced medially
• palatine bones are small and constrained but roughly normal in shape
• palatal primordia are evident at the appropriate stage but are displaced medially compared to controls and do not extend ventrally at E13.5
• at E14.5, the palatal condensations do not extend and appear as one contiguous domain by E16
• however, a recognizable palate can form despite an initial failure of shelf outgrowth and the majority of midfacial bones are present and ossifying
• mice show expanded mesenchyme within the oral cavity
• palatal narrowing and palatine bone defects are seen throughout the anterior-posterior extent of the secondary palate
• mice appear to have a cleft secondary palate but frontal sections show that palatal shelves are not clefted and instead, palatine bones show the classic inverted-V shape typical of high arched palate, commonly referred to as pseudo-cleft

limbs/digits/tail

nervous system
• brain overgrowth is seen at E12.5

respiratory system
• upper airway anomalies

skeleton
• cell numbers are increased in the maxilla, but no increase in mitotic cells or change in apoptosis is seen
• palatine bone defects are seen throughout the anterior-posterior extent of the secondary palate
• palatine bones are displaced medially
• palatine bones are small and constrained but roughly normal in shape
• mice show complete synostosis of the coronal sutures

vision/eye
• eye defects are seen at E12.5

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
ciliopathy DOID:0060340 J:198631




Genotype
MGI:5297069
hm2
Allelic
Composition
FuzGt(OSTGST001398)Lex/FuzGt(OSTGST001398)Lex
Genetic
Background
involves: 129S5/SvEvBrd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
FuzGt(OSTGST001398)Lex mutation (0 available); any Fuz mutation (23 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Craniofacial defects in FuzGt(OSTGST001398)Lex/FuzGt(OSTGST001398)Lex mice

mortality/aging
• die immediately after birth

craniofacial
• defects in the nasal cavity, facial bones and craniofacial cartilage
• at E14.5, elongates dorsoventrally and expands the dorsal end along the median-lateral axis
• at E16.0 the anterior cartilage is malformed with an ascending branch and reduced ossification around the cartilage
• increased proliferation of cells at E14.5
• hyperplastic and malformed at E18.5
• at E18.5
• fail to form incisor tooth buds
• upper and lower incisor initiation does not occur
• however, molar buds do form
• at E14.5 the mandible is thickened in the dorsal-ventral axis and shortened in the anterior-posterior axis
• at E18.5, the tongue muscles appear to be fused with the mandible and there is an abnormal ventral bulge in the mandible
• at E18.5
• at E18.5
• abnormal growth of palate tissue at E14.5
• descending palate shelves at E16.5 suggesting a delay in elevation
• at E14.5 the tongue has a rudimentary root structure but fails to elongate in the anterior-posterior axis
• at E18.5, the tongue muscles appear to be fused with the mandible

nervous system
• many brain structures are undeveloped at E14.5
• the corpus striatum mediale is displaced at E12.5
• absence of the optic recess at E12.5
• the trigeminal nerve appears to replace Rathke's pouch

vision/eye
• at E18.5

hearing/vestibular/ear
• displaced at E12.5

endocrine/exocrine glands
• the trigeminal nerve appears to replace Rathke's pouch

digestive/alimentary system
• abnormal growth of palate tissue at E14.5
• descending palate shelves at E16.5 suggesting a delay in elevation
• at E14.5 the tongue has a rudimentary root structure but fails to elongate in the anterior-posterior axis
• at E18.5, the tongue muscles appear to be fused with the mandible

skeleton
• at E14.5, elongates dorsoventrally and expands the dorsal end along the median-lateral axis
• at E16.0 the anterior cartilage is malformed with an ascending branch and reduced ossification around the cartilage
• increased proliferation of cells at E14.5
• hyperplastic and malformed at E18.5
• at E18.5
• fail to form incisor tooth buds
• upper and lower incisor initiation does not occur
• however, molar buds do form
• at E14.5 the mandible is thickened in the dorsal-ventral axis and shortened in the anterior-posterior axis
• at E18.5, the tongue muscles appear to be fused with the mandible and there is an abnormal ventral bulge in the mandible
• at E18.5
• at E18.5
• delay in ossification of the sphenoid and basioccipital bones at E16.0
• membranous ossification of the premaxilla, maxilla, mandible, frontal and parietal bones is missing at E16.0

embryo
• at E14.5 the amount of primary cilium is significantly decreased in the mandibular mesenchyme

growth/size/body
• at E18.5
• fail to form incisor tooth buds
• upper and lower incisor initiation does not occur
• however, molar buds do form
• at E14.5 the mandible is thickened in the dorsal-ventral axis and shortened in the anterior-posterior axis
• at E18.5, the tongue muscles appear to be fused with the mandible and there is an abnormal ventral bulge in the mandible
• at E18.5
• at E18.5
• abnormal growth of palate tissue at E14.5
• descending palate shelves at E16.5 suggesting a delay in elevation
• at E14.5 the tongue has a rudimentary root structure but fails to elongate in the anterior-posterior axis
• at E18.5, the tongue muscles appear to be fused with the mandible
• at E14.5 the amount of primary cilium is significantly decreased in the mandibular mesenchyme




Genotype
MGI:4438900
hm3
Allelic
Composition
FuzGt(OSTGST001398)Lex/FuzGt(OSTGST001398)Lex
Genetic
Background
involves: 129S5/SvEvBrd * C57BL/6N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
FuzGt(OSTGST001398)Lex mutation (0 available); any Fuz mutation (23 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• mice exhibit conotruncal defects unlike wild-type mice
• some mice exhibit ventral septal defects unlike wild-type mice
• some mice exhibit single outflow tracks unlike wild-type mice

skeleton
• primary ciliogenesis in Meckel's cartilage is abnormal compared to in wild-type mice with shorter cilia

nervous system
• even mice with mild overt neural tube closure defects had severely dilated brain ventricles
• in some mice at E18.5
• in some mice at E18.5

limbs/digits/tail
• on all limbs

vision/eye

craniofacial
• primary ciliogenesis in Meckel's cartilage is abnormal compared to in wild-type mice with shorter cilia

respiratory system




Genotype
MGI:8266923
cn4
Allelic
Composition
FuzGt(OSTGST001398)Lex/FuzGt(OSTGST001398)Lex
Gt(ROSA)26Sortm4(ACTB-tdTomato,-EGFP)Luo/Gt(ROSA)26Sor+
H2az2Tg(Wnt1-cre)11Rth/H2az2+
Genetic
Background
involves: 129S1/Sv * 129S5/SvEvBrd * 129X1/SvJ * C57BL/6J * CBA/J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
FuzGt(OSTGST001398)Lex mutation (0 available); any Fuz mutation (23 available)
Gt(ROSA)26Sortm4(ACTB-tdTomato,-EGFP)Luo mutation (10 available); any Gt(ROSA)26Sor mutation (1058 available)
H2az2Tg(Wnt1-cre)11Rth mutation (2 available); any H2az2 mutation (25 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
craniofacial
• enlarged maxillae are comprised of neural crest-derived mesenchyme

embryo
• the depth of the migratory mesencephalic neural crest streams is far greater than in wild-type embryos
• cells rostral to the trigeminal ganglion are seen in a region that should normally be clear of neural crest cells
• E9.25 cranial tissues show an increase in the proportion of neural crest cells

nervous system
• E9.25 cranial tissues show an increase in the proportion of neural crest cells

cellular
• the depth of the migratory mesencephalic neural crest streams is far greater than in wild-type embryos
• cells rostral to the trigeminal ganglion are seen in a region that should normally be clear of neural crest cells




Genotype
MGI:8266921
cn5
Allelic
Composition
FuzGt(OSTGST001398)Lex/FuzGt(OSTGST001398)Lex
H2az2Tg(Wnt1-cre)11Rth/H2az2+
Genetic
Background
involves: 129S5/SvEvBrd * C57BL/6J * CBA/J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
FuzGt(OSTGST001398)Lex mutation (0 available); any Fuz mutation (23 available)
H2az2Tg(Wnt1-cre)11Rth mutation (2 available); any H2az2 mutation (25 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cellular
• E9.25 embryos show an early expansion of cranial neural crest cells from the posterior mesenchephalon to the first and second branchial arches (BA1 and BA2) such that the BA1 and BA2 neural crest streams are wider
• BA2 neural crest stream is increased in size and fails to migrate as far as BA2 control
• a general expansion of midhindbrain streams

embryo
• E9.25 embryos show an early expansion of cranial neural crest cells from the posterior mesenchephalon to the first and second branchial arches (BA1 and BA2) such that the BA1 and BA2 neural crest streams are wider
• BA2 neural crest stream is increased in size and fails to migrate as far as BA2 control
• a general expansion of midhindbrain streams
• frequently, a large proportion of rostral crest cells is seen collecting ectopically in the optic cup regions of E9.25 embryos

nervous system
• frequently, a large proportion of rostral crest cells is seen collecting ectopically in the optic cup regions of E9.25 embryos




Genotype
MGI:8266924
cx6
Allelic
Composition
Fgf8tm2Mrt/Fgf8+
FuzGt(OSTGST001398)Lex/FuzGt(OSTGST001398)Lex
Genetic
Background
involves: 129 * 129S5/SvEvBrd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fgf8tm2Mrt mutation (1 available); any Fgf8 mutation (25 available)
FuzGt(OSTGST001398)Lex mutation (0 available); any Fuz mutation (23 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
craniofacial
N
• E16.5 fetuses show substantial rescue of maxillary hyperplasia that is seen in single Fuz homozygotes and show normal width of the palate

limbs/digits/tail

nervous system
N
• E12.5 fetuses show rescue of the brain overgrowth that is seen in single Fuz homozygotes

vision/eye
N
• E12.5 fetuses show rescue of the eye defects seen in single Fuz homozygotes





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last database update
01/06/2026
MGI 6.24
The Jackson Laboratory