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Phenotypes Associated with This Genotype
Genotype
MGI:7764025
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


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
01/06/2026
MGI 6.24
The Jackson Laboratory