Phenotypes Associated with This Genotype

Genotype
MGI:3716198

cn28

• Allelic Composition: Smo^tm1Amc/Smo^tm2Amc; H2az2^{Tg(Wnt1-cre)11Rth}/H2az2⁺
• Genetic Background: involves: C57BL/6J * CBA/J

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

craniofacial

abnormal cranium morphology ( J:89445 )

abnormal sphenoid bone morphology ( J:89445 )

abnormal basisphenoid bone morphology ( J:89445 )

• rostral half is missing

small alisphenoid bone ( J:89445 )

• reduced

absent orbitosphenoid bone ( J:89445 )

• missing

absent presphenoid bone ( J:89445 )

absent pterygoid process ( J:89445 )

abnormal temporal bone morphology ( J:89445 )

absent styloid process ( J:89445 )

small temporal bone squamous part ( J:89445 )

• reduced

abnormal ethmoid bone morphology ( J:89445 )

• mesethmoid bone is missing

abnormal tooth morphology ( J:89445 )

• teeth are malformed and arrested

absent lower incisors ( J:89445 )

abnormal mandible morphology ( J:89445 )

• the dentate is reduced in length but the lamina is thicker

• at E9.5, there is an increase in apoptotic cells along the midline

• at E10.5, apoptotic cells are observed along the midline and laterally

• at E10.5, mandibles are 9% shorter than the wild-type and only undergoes a 1.5-fold along the dorsal-ventral axis compared to a 4-fold expansion in wild-type

• at E11.5, cell proliferation is decreased

abnormal mandibular condyloid process morphology ( J:89445 )

• mice have an extra condylar process

abnormal maxilla morphology ( J:89445 )

• premaxilla and maxilla retain their lateral-most parts only

absent lacrimal bone ( J:89445 )

• absent or appears as a tiny fragment

nasal bone hypoplasia ( J:89445 )

• nasal bone is hypoplastic

absent palatine bone ( J:89445 )

absent vomer bone ( J:89445 )

small zygomatic bone ( J:89445 )

• reduced

absent middle ear ossicles ( J:89445 )

absent incus ( J:89445 )

absent gonial bone ( J:89445 )

absent malleus ( J:89445 )

absent stapes ( J:89445 )

abnormal craniofacial development ( J:89445 )

Meckel's cartilage hypoplasia ( J:89445 )

• hypoplastic and short

short Meckel's cartilage ( J:89445 )

absent Reichert cartilage ( J:89445 )

• the basi- cerato- and thyro hyoid elements are missing

absent tongue ( J:89445 )

nasal septum hypoplasia ( J:89445 )

• the nasal septum is incomplete

hearing/vestibular/ear

absent middle ear ossicles ( J:89445 )

absent incus ( J:89445 )

absent gonial bone ( J:89445 )

absent malleus ( J:89445 )

absent stapes ( J:89445 )

absent tympanic ring ( J:89445 )

digestive/alimentary system

absent tongue ( J:89445 )

respiratory system

nasal bone hypoplasia ( J:89445 )

• nasal bone is hypoplastic

nasal septum hypoplasia ( J:89445 )

• the nasal septum is incomplete

abnormal thyroid cartilage morphology ( J:89445 )

skeleton

abnormal cranium morphology ( J:89445 )

Meckel's cartilage hypoplasia ( J:89445 )

• hypoplastic and short

short Meckel's cartilage ( J:89445 )

abnormal sphenoid bone morphology ( J:89445 )

abnormal basisphenoid bone morphology ( J:89445 )

• rostral half is missing

small alisphenoid bone ( J:89445 )

• reduced

absent orbitosphenoid bone ( J:89445 )

• missing

absent presphenoid bone ( J:89445 )

absent pterygoid process ( J:89445 )

abnormal temporal bone morphology ( J:89445 )

absent styloid process ( J:89445 )

small temporal bone squamous part ( J:89445 )

• reduced

abnormal ethmoid bone morphology ( J:89445 )

• mesethmoid bone is missing

abnormal tooth morphology ( J:89445 )

• teeth are malformed and arrested

absent lower incisors ( J:89445 )

abnormal mandible morphology ( J:89445 )

• the dentate is reduced in length but the lamina is thicker

• at E9.5, there is an increase in apoptotic cells along the midline

• at E10.5, apoptotic cells are observed along the midline and laterally

• at E10.5, mandibles are 9% shorter than the wild-type and only undergoes a 1.5-fold along the dorsal-ventral axis compared to a 4-fold expansion in wild-type

• at E11.5, cell proliferation is decreased

abnormal mandibular condyloid process morphology ( J:89445 )

• mice have an extra condylar process

abnormal maxilla morphology ( J:89445 )

• premaxilla and maxilla retain their lateral-most parts only

absent lacrimal bone ( J:89445 )

• absent or appears as a tiny fragment

nasal bone hypoplasia ( J:89445 )

• nasal bone is hypoplastic

absent palatine bone ( J:89445 )

absent vomer bone ( J:89445 )

small zygomatic bone ( J:89445 )

• reduced

absent middle ear ossicles ( J:89445 )

absent incus ( J:89445 )

absent gonial bone ( J:89445 )

absent malleus ( J:89445 )

absent stapes ( J:89445 )

absent Reichert cartilage ( J:89445 )

• the basi- cerato- and thyro hyoid elements are missing

abnormal thyroid cartilage morphology ( J:89445 )

vision/eye

absent orbitosphenoid bone ( J:89445 )

• missing

growth/size/body

abnormal tooth morphology ( J:89445 )

• teeth are malformed and arrested

absent lower incisors ( J:89445 )

nasal bone hypoplasia ( J:89445 )

• nasal bone is hypoplastic

absent tongue ( J:89445 )

nasal septum hypoplasia ( J:89445 )

• the nasal septum is incomplete