Phenotypes Associated with This Genotype

Genotype
MGI:2654857

• Allelic Composition: Zeb1^Tw/Zeb1^Tw
• Genetic Background: involves: C57BL/6J

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

mortality/aging

neonatal lethality, complete penetrance ( J:60496 )

• homozygotes are born alive but die within 24 hrs of birth

craniofacial

abnormal premaxilla morphology ( J:60496 )

• homozygotes show absence of fusion of the premaxillary and lateral walls of the lips

• in mice with bilateral cleft lips, the premaxillary or prolabium region is protruded with absent hair follicles

abnormal turbinate morphology ( J:60496 )

• all homozygotes display malformed nasal turbinates

turbinate hypoplasia ( J:60496 )

• the nasal turbinates are reduced in size and number

abnormal palatal shelf morphology ( J:60496 )

• the epithelial layer of the palatal shelves is lined with ciliated columnar epithelium on the nasal side and keratinized epithelium on the oral side

palatal shelves fail to meet at midline ( J:60496 )

• in homozygotes, the shelves of the secondary palate are significantly shorter and smaller than normal, thus exposing the nasal septum

decreased palatal shelf size ( J:60496 )

cleft upper lip ( J:60496 )

• homozygotes exhibit either incomplete cleft lips (where the facial processes destined to form the upper lip, medial and lateral nasal prominences appear nearly fused), or complete cleft lips (with visible separation of the facial processes)

• cleft lips are either unilateral or bilateral; complete unilateral clefts are frequently associated with partial cleft lips on the other side

• 11 of 16 homozygotets displayed bilateral cleft lips; 6 of these bilateral cleft lips were complete on one side and partial on the other, 4 were complete, and 1 was partial

• the remaining 5 of 11 homozygotes showed unilateral cleft lips, 3 complete and 2 partial

bilateral cleft upper lip ( J:60496 )

• 11 of 16 homozygotets displayed bilateral cleft lips; 6 of these bilateral cleft lips were complete on one side and partial on the other, 4 were complete, and 1 was partial

unilateral cleft upper lip ( J:60496 )

• complete unilateral clefts are frequently associated with partial cleft lips on the other side; 5 of 11 homozygotes showed unilateral cleft lips, 3 complete and 2 partial

cleft palate ( J:60496 )

• in some homozygotes with incomplete cleft lips the premaxilla is normal but the hard palate is completely clefted and the nasal septum is exposed

• in others, the unilateral or bilateral clefts of the lip are complete and extend into the premaxillary region and hard palate

cleft secondary palate ( J:60496 )

• all homozygotes display a secondary cleft palate, regardless of whether the primary palate is intact or not

abnormal nasal cavity morphology ( J:60496 )

• homozygotes exhibit a disrupted nasal cavity

• however, the nasal septum remains unaffected and is straight with a midline cartilage

midline facial cleft ( J:60496 )

• all homozygotes display clefts of the midfacial region, including clefts of the palate combined with either unilateral or bilateral clefts of the lip

growth/size/body

abnormal premaxilla morphology ( J:60496 )

• homozygotes show absence of fusion of the premaxillary and lateral walls of the lips

• in mice with bilateral cleft lips, the premaxillary or prolabium region is protruded with absent hair follicles

abnormal turbinate morphology ( J:60496 )

• all homozygotes display malformed nasal turbinates

turbinate hypoplasia ( J:60496 )

• the nasal turbinates are reduced in size and number

abnormal palatal shelf morphology ( J:60496 )

• the epithelial layer of the palatal shelves is lined with ciliated columnar epithelium on the nasal side and keratinized epithelium on the oral side

palatal shelves fail to meet at midline ( J:60496 )

• in homozygotes, the shelves of the secondary palate are significantly shorter and smaller than normal, thus exposing the nasal septum

decreased palatal shelf size ( J:60496 )

cleft upper lip ( J:60496 )

• homozygotes exhibit either incomplete cleft lips (where the facial processes destined to form the upper lip, medial and lateral nasal prominences appear nearly fused), or complete cleft lips (with visible separation of the facial processes)

• cleft lips are either unilateral or bilateral; complete unilateral clefts are frequently associated with partial cleft lips on the other side

• 11 of 16 homozygotets displayed bilateral cleft lips; 6 of these bilateral cleft lips were complete on one side and partial on the other, 4 were complete, and 1 was partial

• the remaining 5 of 11 homozygotes showed unilateral cleft lips, 3 complete and 2 partial

bilateral cleft upper lip ( J:60496 )

• 11 of 16 homozygotets displayed bilateral cleft lips; 6 of these bilateral cleft lips were complete on one side and partial on the other, 4 were complete, and 1 was partial

unilateral cleft upper lip ( J:60496 )

• complete unilateral clefts are frequently associated with partial cleft lips on the other side; 5 of 11 homozygotes showed unilateral cleft lips, 3 complete and 2 partial

cleft palate ( J:60496 )

• in some homozygotes with incomplete cleft lips the premaxilla is normal but the hard palate is completely clefted and the nasal septum is exposed

• in others, the unilateral or bilateral clefts of the lip are complete and extend into the premaxillary region and hard palate

cleft secondary palate ( J:60496 )

• all homozygotes display a secondary cleft palate, regardless of whether the primary palate is intact or not

abnormal nasal cavity morphology ( J:60496 )

• homozygotes exhibit a disrupted nasal cavity

• however, the nasal septum remains unaffected and is straight with a midline cartilage

midline facial cleft ( J:60496 )

• all homozygotes display clefts of the midfacial region, including clefts of the palate combined with either unilateral or bilateral clefts of the lip

distended abdomen ( J:60496 )

• many newborn homozygotes display a bloated abdominal region

respiratory system

abnormal turbinate morphology ( J:60496 )

• all homozygotes display malformed nasal turbinates

turbinate hypoplasia ( J:60496 )

• the nasal turbinates are reduced in size and number

abnormal nasal cavity morphology ( J:60496 )

• homozygotes exhibit a disrupted nasal cavity

• however, the nasal septum remains unaffected and is straight with a midline cartilage

digestive/alimentary system

abnormal palatal shelf morphology ( J:60496 )

• the epithelial layer of the palatal shelves is lined with ciliated columnar epithelium on the nasal side and keratinized epithelium on the oral side

palatal shelves fail to meet at midline ( J:60496 )

• in homozygotes, the shelves of the secondary palate are significantly shorter and smaller than normal, thus exposing the nasal septum

decreased palatal shelf size ( J:60496 )

cleft palate ( J:60496 )

• in some homozygotes with incomplete cleft lips the premaxilla is normal but the hard palate is completely clefted and the nasal septum is exposed

• in others, the unilateral or bilateral clefts of the lip are complete and extend into the premaxillary region and hard palate

cleft secondary palate ( J:60496 )

• all homozygotes display a secondary cleft palate, regardless of whether the primary palate is intact or not

skeleton

abnormal premaxilla morphology ( J:60496 )

• homozygotes show absence of fusion of the premaxillary and lateral walls of the lips

• in mice with bilateral cleft lips, the premaxillary or prolabium region is protruded with absent hair follicles

abnormal turbinate morphology ( J:60496 )

• all homozygotes display malformed nasal turbinates

turbinate hypoplasia ( J:60496 )

• the nasal turbinates are reduced in size and number

hearing/vestibular/ear

abnormal inner ear morphology ( J:60496 )

• homozygotes exhibit inner ear defects (not shown)