All Phenotypes Dlx5<tm1Levi> MGI Mouse

Phenotypes associated with this allele

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Dlx5^tm1Levi/Dlx5^tm1Levi	involves: 129P2/OlaHsd	MGI:5505547
hm2	Dlx5^tm1Levi/Dlx5^tm1Levi	involves: 129P2/OlaHsd * C57BL/6 * DBA/2	MGI:3701347
ht3	Dlx5^tm1Levi/Dlx5⁺	involves: 129P2/OlaHsd * C57BL/6 * DBA/2	MGI:3701346
cx4	Trp63^tm1Aam/Trp63^tm1Aam Dlx5^tm1Levi/Dlx5/Dlx6^tm1Levi	involves: 129P2/OlaHsd	MGI:3797872
cx5	Dlx5^tm1Levi/Dlx5/Dlx6^tm1Levi Trp63^tm1Aam/Trp63^tm1Aam	involves: 129P2/OlaHsd	MGI:3797869
cx6	Dlx5^tm1Levi/Dlx5^tm1Levi Msx1^tm1Bero/Msx1^tm1Bero	involves: 129P2/OlaHsd * C57BL/6 * DBA/2	MGI:3702326

Allelic Composition	Dlx5^tm1Levi/Dlx5^tm1Levi
Genetic Background	involves: 129P2/OlaHsd

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dlx5^tm1Levi mutation (1 available); any Dlx5 mutation (23 available)

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

hearing/vestibular/ear

otic vesicle hypoplasia ( J:78406 )

• at E12.5, the ototcyst displays severe hypoplasia of the primordia of semicircular canals and a failure to elongate the endolymphatic duct, with a varying degree of severity

abnormal cochlea morphology ( J:78406 )

• at E13.5, the mutant cochlea shows a slight growth delay which becomes evident at E16.5

decreased cochlea coiling ( J:78406 )

• at E16.5, the number of cochlear turns is reduced such that proper coiling is never achieved

abnormal semicircular canal morphology ( J:78406 )

• at E12.5, anterior and posterior canals are rarely recongizable in mildly affected mice

• in mild cases, the epithelium precursor of canals folds (with the lateral canal often identified), but canal plates never form properly and no fusion of the central plates occurs

• in severe cases, the entire canal region is reduced to a single vesicle by a thinner single-layered epithelium

• canal malformations are associated with altered patterns of apoptosis and proliferation at E11.5, and a severe reduction of Bmp4 expression within the developing otocyst

decreased lateral semicircular canal size ( J:78406 )

• at E12.5, a hypoplastic lateral canal is often recognized in mildly affected mice

abnormal crista ampullaris morphology ( J:78406 )

absent semicircular canals ( J:78406 )

• at E13.5, no canal-like structures are identified in severely affected mice

abnormal utricular macula morphology ( J:78406 )

• at E16.5, the sensory cells of utricular maculae are sparse and disorganized

• ectopic sensory cells are also observed

enlarged utricle ( J:78406 )

• at E12.5, the utricle is enlarged and malformed in severely affected mice

• at E16.5, the utricle is fused with the remnants of canals into a single vesicle

abnormal vestibular saccule morphology ( J:78406 )

• at E12.5, the saccule is slightly abnormal only in severely affected mice

abnormal vestibular saccular macula morphology ( J:78406 )

• at E16.5, the sensory cells of saccular maculae are sparse and disorganized

• ectopic sensory cells are also observed

short endolymphatic duct ( J:78406 )

• at E12.5, the endolymphatic duct forms but remains shorter and broader than normal in mildly affected mice

• in severe cases, the endolymphatic duct remains rudimentary

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

mortality/aging

neonatal lethality, complete penetrance ( J:56557 )

• no homozygotes survive beyond 24 hrs after birth

behavior/neurological

absent gastric milk in neonates ( J:56557 )

• newborn homozygotes contain no milk in their stomachs

abnormal suckling behavior ( J:56557 )

• newborn homozygotes are unable to suckle

decreased locomotor activity ( J:56557 )

• newborn homozygotes display reduced motility

growth/size/body

decreased maxillary shelf size ( J:56557 )

• at birth, the palatine processes of the maxilla are reduced, esp. with respect to their posterior development, and fail to properly connect with the palatine bones

small nasal bone ( J:56557 )

short nasal bone ( J:56557 )

absent palatine bone horizontal plate ( J:56557 )

• at birth, the horizontal laminae of the palatine bones are absent, exposing the presphenoid bone in ventral view

cleft secondary palate ( J:56557 )

• at birth, homozygotes display a cleft secondary palate

short snout ( J:56557 )

• at E14.5, homozygotes display a short snout

abnormal head development ( J:56557 )

• at E14.5, homozygotes show abnormal development of head structures

• in contrast, development of limbs and axial skeleton appears unaffected

distended abdomen ( J:56557 )

• newborn homozygotes have a bloated abdomen

meteorism ( J:56557 )

• newborn homozygotes accumulate air in their stomachs and intestines

respiratory system

small nasal bone ( J:56557 )

short nasal bone ( J:56557 )

abnormal superior horn of thyroid cartilage morphology ( J:56557 )

• at birth, the thyroid cartilage lacks its superior horns

respiratory distress ( J:56557 )

• newborn homozygotes display gasping

craniofacial

abnormal cranium morphology ( J:56557 )

• at birth, homozygotes exhibit severe defects in bones derived from the first four branchial arches that form the base and sides of the skull

wide cranial sutures ( J:56557 )

• at birth, homozygotes display wide cranial sutures with multiple Wormian bones

abnormal neurocranium morphology ( J:56557 )

• at birth, homozygotes display minor defects in neurocranial bones

Wormian bones ( J:56557 )

• multiple Wormian bones are present at birth

abnormal basioccipital bone morphology ( J:56557 )

• at birth, the basioccipital bone is elongated at the base of the skull, along the midline

abnormal styloid process morphology ( J:56557 )

• at birth, the styloid process is shorter than normal and is rotated toward the midline

short styloid process ( J:56557 )

• at birth

large anterior fontanelle ( J:56557 )

• at E14.5, homozygotes exhibit an open fontanelle

abnormal alisphenoid bone morphology ( J:56557 )

• at birth, the foramen rotundum and ovalis of the alisphenoid bone is malformed, with the foramina fused to form an abnormally large opening

abnormal pterygoid bone morphology ( J:56557 )

• at birth, the anterior part of the pterygoids is malformed

• mutant pterygoids display abnormal angles with respect to the basisphenoid bone

abnormal hyoid bone morphology ( J:56557 )

abnormal hyoid bone greater horn morphology ( J:56557 )

• the bigger horns are less affected but form an abnormal angle with the body of the hyoid bone

abnormal hyoid bone lesser horn morphology ( J:56557 )

• at birth, the lesser horns of the hyoid are reduced and rotated from their normal angle of attachment with the central bony structure

absent mandibular coronoid process ( J:56557 )

short mandible ( J:56557 )

• at birth, the mandible is malformed and shorter than normal

decreased maxillary shelf size ( J:56557 )

• at birth, the palatine processes of the maxilla are reduced, esp. with respect to their posterior development, and fail to properly connect with the palatine bones

short maxilla ( J:56557 )

small nasal bone ( J:56557 )

short nasal bone ( J:56557 )

absent palatine bone horizontal plate ( J:56557 )

• at birth, the horizontal laminae of the palatine bones are absent, exposing the presphenoid bone in ventral view

ectopic cranial bone ( J:56557 )

• at birth, the gonial bone is malformed and often fused to one or two ectopic bones not present in wild-type skulls

• ectopic bones extend from the gonial towards the pterygoid, and vary significantly in size and shape among individual mice as well as within the same individual between the left and right side

abnormal gonial bone morphology ( J:56557 )

• at birth, the gonial bone is malformed and often fused to one or two ectopic bones not present in wild-type skulls

absent stapes ( J:56557 )

• at E14.5, a few exencephalic homozygotes lack a stapes

• however, the shape, position and articulation of middle ear ossicles is generally normal at birth

abnormal craniofacial development ( J:56557 )

• at E14.5, exencephalic homozygotes exhibit a highly abnormal head and brain development, with more severe skull alterations than non-exencephalic homozygotes

• at birth, exencephalic homozygotes completely lack roof bones

abnormal Meckel's cartilage morphology ( J:56557 )

• at E14.5, homozygotes show shortening and rotation of the Meckel's cartilage

• Meckel's cartilage is disrupted near its proximal end generating an additional joint and and leaves the malleus primordium as an individual cartilage element

• one to two additional cartilage elements are ectopically formed where the gonial and tympanic bones are expected to form

• in some cases, one of these elements is fused with the distalmost portion of the malleus

short Meckel's cartilage ( J:56557 )

• shortening of Meckel's cartilage at E14.5

cleft secondary palate ( J:56557 )

• at birth, homozygotes display a cleft secondary palate

short snout ( J:56557 )

• at E14.5, homozygotes display a short snout

nervous system

exencephaly ( J:56557 )

• ~12% of mutant embryos and newborn mice display exencephaly

skeleton

abnormal osteoblast differentiation ( J:56557 )

• homozygotes exhibit increased osteocalcin expression in the periosteum, suggesting a lesion in osteoblast differentiation

abnormal cranium morphology ( J:56557 )

• at birth, homozygotes exhibit severe defects in bones derived from the first four branchial arches that form the base and sides of the skull

wide cranial sutures ( J:56557 )

• at birth, homozygotes display wide cranial sutures with multiple Wormian bones

abnormal Meckel's cartilage morphology ( J:56557 )

• at E14.5, homozygotes show shortening and rotation of the Meckel's cartilage

• Meckel's cartilage is disrupted near its proximal end generating an additional joint and and leaves the malleus primordium as an individual cartilage element

• one to two additional cartilage elements are ectopically formed where the gonial and tympanic bones are expected to form

• in some cases, one of these elements is fused with the distalmost portion of the malleus

short Meckel's cartilage ( J:56557 )

• shortening of Meckel's cartilage at E14.5

abnormal neurocranium morphology ( J:56557 )

• at birth, homozygotes display minor defects in neurocranial bones

Wormian bones ( J:56557 )

• multiple Wormian bones are present at birth

abnormal basioccipital bone morphology ( J:56557 )

• at birth, the basioccipital bone is elongated at the base of the skull, along the midline

abnormal styloid process morphology ( J:56557 )

• at birth, the styloid process is shorter than normal and is rotated toward the midline

short styloid process ( J:56557 )

• at birth

large anterior fontanelle ( J:56557 )

• at E14.5, homozygotes exhibit an open fontanelle

abnormal alisphenoid bone morphology ( J:56557 )

• at birth, the foramen rotundum and ovalis of the alisphenoid bone is malformed, with the foramina fused to form an abnormally large opening

abnormal pterygoid bone morphology ( J:56557 )

• at birth, the anterior part of the pterygoids is malformed

• mutant pterygoids display abnormal angles with respect to the basisphenoid bone

abnormal hyoid bone morphology ( J:56557 )

abnormal hyoid bone greater horn morphology ( J:56557 )

• the bigger horns are less affected but form an abnormal angle with the body of the hyoid bone

abnormal hyoid bone lesser horn morphology ( J:56557 )

• at birth, the lesser horns of the hyoid are reduced and rotated from their normal angle of attachment with the central bony structure

absent mandibular coronoid process ( J:56557 )

short mandible ( J:56557 )

• at birth, the mandible is malformed and shorter than normal

decreased maxillary shelf size ( J:56557 )

• at birth, the palatine processes of the maxilla are reduced, esp. with respect to their posterior development, and fail to properly connect with the palatine bones

short maxilla ( J:56557 )

small nasal bone ( J:56557 )

short nasal bone ( J:56557 )

absent palatine bone horizontal plate ( J:56557 )

• at birth, the horizontal laminae of the palatine bones are absent, exposing the presphenoid bone in ventral view

ectopic cranial bone ( J:56557 )

• at birth, the gonial bone is malformed and often fused to one or two ectopic bones not present in wild-type skulls

• ectopic bones extend from the gonial towards the pterygoid, and vary significantly in size and shape among individual mice as well as within the same individual between the left and right side

abnormal gonial bone morphology ( J:56557 )

• at birth, the gonial bone is malformed and often fused to one or two ectopic bones not present in wild-type skulls

absent stapes ( J:56557 )

• at E14.5, a few exencephalic homozygotes lack a stapes

• however, the shape, position and articulation of middle ear ossicles is generally normal at birth

abnormal superior horn of thyroid cartilage morphology ( J:56557 )

• at birth, the thyroid cartilage lacks its superior horns

ectopic cartilage ( J:56557 )

• at E14.5, one to two additional cartilage elements are ectopically formed at the site of interruption in Meckel's cartilage, where the gonial and tympanic bones are expected to form

abnormal bone ossification ( J:56557 )

• homozygotes show increased complexity of the structure of woven bone and a reduction in periosteal bone lamina

delayed bone ossification ( J:56557 )

• homozygotes display delayed ossification of the parietal, interparietal and superoccipital bones, resulting in an open fontanelle and wide cranial sutures

• at birth, exencephalic homozygotes completely lack roof bones

delayed cranial suture closure ( J:56557 )

hearing/vestibular/ear

abnormal gonial bone morphology ( J:56557 )

• at birth, the gonial bone is malformed and often fused to one or two ectopic bones not present in wild-type skulls

absent stapes ( J:56557 )

• at E14.5, a few exencephalic homozygotes lack a stapes

• however, the shape, position and articulation of middle ear ossicles is generally normal at birth

abnormal semicircular canal morphology ( J:56557 )

• the anterior and posterior canals fail to develop and are fused into one single large vesicle

decreased lateral semicircular canal size ( J:56557 )

• at birth, the horizontal canal is reduced in size

absent posterior semicircular canal ( J:56557 )

abnormal crista ampullaris morphology ( J:56557 )

• no crista ampullaris is identified at birth or any stage of development

abnormal crista ampullaris neuroepithelium morphology ( J:56557 )

• the epithelium is much thinner and consists of large flat cells

absent superior semicircular canal ( J:56557 )

abnormal inner ear vestibule morphology ( J:56557 )

• at E10.5, the vestibulum is always reduced and severely malformed

• vestibular dysgenesis is variable among individuals and between both ears within individuals

abnormal otic capsule morphology ( J:56557 )

• at birth, the vestibular portion of the otic capsule is always smaller, malformed and rotated

decreased tympanic ring size ( J:56557 )

• at birth, the tympanic ring is always reduced, although with significant variation among individual mice

digestive/alimentary system

decreased maxillary shelf size ( J:56557 )

• at birth, the palatine processes of the maxilla are reduced, esp. with respect to their posterior development, and fail to properly connect with the palatine bones

absent palatine bone horizontal plate ( J:56557 )

• at birth, the horizontal laminae of the palatine bones are absent, exposing the presphenoid bone in ventral view

cleft secondary palate ( J:56557 )

• at birth, homozygotes display a cleft secondary palate

meteorism ( J:56557 )

• newborn homozygotes accumulate air in their stomachs and intestines

cellular

abnormal osteoblast differentiation ( J:56557 )

• homozygotes exhibit increased osteocalcin expression in the periosteum, suggesting a lesion in osteoblast differentiation

abnormal cell proliferation ( J:56557 )

• at E10.5 and E11.5, homozygotes display the highest density of proliferating cells within the first branchial arch

Allelic Composition	Dlx5^tm1Levi/Dlx5⁺
Genetic Background	involves: 129P2/OlaHsd * C57BL/6 * DBA/2

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dlx5^tm1Levi mutation (1 available); any Dlx5 mutation (23 available)

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

normal phenotype

no abnormal phenotype detected ( J:56557 )

• in contrast to Dlx5^tm1.1Levi heterozygotes, these heterozygotes are viable and fertile and do not display any obvious abnormalities

Allelic Composition	Trp63^tm1Aam/Trp63^tm1Aam Dlx5^tm1Levi/Dlx5/Dlx6^tm1Levi
Genetic Background	involves: 129P2/OlaHsd

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dlx5/Dlx6^tm1Levi mutation (0 available); any Dlx6 mutation (29 available)
Dlx5^tm1Levi mutation (1 available); any Dlx5 mutation (23 available)
Trp63^tm1Aam mutation (0 available); any Trp63 mutation (60 available)

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

limbs/digits/tail

abnormal phalanx morphology ( J:135784 )

• mice exhibit dysmorphologies of the most proximal segments of the central phalanges

• in 2 of 3 mice the posterior fingers are missing

ectrodactyly ( J:135784 )

• all mice exhibit severe ectrodactyly in the hindlimbs with two to four fingers missing

monodactyly ( J:135784 )

• in one mouse

syndactyly ( J:135784 )

• in hind- and forelimbs

skeleton

abnormal phalanx morphology ( J:135784 )

• mice exhibit dysmorphologies of the most proximal segments of the central phalanges

• in 2 of 3 mice the posterior fingers are missing

Allelic Composition	Dlx5^tm1Levi/Dlx5/Dlx6^tm1Levi Trp63^tm1Aam/Trp63^tm1Aam
Genetic Background	involves: 129P2/OlaHsd

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dlx5/Dlx6^tm1Levi mutation (0 available); any Dlx5 mutation (23 available)
Dlx5^tm1Levi mutation (1 available); any Dlx5 mutation (23 available)
Trp63^tm1Aam mutation (0 available); any Trp63 mutation (60 available)

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

limbs/digits/tail

abnormal phalanx morphology ( J:135784 )

• mice exhibit dysmorphologies of the most proximal segments of the central phalanges

• in 2 of 3 mice the posterior fingers are missing

ectrodactyly ( J:135784 )

• all mice exhibit severe ectrodactyly in the hindlimbs with two to four fingers missing

monodactyly ( J:135784 )

• in one mouse

syndactyly ( J:135784 )

• in hind- and forelimbs

skeleton

abnormal phalanx morphology ( J:135784 )

• mice exhibit dysmorphologies of the most proximal segments of the central phalanges

• in 2 of 3 mice the posterior fingers are missing

Allelic Composition	Dlx5^tm1Levi/Dlx5^tm1Levi Msx1^tm1Bero/Msx1^tm1Bero
Genetic Background	involves: 129P2/OlaHsd * C57BL/6 * DBA/2

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dlx5^tm1Levi mutation (1 available); any Dlx5 mutation (23 available)
Msx1^tm1Bero mutation (1 available); any Msx1 mutation (18 available)

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

mortality/aging

neonatal lethality, complete penetrance ( J:104286 )

• double homozygotes die soon after delivery

craniofacial

abnormal neurocranium morphology ( J:104286 )

• at P0, double homozygotes exhibit severe retardation of mineralized bone deposition in calvaria

• at P0, double mutant vault bones are smaller and severely perforated with irregular borders relative to those seen in either single homozygote

abnormal basioccipital bone morphology ( J:104286 )

• at P0, double homozygotes display deformed basioccipitals, similar to Dlx5^tm1Levi homozygotes

abnormal basisphenoid bone morphology ( J:104286 )

• at P0, double homozygotes display deformed basisphenoids, similar to Dlx5^tm1Levi homozygotes

abnormal fontanelle morphology ( J:104286 )

• at P0, double mutant fontanels appear to be open on the midline

abnormal alisphenoid bone morphology ( J:104286 )

• at P0, double homozygotes display a disorganized foramina of the ala temporalis, similar to Dlx5^tm1Levi homozygotes

abnormal pterygoid bone morphology ( J:104286 )

• at P0, double homozygotes display deformed pterygoids, similar to Dlx5^tm1Levi homozygotes

abnormal temporal bone squamous part morphology ( J:104286 )

• at P0, double homozygotes display deformed squamosal bones, similar to Msx1^tm1Bero homozygotes

absent alveolar process ( J:104286 )

absent molars ( J:104286 )

• at P0, double homozygotes lack molar teeth, similar to Msx1^tm1Bero homozygotes

absent mandibular coronoid process ( J:104286 )

• at P0, double homozygotes lack the coronoid process, similar to single Dlx5^tm1Levi homozygotes

micrognathia ( J:104286 )

• at P0, double homozygotes display a shorter and coarser jaw relative to wild-type or to Msx1^tm1Bero homozygotes, although not as short as in Dlx5^tm1Levi homozygotes

abnormal malleus morphology ( J:104286 )

• at P0, double homozygotes display a malformed malleus with no head, similar to Msx1^tm1Bero homozygotes

• in addition, an ectopic bone is present, similar to Dlx5^tm1Levi homozygotes

absent malleus head ( J:104286 )

• at P0, double homozygotes display absence of caput mallei

abnormal nasal capsule morphology ( J:104286 )

• at P0, double homozygotes display hypoplasia and asymmetry of the nasal capsules, similar to Dlx5^tm1Levi homozygotes

palatal shelves fail to meet at midline ( J:104286 )

cleft secondary palate ( J:104286 )

• at E13.5, double homozygotes (2/2) display partly formed and elevated shelves which fail to come to contact and fuse

• at P0, double homozygotes (3/3) exhibit a mild clefting, as elevated shelves fail to fuse in the midline, indicating partial rescue of the severe Msx1-related palatal phenotype

digestive/alimentary system

palatal shelves fail to meet at midline ( J:104286 )

cleft secondary palate ( J:104286 )

• at E13.5, double homozygotes (2/2) display partly formed and elevated shelves which fail to come to contact and fuse

• at P0, double homozygotes (3/3) exhibit a mild clefting, as elevated shelves fail to fuse in the midline, indicating partial rescue of the severe Msx1-related palatal phenotype

skeleton

abnormal neurocranium morphology ( J:104286 )

• at P0, double homozygotes exhibit severe retardation of mineralized bone deposition in calvaria

• at P0, double mutant vault bones are smaller and severely perforated with irregular borders relative to those seen in either single homozygote

abnormal basioccipital bone morphology ( J:104286 )

• at P0, double homozygotes display deformed basioccipitals, similar to Dlx5^tm1Levi homozygotes

abnormal basisphenoid bone morphology ( J:104286 )

• at P0, double homozygotes display deformed basisphenoids, similar to Dlx5^tm1Levi homozygotes

abnormal fontanelle morphology ( J:104286 )

• at P0, double mutant fontanels appear to be open on the midline

abnormal alisphenoid bone morphology ( J:104286 )

• at P0, double homozygotes display a disorganized foramina of the ala temporalis, similar to Dlx5^tm1Levi homozygotes

abnormal pterygoid bone morphology ( J:104286 )

• at P0, double homozygotes display deformed pterygoids, similar to Dlx5^tm1Levi homozygotes

abnormal temporal bone squamous part morphology ( J:104286 )

• at P0, double homozygotes display deformed squamosal bones, similar to Msx1^tm1Bero homozygotes

absent alveolar process ( J:104286 )

absent molars ( J:104286 )

• at P0, double homozygotes lack molar teeth, similar to Msx1^tm1Bero homozygotes

absent mandibular coronoid process ( J:104286 )

• at P0, double homozygotes lack the coronoid process, similar to single Dlx5^tm1Levi homozygotes

micrognathia ( J:104286 )

• at P0, double homozygotes display a shorter and coarser jaw relative to wild-type or to Msx1^tm1Bero homozygotes, although not as short as in Dlx5^tm1Levi homozygotes

abnormal malleus morphology ( J:104286 )

• at P0, double homozygotes display a malformed malleus with no head, similar to Msx1^tm1Bero homozygotes

• in addition, an ectopic bone is present, similar to Dlx5^tm1Levi homozygotes

absent malleus head ( J:104286 )

• at P0, double homozygotes display absence of caput mallei

abnormal nasal capsule morphology ( J:104286 )

• at P0, double homozygotes display hypoplasia and asymmetry of the nasal capsules, similar to Dlx5^tm1Levi homozygotes

delayed bone ossification ( J:104286 )

• at P0, double hoomozygotes display delayed fore- and hindlimb ossification and a severe retardation of mineralized bone deposition in calvaria; only slight to mild delays are noted in single homozygotes

hearing/vestibular/ear

abnormal malleus morphology ( J:104286 )

• at P0, double homozygotes display a malformed malleus with no head, similar to Msx1^tm1Bero homozygotes

• in addition, an ectopic bone is present, similar to Dlx5^tm1Levi homozygotes

absent malleus head ( J:104286 )

• at P0, double homozygotes display absence of caput mallei

abnormal otic capsule morphology ( J:104286 )

• at birth, the vestibular portion of the otic capsule is always malformed, similar to Dlx5^tm1Levi homozygotes

respiratory system

abnormal nasal capsule morphology ( J:104286 )

• at P0, double homozygotes display hypoplasia and asymmetry of the nasal capsules, similar to Dlx5^tm1Levi homozygotes

growth/size/body

absent alveolar process ( J:104286 )

absent molars ( J:104286 )

• at P0, double homozygotes lack molar teeth, similar to Msx1^tm1Bero homozygotes

abnormal nasal capsule morphology ( J:104286 )

• at P0, double homozygotes display hypoplasia and asymmetry of the nasal capsules, similar to Dlx5^tm1Levi homozygotes

palatal shelves fail to meet at midline ( J:104286 )

cleft secondary palate ( J:104286 )

• at E13.5, double homozygotes (2/2) display partly formed and elevated shelves which fail to come to contact and fuse

• at P0, double homozygotes (3/3) exhibit a mild clefting, as elevated shelves fail to fuse in the midline, indicating partial rescue of the severe Msx1-related palatal phenotype

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