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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID 		Dlx1tm1Jlr
targeted mutation 1, John L R Rubenstein
MGI:1857826
Summary 3 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
 		Dlx1tm1Jlr/Dlx1tm1Jlr involves: 129X1/SvJ MGI:3607399
hm2
 		Dlx1tm1Jlr/Dlx1tm1Jlr involves: 129X1/SvJ * C57BL/6J * CD-1 MGI:3701138
cx3
 		Dlx1tm1Jlr/Dlx1tm1Jlr
Dlx6tm1Jlr/Dlx6tm1Jlr 		involves: 129X1/SvJ MGI:3809893


Genotype
MGI:3607399
hm1
Allelic
Composition		 Dlx1tm1Jlr/Dlx1tm1Jlr
Genetic
Background		 involves: 129X1/SvJ
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dlx1tm1Jlr mutation (1 available); any Dlx1 mutation (21 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
mortality/aging
premature death ( J:40427 )
• homozygotes die within 1 month of birth

craniofacial
abnormal stapes morphology ( J:40427 )
• in about 50% of homozygotes the stapes is smaller and lacks a central hole while the styloids lack a connection with the crista parotica of the otic capsule
absent stapes obturator foramen ( J:40427 )
• the stapes lacks a central hole in about 50% of homozygotes
small stapes ( J:40427 )
• the stapes is smaller in about 50% of homozygotes
cleft palate ( J:40427 )
• in about 10% of homozygotes a small cleft is present

hearing/vestibular/ear
absent stapedial artery ( J:40427 )
• the stapedial artery is absent in those homozygotes where the stapes lacks a central hole
abnormal stapes morphology ( J:40427 )
• in about 50% of homozygotes the stapes is smaller and lacks a central hole while the styloids lack a connection with the crista parotica of the otic capsule
absent stapes obturator foramen ( J:40427 )
• the stapes lacks a central hole in about 50% of homozygotes
small stapes ( J:40427 )
• the stapes is smaller in about 50% of homozygotes

cardiovascular system
absent stapedial artery ( J:40427 )
• the stapedial artery is absent in those homozygotes where the stapes lacks a central hole

growth/size/body
cleft palate ( J:40427 )
• in about 10% of homozygotes a small cleft is present
decreased body size ( J:40427 )

nervous system
abnormal facial nerve morphology ( J:40427 )
• the proximal parasympathetic branch to the maxillary region runs medially over the dorsum of the cochlear promontory leaving the cranial cavity via the carotid foramen rather than being entirely extracranial

skeleton
abnormal stapes morphology ( J:40427 )
• in about 50% of homozygotes the stapes is smaller and lacks a central hole while the styloids lack a connection with the crista parotica of the otic capsule
absent stapes obturator foramen ( J:40427 )
• the stapes lacks a central hole in about 50% of homozygotes
small stapes ( J:40427 )
• the stapes is smaller in about 50% of homozygotes

digestive/alimentary system
cleft palate ( J:40427 )
• in about 10% of homozygotes a small cleft is present




Genotype
MGI:3701138
hm2
Allelic
Composition		 Dlx1tm1Jlr/Dlx1tm1Jlr
Genetic
Background		 involves: 129X1/SvJ * C57BL/6J * CD-1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dlx1tm1Jlr mutation (1 available); any Dlx1 mutation (21 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
hearing/vestibular/ear
increased or absent threshold for auditory brainstem response ( J:108265 )
• at 7-8 weeks of age, homozygotes display significantly elevated ABR thresholds for frequencies less than 32 kHz relative to wild-type mice
conductive hearing loss ( J:108265 )
• at 7-8 weeks of age, homozygotes exhibit a severe peripheral hearing loss that is attributed to middle ear dysfunction




Genotype
MGI:3809893
cx3
Allelic
Composition		 Dlx1tm1Jlr/Dlx1tm1Jlr
Dlx6tm1Jlr/Dlx6tm1Jlr
Genetic
Background		 involves: 129X1/SvJ
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dlx1tm1Jlr mutation (1 available); any Dlx1 mutation (21 available)
Dlx6tm1Jlr mutation (1 available); any Dlx6 mutation (29 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
craniofacial
abnormal cranium morphology ( J:139700 )
• the ventrolateral side of the skull exhibits a duplicate lamina obturans beside the endogenous one
abnormal pterygoid bone morphology ( J:139700 )
• unlike in wild-type mice, dentaries are shortened, fragmented and bifurcated to become bones resembling the maxilla, jugal and pterygoid
abnormal hyoid bone morphology ( J:139700 )
• mice exhibit an abnormal basihyoid and lesser horn of the hyoid
abnormal mandible morphology ( J:139700 )
• unlike in wild-type mice, dentaries are shortened, fragmented and bifurcated to become bones resembling the maxilla, jugal and pterygoid
abnormal zygomatic bone morphology ( J:139700 )
• unlike in wild-type mice, dentaries are shortened, fragmented and bifurcated to become bones resembling the maxilla, jugal and pterygoid

hearing/vestibular/ear
absent tympanic ring ( J:139700 )
• the ectotympanic is reduced or lost
decreased tympanic ring size ( J:139700 )
• the ectotympanic is reduced or lost

skeleton
abnormal cranium morphology ( J:139700 )
• the ventrolateral side of the skull exhibits a duplicate lamina obturans beside the endogenous one
abnormal pterygoid bone morphology ( J:139700 )
• unlike in wild-type mice, dentaries are shortened, fragmented and bifurcated to become bones resembling the maxilla, jugal and pterygoid
abnormal hyoid bone morphology ( J:139700 )
• mice exhibit an abnormal basihyoid and lesser horn of the hyoid
abnormal mandible morphology ( J:139700 )
• unlike in wild-type mice, dentaries are shortened, fragmented and bifurcated to become bones resembling the maxilla, jugal and pterygoid
abnormal zygomatic bone morphology ( J:139700 )
• unlike in wild-type mice, dentaries are shortened, fragmented and bifurcated to become bones resembling the maxilla, jugal and pterygoid

growth/size/body
abnormal hyoid bone morphology ( J:139700 )
• mice exhibit an abnormal basihyoid and lesser horn of the hyoid
abnormal hyoid bone body morphology ( J:139700 )
abnormal hyoid bone lesser horn morphology ( J:139700 )
abnormal mandible morphology ( J:139700 )
• unlike in wild-type mice, dentaries are shortened, fragmented and bifurcated to become bones resembling the maxilla, jugal and pterygoid
abnormal zygomatic bone morphology ( J:139700 )
• unlike in wild-type mice, dentaries are shortened, fragmented and bifurcated to become bones resembling the maxilla, jugal and pterygoid




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09/30/2025
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