Phenotypes associated with this allele

• Allele Symbol: Tg(Dspp-TGFB1)3Kul
• Allele Name: transgene insertion 3, Ashok B Kulkarni
• Allele ID: MGI:3828146
• Summary: 2 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
tg1	Tg(Dspp-TGFB1)3Kul/?	FVB/N-Tg(Dspp-TGFB1)3Kul	MGI:3835822
tg2	Tg(Dspp-TGFB1)3Kul/?	involves: FVB/N	MGI:3835811

Genotype
MGI:3835822

tg1

• Allelic Composition: Tg(Dspp-TGFB1)3Kul/?
• Genetic Background: FVB/N-Tg(Dspp-TGFB1)3Kul

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

Gross tooth abnormalities in Tg(Dspp-TGFB1)3Kul/? mice

craniofacial

abnormal tooth mineralization ( J:118918 )

• mineralization of the incisors is reduced by about 90% compared to controls based on X-ray analysis

• molar mineralization is reduced by 62%

• calcium mineralization is unevenly distributed throughout the dentin and enamel

abnormal dentin morphology ( J:118918 )

• incisors have a highly disorganized odontoblast layer and irregular dentinal tubules all along the dentinal layer

brittle teeth ( J:118918 )

• teeth turn chalky-white and then fracture starting around 2 weeks of age

growth/size/body

abnormal tooth mineralization ( J:118918 )

• mineralization of the incisors is reduced by about 90% compared to controls based on X-ray analysis

• molar mineralization is reduced by 62%

• calcium mineralization is unevenly distributed throughout the dentin and enamel

abnormal dentin morphology ( J:118918 )

• incisors have a highly disorganized odontoblast layer and irregular dentinal tubules all along the dentinal layer

brittle teeth ( J:118918 )

• teeth turn chalky-white and then fracture starting around 2 weeks of age

skeleton

abnormal tooth mineralization ( J:118918 )

• mineralization of the incisors is reduced by about 90% compared to controls based on X-ray analysis

• molar mineralization is reduced by 62%

• calcium mineralization is unevenly distributed throughout the dentin and enamel

abnormal dentin morphology ( J:118918 )

• incisors have a highly disorganized odontoblast layer and irregular dentinal tubules all along the dentinal layer

brittle teeth ( J:118918 )

• teeth turn chalky-white and then fracture starting around 2 weeks of age

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
dentin dysplasia		DOID:701	OMIM:125400 OMIM:125420	J:118918

Genotype
MGI:3835811

tg2

• Allelic Composition: Tg(Dspp-TGFB1)3Kul/?
• Genetic Background: involves: FVB/N

craniofacial

absent Tomes' process ( J:145682 )

ameloblast degeneration ( J:145682 )

• ameloblasts appear normal until the presecretory stage when they began to detach from the dentin surface

• in incisors of 2 day old mice, ameloblasts detach from the labial dentin surface of the teeth, secrete bubble-like masses, and form cysts without the formation of a Tomes' process

• ameoblasts adjacent these cysts fail to properly mature

• ameoblasts also detach from the dentin of the first and second molars starting at postnatal day 7

abnormal dentin morphology ( J:145682 )

• dentin is disorganized and fractured around the root of incisors in 30 day old mice

• odontoblasts are sometimes trapped in the dentin resulting in an osteodentin phenotype

abnormal dentin mineralization ( J:145682 )

• dentin is abnormally mineralized

abnormal enamel rod pattern ( J:145682 )

delaminated enamel ( J:145682 )

• the very thin enamel of young mice is quickly lost through attrition or fracturing

enamel pits ( J:145682 )

• the enamel of molars has a pitted appearance

reduced enamel thickness ( J:145682 )

• most mice initially have very thin enamel on their teeth before losing it

• the enamel of incisors from 7 day old mice is aprismatic and amorphous

• enamel lacks the rod-like that help characterize the enamel-dentin junction

skeleton

absent Tomes' process ( J:145682 )

ameloblast degeneration ( J:145682 )

• ameloblasts appear normal until the presecretory stage when they began to detach from the dentin surface

• in incisors of 2 day old mice, ameloblasts detach from the labial dentin surface of the teeth, secrete bubble-like masses, and form cysts without the formation of a Tomes' process

• ameoblasts adjacent these cysts fail to properly mature

• ameoblasts also detach from the dentin of the first and second molars starting at postnatal day 7

abnormal dentin morphology ( J:145682 )

• dentin is disorganized and fractured around the root of incisors in 30 day old mice

• odontoblasts are sometimes trapped in the dentin resulting in an osteodentin phenotype

abnormal dentin mineralization ( J:145682 )

• dentin is abnormally mineralized

abnormal enamel rod pattern ( J:145682 )

delaminated enamel ( J:145682 )

• the very thin enamel of young mice is quickly lost through attrition or fracturing

enamel pits ( J:145682 )

• the enamel of molars has a pitted appearance

reduced enamel thickness ( J:145682 )

• most mice initially have very thin enamel on their teeth before losing it

• the enamel of incisors from 7 day old mice is aprismatic and amorphous

• enamel lacks the rod-like that help characterize the enamel-dentin junction

growth/size/body

absent Tomes' process ( J:145682 )

ameloblast degeneration ( J:145682 )

• ameloblasts appear normal until the presecretory stage when they began to detach from the dentin surface

• in incisors of 2 day old mice, ameloblasts detach from the labial dentin surface of the teeth, secrete bubble-like masses, and form cysts without the formation of a Tomes' process

• ameoblasts adjacent these cysts fail to properly mature

• ameoblasts also detach from the dentin of the first and second molars starting at postnatal day 7

abnormal dentin morphology ( J:145682 )

• dentin is disorganized and fractured around the root of incisors in 30 day old mice

• odontoblasts are sometimes trapped in the dentin resulting in an osteodentin phenotype

abnormal dentin mineralization ( J:145682 )

• dentin is abnormally mineralized

abnormal enamel rod pattern ( J:145682 )

delaminated enamel ( J:145682 )

• the very thin enamel of young mice is quickly lost through attrition or fracturing

enamel pits ( J:145682 )

• the enamel of molars has a pitted appearance

reduced enamel thickness ( J:145682 )

• most mice initially have very thin enamel on their teeth before losing it

• the enamel of incisors from 7 day old mice is aprismatic and amorphous

• enamel lacks the rod-like that help characterize the enamel-dentin junction