All Phenotypes Tbx10<Dc> MGI Mouse

small vestibular ganglion ( J:5022 )

• the vestibular ganglion does not extend much beyond the superior border of the saccular macula and has fewer, less densely packed cells

vestibular ganglion hypoplasia ( J:5022 )

abnormal vestibular nerve morphology ( J:5022 )

• the superior vestibular nerve enters the saccular macula near the center of the macula, rather than the dorsal border

• along with the utricular macula, the utricular branch of the superior vestibular nerve is entirely missing

• the pathway of the nerve from the superior vestibular ganglion to the crista rarely takes a normal course along the base of the utricle, but often passes through the otic capsule and tympanic cavity, and is often branched

• the lateral vestibular nerve rarely follows a normal course, and the nerve is very thin in instances when the crista is small

hearing/vestibular/ear phenotype ( J:5022 )

N	• heterozygotes are not deaf, the cochlea appears normal

abnormal bony labyrinth ( J:5022 )

abnormal ear development ( J:5022 )

• by embryonic day 10 the acoustic ganglion is smaller and does not curve around to reach the anterior aspect of the vesicle, and the epithelium on the antero-lateral aspect is not thickened as in normal embryos

• by embryonic day 11 the nerve that would normally innervate the epithelium destined to become the utricular macula and superior lateral cristae is deficient and aberrent or entirely lacking

• by embryonic day 12 the utriculo-saccular folds are abnormal in appearance, the utricular nerve is always missing, the nerves of the superior and lateral cristae are sometimes missing, and the accessory nerve from the superior vestibular ganglion sometimes branches to one of the critae and is always thinner than normal

• by embryonic day 13 the shape and size of the utricle and saccule are abnormal, the utricle has no nerve supply and no macula, the nerves of the superior and lateral semicircular ducts are often missing, and the utriculo-saccular fold is thicker than normal, slants in the medial direction, and is foreshortened posteriorly

• by embryonic day 16 the absence of the utricular valve and saccular duct is evident

abnormal semicircular canal morphology ( J:5022 )

abnormal lateral semicircular canal morphology ( J:5022 )

• the majority of heterozygotes have a unilateral or bilateral severe narrowing, complete obliteration of the lumen, or absence of the ampullar end of the lateral semicircular duct and constriction or absence of the bony canal in this region

• this abnormality is more severe in the lateral than the superior semicircular canal

abnormal semicircular canal ampulla morphology ( J:5022 )

abnormal crista ampullaris morphology ( J:5022 )

• when narrowing is found in the superior semicircular duct, the crista is either missing or poorly formed, and even when normal in appearance the crista is often shifted in its location

abnormal superior semicircular canal morphology ( J:5022 )

• the majority of heterozygotes have a unilateral or bilateral severe narrowing or complete obliteration of the lumen of the ampullar end of the superior semicircular duct and constriction of the bony canal in this region

abnormal inner ear vestibule morphology ( J:5022 )

abnormal vestibular labyrinth morphology ( J:5022 )

abnormal utricle morphology ( J:5022 )

abnormal utricular macula morphology ( J:5022 )

• in all dancer mice the utricular macula and utricular branch of the superior vestibular nerve are entirely missing

small utricle ( J:5022 )

• the utricle is smaller than normal, particularly dorso-ventrally

abnormal vestibular saccule morphology ( J:5022 )

• the saccule, affected in all heterozygotes, does not extent dorsally as far as the utricle and is attached to the utricle by a thick strand of cells without any lumen

• the free wall of the saccule is sometimes highly convoluted

small vestibular saccule ( J:5022 )

• the saccule is smaller and narrower than normal

abnormal postural reflex ( J:5022 )

• when lifted by the tail at approximately 3 days of age a heterozygote will hump its back instead of arching it, retract the front legs and bend the head toward the belly

• when lifted by the tail as an adult, a heterozygote will twist and turn and throw its body about

impaired swimming ( J:5022 , J:24773 )

head tilt ( J:5022 )

• some, but not all, may hold their heads tilted to the side

head tossing ( J:5022 )

• heterozygotes make jerking movements of the head in both horizontal and vertical planes

abnormal gait ( J:5022 )

• by 2 weeks of age heterozygotes decelop a weaving gait with bi-directional circling

hyperactivity ( J:5022 )

• evident by 1 week of age and increasing by 2 weeks of age

circling ( J:24773 )

• similar circling behavior as that of waltzer mutants but with less head movement

bidirectional circling ( J:5022 )

cleft palate ( J:7364 )

• Background Sensitivity: does not occur spontaneously but can be induced on this background by treatment of pregnant mothers with 6-aminonicotinamide

decreased body size ( J:5022 )

• smaller than wild-type littermates by 1 week of age

head spot ( J:5022 )

• most dancer mutants have a white spot, varying in size, in the middle of the head

abnormal bony labyrinth ( J:5022 )

cleft palate ( J:7364 )

• Background Sensitivity: does not occur spontaneously but can be induced on this background by treatment of pregnant mothers with 6-aminonicotinamide

cleft palate ( J:7364 )

• Background Sensitivity: does not occur spontaneously but can be induced on this background by treatment of pregnant mothers with 6-aminonicotinamide

head spot ( J:5022 )

• most dancer mutants have a white spot, varying in size, in the middle of the head

abnormal bony labyrinth ( J:5022 )

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

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

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

Contributing Projects: Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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