Development of the inner ear in Splotch mutant mice
Copyright policy )Development of the inner ear in Splotch mutant mice
The Splotch mouse, a Pax 3 mutation, represents a model of Waardenburg syndrome I. We show that the homozygous Splotch mutation (Sp(2H)) is associated with severe defects that prevent the formation of the cochlea and vestibulo-cochlear ganglion. To clarify the role of Pax 3 in inner ear formation, we examined the expression of polysialic acid (PSA) associated with neural cell adhesion molecule (NCAM). In accordance with the occurrence of phenotypic abnormalities, PSA NCAM was expressed early in otocyst development in the otic epithelium and the vestibulo-cochlear anlage. During the period of vestibular and cochlear ganglia formation, PSA NCAM expression was decreased. In the late phase of embryonic development, the expression of calcium binding proteins (S100) in the vestibulo-cochlear ganglion was also decreased. Minor differences in S100 immunostaining were found postnatally between the cochleas of heterozygous and wild type animals.
- Institute of Experimental Medicine Russian Federation
- Department of Medical Sciences Russian Federation
- Czech Academy of Sciences Czech Republic
Cell Death, S100 Proteins, Age Factors, Gene Expression Regulation, Developmental, Cell Count, Neural Cell Adhesion Molecule L1, Embryo, Mammalian, Immunohistochemistry, Mice, Mutant Strains, DNA-Binding Proteins, Mice, Animals, Newborn, Ear, Inner, Mutation, In Situ Nick-End Labeling, Sialic Acids, Animals, Paired Box Transcription Factors, PAX3 Transcription Factor, Transcription Factors
Cell Death, S100 Proteins, Age Factors, Gene Expression Regulation, Developmental, Cell Count, Neural Cell Adhesion Molecule L1, Embryo, Mammalian, Immunohistochemistry, Mice, Mutant Strains, DNA-Binding Proteins, Mice, Animals, Newborn, Ear, Inner, Mutation, In Situ Nick-End Labeling, Sialic Acids, Animals, Paired Box Transcription Factors, PAX3 Transcription Factor, Transcription Factors
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