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Publication : Hearing impairment in mice with the cmd/cmd (cartilage matrix deficiency) mutant gene.

First Author  Yoo TJ Year  1991
Journal  Ann N Y Acad Sci Volume  630
Pages  265-7 PubMed ID  1952601
Mgi Jnum  J:553 Mgi Id  MGI:49090
Doi  10.1111/j.1749-6632.1991.tb19600.x Citation  Yoo TJ, et al. (1991) Hearing impairment in mice with the cmd/cmd (cartilage matrix deficiency) mutant gene. Ann N Y Acad Sci 630:265-7
abstractText  Mice homozygous for the autosomal recessive gene cartilage matrix deficiency (cmd) are afflicted with lesions involving cartilaginous tissue which give rise to, among other things, marked hearing loss as evidenced by auditory evoked potentials. Ultrastructural studies of the inner ear reveal that while inner hair cells are normal in shape and content, the outer hair cells have disappeared and there is some debris in Nuel's space. The pillar cells are normal as are the stria vascularis, basilar membrane, and tectorial membrane. We conclude that the cmd gene, thought to be vital in the regulation of proteoglycan synthesis, is responsible for the hearing impairment and structural anomalies of the cochlea seen in these mutant, homozygous mice.
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