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Usage Information

Nucleocytoplasmic connections and deafness
Howard J. Worman, Neil Segil
Howard J. Worman, Neil Segil
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Nucleocytoplasmic connections and deafness

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Abstract

The linker of nucleoskeleton and cytoskeleton (LINC) complex connects the nuclear lamina to the cytoskeleton, in part to aid in nuclear positioning. Mutations in genes encoding LINC complex and lamina components cause a range of human diseases. In this issue of the JCI, Horn et al. report that mutations in the gene SYNE4 encoding the LINC complex protein nesprin-4 lead to progressive high-frequency hearing loss. Further, in mice deficient in nesprin-4 and Sun1, another LINC complex component, outer hair cells of the cochlea form normally during development, but die in the early postnatal weeks. These results link improper nuclear positioning specifically to the death of outer hair cells in the organ of Corti and ultimately to deafness.

Authors

Howard J. Worman, Neil Segil

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Usage data is cumulative from November 2025 through November 2026.

Usage JCI PMC
Text version 646 24
PDF 314 9
Figure 218 1
Citation downloads 246 0
Totals 1,424 34
Total Views 1,458

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ISSN: 0021-9738 (print), 1558-8238 (online)

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