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Trafficking abnormality and ER stress underlie functional deficiency of hearing impairment-associated connexin-31 mutants.

Abstract：

:Hearing impairment (HI) affects 1/1000 children and over 2% of the aged population. We have previously reported that mutations in the gene encoding gap junction protein connexin-31 (C×31) are associated with HI. The pathological mechanism of the disease mutations remains unknown. Here, we show that expression of C×31 in the mouse inner ear is developmentally regulated with a high level in adult inner hair cells and spiral ganglion neurons that are critical for the hearing process. In transfected cells, wild type C×31 protein (C×31wt) forms functional gap junction at cell-cell-contacts. In contrast, two HI-associated C×31 mutants, C×31R180X and C×31E183K resided primarily in the ER and Golgi-like intracellular punctate structures, respectively, and failed to mediate lucifer yellow transfer. Expression of C×31 mutants but not C×31wt leads to upregulation of and increased association with the ER chaperone BiP indicating ER stress induction. Together, the HI-associated C×31 mutants are impaired in trafficking, promote ER stress, and hence lose the ability to assemble functional gap junctions. The study reveals a potential pathological mechanism of HI-associated C×31 mutations.

journal_name

Protein Cell

journal_title

Protein & cell

authors

Xia K,Ma H,Xiong H,Pan Q,Huang L,Wang D,Zhang Z

doi

10.1007/s13238-010-0118-7

subject

Has Abstract

pub_date

2010-10-01 00:00:00

pages

935-43

issue

10

eissn

1674-800X

issn

1674-8018

journal_volume

1

pub_type

杂志文章

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