Mutations in ap1b1 cause mistargeting of the Na(+)/K(+)-ATPase pump in sensory hair cells.
Mutations in ap1b1 cause mistargeting of the Na(+)/K(+)-ATPase pump in sensory hair cells.
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DOI：
10.1371/journal.pone.0060866
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发表时间：
2013
期刊：
影响因子：
3.7
通讯作者：
Nicolson T
中科院分区：
文献类型：
--
作者：
Clemens Grisham R;Kindt K;Finger-Baier K;Schmid B;Nicolson T
The hair cells of the inner ear are polarized epithelial cells with a specialized structure at the apical surface, the mechanosensitive hair bundle. Mechanotransduction occurs within the hair bundle, whereas synaptic transmission takes place at the basolateral membrane. The molecular basis of the development and maintenance of the apical and basal compartments in sensory hair cells is poorly understood. Here we describe auditory/vestibular mutants isolated from forward genetic screens in zebrafish with lesions in the adaptor protein 1 beta subunit 1 (ap1b1) gene. Ap1b1 is a subunit of the adaptor complex AP-1, which has been implicated in the targeting of basolateral membrane proteins. In ap1b1 mutants we observed that although the overall development of the inner ear and lateral-line organ appeared normal, the sensory epithelium showed progressive signs of degeneration. Mechanically-evoked calcium transients were reduced in mutant hair cells, indicating that mechanotransduction was also compromised. To gain insight into the cellular and molecular defects in ap1b1 mutants, we examined the localization of basolateral membrane proteins in hair cells. We observed that the Na+/K+-ATPase pump (NKA) was less abundant in the basolateral membrane and was mislocalized to apical bundles in ap1b1 mutant hair cells. Accordingly, intracellular Na+ levels were increased in ap1b1 mutant hair cells. Our results suggest that Ap1b1 is essential for maintaining integrity and ion homeostasis in hair cells.
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DOI：
10.1002/cne.22047
发表时间：
2009-06-20
期刊：
The Journal of comparative neurology
影响因子：
--
作者：
Altick AL;Baryshnikova LM;Vu TQ;von Bartheld CS
通讯作者：
von Bartheld CS
影响因子：
64.5
作者：
Fölsch, H;Ohno, H;Mellman, I
通讯作者：
Mellman, I
影响因子：
4.8
作者：
Efendiev, Riad;Budu, Claudia E.;Pedemonte, Carlos H.
通讯作者：
Pedemonte, Carlos H.
影响因子：
11.8
作者：
Gravotta, Diego;Carvajal-Gonzalez, Jose Maria;Mattera, Rafael;Deborde, Sylvie;Banfelder, Jason R.;Bonifacino, Juan S.;Rodriguez-Boulan, Enrique
通讯作者：
Rodriguez-Boulan, Enrique
影响因子：
2.8
作者：
Kim, Sung Huhn;Marcus, Daniel C.
通讯作者：
Marcus, Daniel C.