In:
Genes to Cells, Wiley, Vol. 21, No. 7 ( 2016-07), p. 728-739
Abstract:
Lrrc6 encodes a cytoplasmic protein that is expressed specifically in cells with motile cilia including the node, trachea and testes of the mice. A mutation of Lrrc6 has been identified in human patients with primary ciliary dyskinesia ( PCD ). Mutant mice lacking Lrrc6 show typical PCD defects such as hydrocephalus and laterality defects. We found that in the absence of Lrrc6, the morphology of motile cilia remained normal, but their motility was completely lost. The 9 + 2 arrangement of microtubules remained normal in Lrrc6 ‐/‐ mice, but the outer dynein arms ( ODA s), the structures essential for the ciliary beating, were absent from the cilia. In the absence of Lrrc6, ODA proteins such as DNAH 5, DNAH 9 and IC 2, which are assembled in the cytoplasm and transported to the ciliary axoneme, remained in the cytoplasm and were not transported to the ciliary axoneme. The IC 2– IC 1 interaction, which is the first step of ODA assembly, was normal in Lrrc6 −/− mice testes. Our results suggest that ODA proteins may be transported from the cytoplasm to the cilia by an Lrrc6‐dependent mechanism.
Type of Medium:
Online Resource
ISSN:
1356-9597
,
1365-2443
DOI:
10.1111/gtc.2016.21.issue-7
Language:
English
Publisher:
Wiley
Publication Date:
2016
detail.hit.zdb_id:
2020308-1
SSG:
12
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