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ILDR1 deficiency causes degeneration of cochlear outer hair cells and disrupts the structure of the organ of Corti: a mouse model for human DFNB42.

Sang Q, Li W, Xu Y, Qu R, Xu Z, Feng R, Jin L, He L, Li H, Wang L - Biol Open (2015)

Bottom Line: ILDR1 deficiency affects expression of tricellulin in vivo, and this provides a possible explanation to hearing loss.Gene ontology classification indicated that a number of differentially expressed proteins are involved in cell adhesion, protein and vesicle-mediated transport, cell death, membrane organization, and cellular homeostasis.A few of these proteins are closely related to hearing development.

View Article: PubMed Central - PubMed

Affiliation: State Key Laboratory of Genetic Engineering and MOE Key Laboratory of Contemporary Anthropology, School of Life Sciences, Fudan University, Shanghai, 200032, PR China Institute of Biomedical Sciences, Fudan University, No 138 Yixueyuan Road, Shanghai, 200032, PR China.

No MeSH data available.


Related in: MedlinePlus

Expression of tricellulin in tight junctions was decreased in Ildr1−/− mice.(A–F) Immunofluorescence of the organ of Corti from the middle turn of P28 mice using tricellulin and ZO-1 antibody. Arrows in B,C show the localization of tricellulin in the tricellular tight junctions. (G) Western blot analysis of tricellulin in the basilar membrane at P15 and P21 Ildr1+/− and Ildr1−/− mice. Gapdh was used as a loading control.
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f07: Expression of tricellulin in tight junctions was decreased in Ildr1−/− mice.(A–F) Immunofluorescence of the organ of Corti from the middle turn of P28 mice using tricellulin and ZO-1 antibody. Arrows in B,C show the localization of tricellulin in the tricellular tight junctions. (G) Western blot analysis of tricellulin in the basilar membrane at P15 and P21 Ildr1+/− and Ildr1−/− mice. Gapdh was used as a loading control.

Mentions: Because ILDR1 has been showed to be localized at the tricellular tight junctions in the organ of Corti of mice and to play a role in the recruitment of tricellulin in epithelial cells (Higashi et al., 2013), we sought to determine if tricellulin is altered in Ildr1−/− mice. As shown by immunofluorescence, tricellulin located at the tricellular tight junctions was significantly decreased in the cochleae of Ildr1−/− mice compared with the Ildr1+/− mice (Fig. 7C,F). This was confirmed by western blot with basilar membrane proteins using tricellulin antibody at P15 and P21 mice (Fig. 7G). It indicates that hearing loss caused by ILDR1 deficiency might be through the abnormal expression of tricellulin.


ILDR1 deficiency causes degeneration of cochlear outer hair cells and disrupts the structure of the organ of Corti: a mouse model for human DFNB42.

Sang Q, Li W, Xu Y, Qu R, Xu Z, Feng R, Jin L, He L, Li H, Wang L - Biol Open (2015)

Expression of tricellulin in tight junctions was decreased in Ildr1−/− mice.(A–F) Immunofluorescence of the organ of Corti from the middle turn of P28 mice using tricellulin and ZO-1 antibody. Arrows in B,C show the localization of tricellulin in the tricellular tight junctions. (G) Western blot analysis of tricellulin in the basilar membrane at P15 and P21 Ildr1+/− and Ildr1−/− mice. Gapdh was used as a loading control.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4400585&req=5

f07: Expression of tricellulin in tight junctions was decreased in Ildr1−/− mice.(A–F) Immunofluorescence of the organ of Corti from the middle turn of P28 mice using tricellulin and ZO-1 antibody. Arrows in B,C show the localization of tricellulin in the tricellular tight junctions. (G) Western blot analysis of tricellulin in the basilar membrane at P15 and P21 Ildr1+/− and Ildr1−/− mice. Gapdh was used as a loading control.
Mentions: Because ILDR1 has been showed to be localized at the tricellular tight junctions in the organ of Corti of mice and to play a role in the recruitment of tricellulin in epithelial cells (Higashi et al., 2013), we sought to determine if tricellulin is altered in Ildr1−/− mice. As shown by immunofluorescence, tricellulin located at the tricellular tight junctions was significantly decreased in the cochleae of Ildr1−/− mice compared with the Ildr1+/− mice (Fig. 7C,F). This was confirmed by western blot with basilar membrane proteins using tricellulin antibody at P15 and P21 mice (Fig. 7G). It indicates that hearing loss caused by ILDR1 deficiency might be through the abnormal expression of tricellulin.

Bottom Line: ILDR1 deficiency affects expression of tricellulin in vivo, and this provides a possible explanation to hearing loss.Gene ontology classification indicated that a number of differentially expressed proteins are involved in cell adhesion, protein and vesicle-mediated transport, cell death, membrane organization, and cellular homeostasis.A few of these proteins are closely related to hearing development.

View Article: PubMed Central - PubMed

Affiliation: State Key Laboratory of Genetic Engineering and MOE Key Laboratory of Contemporary Anthropology, School of Life Sciences, Fudan University, Shanghai, 200032, PR China Institute of Biomedical Sciences, Fudan University, No 138 Yixueyuan Road, Shanghai, 200032, PR China.

No MeSH data available.


Related in: MedlinePlus