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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

X-gal staining for beta-galactosidase activity in the organ of Corti in P2 (A), P5 (B), and P10 (C) Ildr1+/− mice.Arrowheads in A–C point to the three lines of outer hair cells and arrows in A–C point to the single line of inner hair cells.
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f02: X-gal staining for beta-galactosidase activity in the organ of Corti in P2 (A), P5 (B), and P10 (C) Ildr1+/− mice.Arrowheads in A–C point to the three lines of outer hair cells and arrows in A–C point to the single line of inner hair cells.

Mentions: Ildr1, the gene encoding ILDR1, is located on chromosome 16. It has eight exons and encodes the entire protein of 537 amino acids. A targeting vector was constructed and used to put a neomycin cassette and the LacZ reporter gene in the upstream region of exon 2 (Fig. 1A). The LacZ-Neo cassette serves as a positive selection marker in the ES targeting step and as the reporter for exploring the expression pattern of ILDR1 (Fig. 2). Exon 2 and exon 3 were floxed by loxp site. The targeted mouse were then mated with B6.C-Tg (CMV-cre)1Cgn mouse to delete exon 2 and exon 3 of gene Ildr1. The knockout efficiency was evaluated through the detection of exon 2 and exon 3 using RT-PCR. The resulted indicated that exon 2 and exon 3 were deleted from Ildr1−/− mice (Fig. 1C). Mice were genotyped by two PCR primers. The first primer was used to identify the wild-type allele (a single 365 bp band) and the second primer was used to identify the knockout allele (a single 514 bp band) (Fig. 1B).


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)

X-gal staining for beta-galactosidase activity in the organ of Corti in P2 (A), P5 (B), and P10 (C) Ildr1+/− mice.Arrowheads in A–C point to the three lines of outer hair cells and arrows in A–C point to the single line of inner hair cells.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

f02: X-gal staining for beta-galactosidase activity in the organ of Corti in P2 (A), P5 (B), and P10 (C) Ildr1+/− mice.Arrowheads in A–C point to the three lines of outer hair cells and arrows in A–C point to the single line of inner hair cells.
Mentions: Ildr1, the gene encoding ILDR1, is located on chromosome 16. It has eight exons and encodes the entire protein of 537 amino acids. A targeting vector was constructed and used to put a neomycin cassette and the LacZ reporter gene in the upstream region of exon 2 (Fig. 1A). The LacZ-Neo cassette serves as a positive selection marker in the ES targeting step and as the reporter for exploring the expression pattern of ILDR1 (Fig. 2). Exon 2 and exon 3 were floxed by loxp site. The targeted mouse were then mated with B6.C-Tg (CMV-cre)1Cgn mouse to delete exon 2 and exon 3 of gene Ildr1. The knockout efficiency was evaluated through the detection of exon 2 and exon 3 using RT-PCR. The resulted indicated that exon 2 and exon 3 were deleted from Ildr1−/− mice (Fig. 1C). Mice were genotyped by two PCR primers. The first primer was used to identify the wild-type allele (a single 365 bp band) and the second primer was used to identify the knockout allele (a single 514 bp band) (Fig. 1B).

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