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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

Auditory dysfunction in Ildr1−/− mouse.Offspring at P21 derived from Ildr1+/− × Ildr1−/− mating were subjected to auditory-evoked brainstem response (ABR) testing. (A,B) ABR response to tone bursts at a representative frequency of 8 kHz in P21 Ildr1+/− mouse (A) and P21 Ildr1−/− mouse (B). (C) The average click sound pressure level (SPL) threshold of Ildr1+/− and Ildr1−/− mice at P21. Ildr1+/− mice showed normal SPL thresholds at all frequencies measured, but Ildr1−/− mice showed no waveforms at intensities up to 100 dB SPL at P21 (n = 4 mice for each time point).
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f03: Auditory dysfunction in Ildr1−/− mouse.Offspring at P21 derived from Ildr1+/− × Ildr1−/− mating were subjected to auditory-evoked brainstem response (ABR) testing. (A,B) ABR response to tone bursts at a representative frequency of 8 kHz in P21 Ildr1+/− mouse (A) and P21 Ildr1−/− mouse (B). (C) The average click sound pressure level (SPL) threshold of Ildr1+/− and Ildr1−/− mice at P21. Ildr1+/− mice showed normal SPL thresholds at all frequencies measured, but Ildr1−/− mice showed no waveforms at intensities up to 100 dB SPL at P21 (n = 4 mice for each time point).

Mentions: To determine if Idlr1 knockout mice also exhibited a hearing impairment phenotype that mimicked the phenotype of DFNB42, we measured the ABR hearing thresholds in Ildr1+/− and Ildr1−/− mice at P21. Compared with the Ildr1+/− mice, Ildr1−/− mice at P21 did not respond to any tone bursts (≥100 dB) demonstrating profound hearing impairment at all frequencies (Fig. 3A–C).


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)

Auditory dysfunction in Ildr1−/− mouse.Offspring at P21 derived from Ildr1+/− × Ildr1−/− mating were subjected to auditory-evoked brainstem response (ABR) testing. (A,B) ABR response to tone bursts at a representative frequency of 8 kHz in P21 Ildr1+/− mouse (A) and P21 Ildr1−/− mouse (B). (C) The average click sound pressure level (SPL) threshold of Ildr1+/− and Ildr1−/− mice at P21. Ildr1+/− mice showed normal SPL thresholds at all frequencies measured, but Ildr1−/− mice showed no waveforms at intensities up to 100 dB SPL at P21 (n = 4 mice for each time point).
© Copyright Policy - open-access
Related In: Results  -  Collection

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

f03: Auditory dysfunction in Ildr1−/− mouse.Offspring at P21 derived from Ildr1+/− × Ildr1−/− mating were subjected to auditory-evoked brainstem response (ABR) testing. (A,B) ABR response to tone bursts at a representative frequency of 8 kHz in P21 Ildr1+/− mouse (A) and P21 Ildr1−/− mouse (B). (C) The average click sound pressure level (SPL) threshold of Ildr1+/− and Ildr1−/− mice at P21. Ildr1+/− mice showed normal SPL thresholds at all frequencies measured, but Ildr1−/− mice showed no waveforms at intensities up to 100 dB SPL at P21 (n = 4 mice for each time point).
Mentions: To determine if Idlr1 knockout mice also exhibited a hearing impairment phenotype that mimicked the phenotype of DFNB42, we measured the ABR hearing thresholds in Ildr1+/− and Ildr1−/− mice at P21. Compared with the Ildr1+/− mice, Ildr1−/− mice at P21 did not respond to any tone bursts (≥100 dB) demonstrating profound hearing impairment at all frequencies (Fig. 3A–C).

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