Sung Hwa Hong and Hosuk Chu contributed equally to the work.
Article first published online: 27 JUL 2012
Copyright © 2012 Wiley Periodicals, Inc.
American Journal of Medical Genetics Part A
Volume 158A, Issue 9, pages 2131–2138, September 2012
How to Cite
Hong, S. H., Chu, H., Kim, K. R., Ko, M. H., Kwon, S. Y., Moon, I. J., Chung, W.-H., Cho, Y.-S., Kim, C.-H., Suh, M.-W., Choi, E. W., Sohn, Y. B., Park, S. W., Kim, S.-H., Cho, S.-Y., Ko, A.-R. and Jin, D.-K. (2012), Auditory characteristics and therapeutic effects of enzyme replacement in mouse model of the mucopolysaccharidosis (MPS) II. Am. J. Med. Genet., 158A: 2131–2138. doi: 10.1002/ajmg.a.35498
Conflicts of interest: None.
How to Cite this Article: Hong SH, Chu H, Kim KR, Ko MH, Kwon SY, Moon IJ, Chung W-H, Cho Y-S, Kim C-H, Suh M-W, Choi EW, Sohn YB, Park SW, Kim S-H, Cho S-Y, Ko A-R, Jin D-K. 2012. Auditory characteristics and therapeutic effects of enzyme replacement in mouse model of the Mucopolysaccharidosis (MPS) II. Am J Med Genet Part A. 158A:2131–2138.
- Issue published online: 24 AUG 2012
- Article first published online: 27 JUL 2012
- Manuscript Accepted: 6 MAY 2012
- Manuscript Received: 31 DEC 2010
- Ministry for Health, Welfare and Family Affairs, Republic of Korea. Grant Number: A080588
- Samsung Biomedical Research Institute. Grant Number: C-A9-240-2
- In-Sung Foundation for Medical Research
- MPS II;
- Hunter syndrome;
- enzyme replacement;
- mouse model
Mucopolysaccharidosis (MPS) II is an X-linked metabolic disorder caused by dysfunction of iduronate-2-sulfatase (I2S). This abnormality causes the progressive accumulation of incompletely degraded glycosaminoglycans (GAGs) in the lysosomes. The auditory characteristics of MPS II in mouse models have not been reported. In this study, we evaluated the auditory characteristics of the MPS II in IDS knock-out (IDS-KO) mice. In addition, the effect of enzyme replacement therapy (ERT) on hearing was studied. The IDS-KO mice had normal histology of the cochlea and retained good hearing at 7 weeks of age. However, at 17 weeks of age, the hearing thresholds of the IDS-KO mice were elevated and exudates were found in the middle ear. The hearing thresholds of the enzyme-treated IDS-KO (IDS-ERT) mice were similar to the wild-type (WT) mice at 17 weeks. Moreover, the microstructure of the inner ear was similar to the IDS-KO by transmission electron microscopy. The histology findings indicated that the microstructure of the inner ear was similar in comparisons between IDS-KO and IDS-ERT mice, even after 10 weeks of treatment. However, the hearing deficits in the MPS II mouse model can be prevented if ERT is started before the onset of hearing impairment. © 2012 Wiley Periodicals, Inc.