NDLI: Auditory characteristics and therapeutic effects of enzyme replacement in mouse model of the mucopolysaccharidosis (MPS) II.

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Auditory characteristics and therapeutic effects of enzyme replacement in mouse model of the mucopolysaccharidosis (MPS) II.

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Auditory characteristics and therapeutic effects of enzyme replacement in mouse model of the mucopolysaccharidosis (MPS) II.

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Hong, Sung Hwa Chu, Hosuk Kim, Ki Ryung Ko, Moon Hee Kwon, See Youn Moon, Il Joon Chung, Won-Ho Cho, Yang-Sun Kim, Chi-Hwa Suh, Myung-Whan Choi, Eun Wha Sohn, Young Bae Park, Sung Won Kim, Se-Hwa Cho, Sung-Yoon Ko, Ah-Ra Jin, Dong-Kyu
Description	Country affiliation: South Korea Author Affiliation: Hong SH ( Department of Otorhinolaryngology-Head and Neck Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, and Hana ENT Hospital, Seoul, South Korea.)
Abstract	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.
File Format	HTM / HTML
ISSN	15524825
Issue Number	9
Volume Number	158A
e-ISSN	15524833
Journal	American Journal of Medical Genetics Part A
Language	English
Publisher	Wiley-Blackwell
Publisher Date	2012-09-01
Publisher Place	United States
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	Discipline Human Discipline Genetics Auditory Pathways Disease Models, Animal Iduronate Sulfatase Therapeutic Use Mucopolysaccharidosis Ii Drug Therapy Animals Brain Stem Physiopathology Ear, Middle Metabolism Glycosaminoglycans Mice Mice, Knockout Tomography, X-ray Computed Journal Article Research Support, Non-u.s. Gov't
Content Type	Text
Resource Type	Article
Subject	Genetics Genetics (clinical)

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