Article
Mice lacking ganglioside GM3 synthase exhibit complete hearing loss due to selective degeneration of the organ of Corti.
Proceedings of the National Academy of Sciences of the United States of America - 9 Jun 2009
Yoshikawa Misato, Go Shinji, Takasaki Kotaro, Kakazu Yasuhiro, Ohashi Mitsuru, Nagafuku Masakazu, Kabayama Kazuya, Sekimoto Junji, Suzuki Shun-ichi, Takaiwa Kazutaka, Kimitsuki Takashi, Matsumoto Nozomu, Komune Shizuo, Kamei Daisuke, Saito Masaki, Fujiwara Michihiro, Iwasaki Katsunori, Inokuchi Jin-ichi
Abstract excerpt
The ganglioside GM3 synthase (SAT-I), encoded by a single-copy gene, is a primary glycosyltransferase for the synthesis of complex gangliosides. In SAT-I null mice, hearing ability, assessed by brainstem auditory-evoked potentials (BAEP), was impaired at the onset of hearing and had been completely lost by 17 days after birth (P17), showing a deformity in hair cells in the organ of Corti. By 2 months of age, the...
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