Structures of usher syndrome 1 proteins and their complexes.
Usher syndrome 1 (USH1) is the most common and severe form of hereditary loss of hearing and vision. Genetic, physiological, and cell biological studies, together with recent structural investigations, have not only uncovered the physiological functions of the five USH1 proteins but also provided mechanistic explanations for the hearing and visual deficiencies in humans caused by USH1 mutations. This review focuses on the structural basis of the USH1 protein complex organization.
Division of Life Science, State Key Laboratory of Molecular Neuroscience, Hong Kong University of Science and Technology, Clear Water Bay, Kowloon, Hong Kong.
Lifeng
L
Pan
Mingjie
M
Zhang
D000818:Animals; D006309:Hearing / Q000502:physiology; D006801:Humans; D009419:Nerve Tissue Proteins / Q000235:genetics* / Q000378:metabolism*; D052245:Usher Syndromes / Q000235:genetics* / Q000378:metabolism*; D014785:Vision, Ocular / Q000502:physiology
D009419:Nerve Tissue Proteins