Structures of Usher Syndrome 1 Proteins and Their Complexes

Lifeng Pan, Mingjie Zhang

Abstract

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.

Footnotes

  • This work in the author's laboratory was supported by grants from the Research Grants Council of Hong Kong to M. Zhang. (HKUST663808, 664009, 660709, 663610, HKUST6/CRF/10, SEG_HKUST06, and AoE/B-15/01-II). We apologize to the authors whose work cannot be cited here due to the space limitation.

  • No conflicts of interest, financial or otherwise, are declared by the author(s).

  • Author contributions: L.P. prepared figures; L.P. and M.Z. drafted the manuscript; L.P. and M.Z. edited and revised the manuscript; L.P. and M.Z. approved the final version of the manuscript.

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