Liquid-liquid phase separation in hair cell stereocilia development and maintenance

Comput Struct Biotechnol J. 2023 Feb 24:21:1738-1745. doi: 10.1016/j.csbj.2023.02.040. eCollection 2023.

Abstract

As an emerging concept, liquid-liquid phase separation (LLPS) in biological systems has shed light on the formation mechanisms of membrane-less compartments in cells. The process is driven by multivalent interactions of biomolecules such as proteins and/or nucleic acids, allowing them to form condensed structures. In the inner ear hair cells, LLPS-based biomolecular condensate assembly plays a vital role in the development and maintenance of stereocilia, the mechanosensing organelles located at the apical surface of hair cells. This review aims to summarize recent findings on the molecular basis governing the LLPS of Usher syndrome-related gene-encoding proteins and their binding partners, which may ultimately result in the formation of upper tip-link density and tip complex density in hair cell stereocilia, offering a better understanding of this severe inherited disease that causes deaf-blindness.

Keywords: Liquid-liquid phase separation/Usher Syndrome/Stereocilia.

Publication types

  • Review