USP53

Deubiquitinase that mediates 'Lys-63'-linked deubiquitination of tight junction proteins, such as MARVELD2 and LSR, and which is involved in the survival of auditory hair cells and hearing. Specifically cleaves 'Lys-63'-linked polyubiquitin chains composed of at least 3 ubiquitin molecules, while it is not able to deubiquitinate substrates with shorter ubiquitin chains: recognizes ubiquitin chain in position S2 and catalyzes en bloc cleavage of polyubiquitin chains from substrate proteins. Probably acts by modulating the barrier properties and mechanical stability of tight junctions via deubiquitination of MARVELD2 and LSR..

Function from UniProtKB (Q70EK8), read 2026-09-23. CC BY 4.0.