Ataxin-3-like protein (ATXN3L)

Ataxin-3-like protein (ATXN3L) is a deubiquitinating enzyme that belongs to the Josephin DUB sub-family, sharing high sequence identity with Ataxin-3 (ATXN3). ATXN3L contains a conserved Josephin domain, responsible for its cysteine protease activity and is the most active DUB among Josephin family members. Its functional role has been implicated in the regulation of cell proliferation through stabilization of KLF5, a transcription factor involved in cancer biology, by decreasing ubiquitin-mediated degradation. Despite these findings, the physiological and pathological roles of ATXN3L remain insufficiently characterized and there are no currently validated drugs that target it directly. It is distinct from Ataxin-3, which is associated with neurodegenerative disease (Machado–Joseph disease), and there is no evidence linking ATXN3L to neurological diseases at present. Further research and the development of robust detection reagents are needed to fully understand its clinical importance and therapeutic potential.

Other names

Ataxin-3-like proteinATXN3LATX3LMachado-Joseph disease protein 1-likeMJDLputative ataxin-3-like proteinmachado-Joseph disease protein 1-like

Deubiquitination of substrate proteins, especially stabilization of KLF5 by reversing ubiquitin-mediated degradation