Open AccessRole of Ubiquilins in Neurodegenerative Diseases
Author(s) -
Hongmin Wang
Publication year - 2018
Publication title -
annals of advanced biomedical sciences
Language(s) - English
Resource type - Journals
ISSN - 2641-9459
DOI - 10.23880/aabsc-16000101
Subject(s) - proteasome , ubiquitin , autophagy , protein degradation , protein aggregation , microbiology and biotechnology , neurodegeneration , biology , chemistry , computational biology , biochemistry , gene , medicine , disease , apoptosis , pathology
Ubiquilin (Ubqln) proteins, including Ubqln1-4, are a family of ubiquitin-like (UBL) proteins that structurally contain anN-terminalubiquitin-like domain, a C-terminalubiquitin-associated (UBA) domain, and aninternal variable number of Stil repeat motifs. Unlike those small molecule ubiquitin-like proteins, Ubqln proteins cannot be covalently tagged to the proteins that are targeted to the proteasome for degradation. Instead, this family of proteins binds to thepolyubiquitinated proteins via the UBA domain and delivers them to the proteasome for degradation, despite their involving in autophagy-mediated protein degradation. Increasing studies have suggested that Ubqln proteins play important roles in mediating the degradation of damaged/misfolded proteins and thus theirinsufficiencies or dysfunctionsare frequently associated with different neurodegenerative disorders. This review summarizes the role of Ubqln proteins in several neurodegenerative disorders.