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Pharmacological protein targets in polyglutamine diseases: Mutant polypeptides and their interactors
Author(s) -
Margulis Boris A.,
Vigont Vladimir,
Lazarev Vladimir F.,
Kaznacheyeva Elena V.,
Guzhova Irina V.
Publication year - 2013
Publication title -
febs letters
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.593
H-Index - 257
eISSN - 1873-3468
pISSN - 0014-5793
DOI - 10.1016/j.febslet.2013.05.022
Subject(s) - huntingtin , mutant , aggresome , biology , microbiology and biotechnology , androgen receptor , huntingtin protein , gene , biochemistry , genetics , ubiquitin , cancer , prostate cancer
Polyglutamine diseases are a group of pathologies affecting different parts of the brain and causing dysfunction and atrophy of certain neural cell populations. These diseases stem from mutations in various cellular genes that result in the synthesis of proteins with extended polyglutamine tracts. In particular, this concerns huntingtin, ataxins, and androgen receptor. These mutant proteins can form oligomers, aggregates, and, finally, aggresomes with distinct functions and different degrees of cytotoxicity. In this review, we analyze the effects of different forms of polyQ proteins on other proteins and their functions, which are considered as targets for therapeutic intervention.