SUMO-modifying Huntington's disease

Small ubiquitin-like modifiers, SUMOs, are proteins that are conjugated to target substrates and regulate their functions in a post-translational modification called SUMOylation. In addition to its physiological roles, SUMOylation has been implicated in several neurodegenerative diseases, such as Alzheimer's, Parkinson's, and Huntington's diseases (HD). HD is a neurodegenerative monogenetic autosomal dominant disorder caused by a mutation in the CAG repeat of the huntingtin (htt) gene, which expresses a mutant Htt protein more susceptible to aggregation and toxicity. Besides Htt, other SUMO ligases, enzymes, mitochondrial and autophagic components are also important for the progression of the disease. Here we review the main aspects of Htt SUMOylation and its role in cellular processes involved in the pathogenesis of HD.

Automated summary

SUMOs are proteins that regulate the functions of target substrates through a post-translational modification called SUMOylation. In addition to their physiological roles, SUMOylation has been implicated in neurodegenerative diseases such as Alzheimer's, Parkinson's, and Huntington's diseases. This review focuses on the role of Htt SUMOylation in cellular processes involved in the pathogenesis of HD.