UBA1: At the Crossroads of Ubiquitin Homeostasis and Neurodegeneration

Ewout J N Groen, Thomas H Gillingwater

Research output: Contribution to journalLiterature reviewpeer-review

Abstract

Neurodegenerative diseases are a leading cause of disability and early death. A common feature of these conditions is disruption of protein homeostasis. Ubiquitin-like modifier activating enzyme 1 (UBA1), the E1 ubiquitin-activating enzyme, sits at the apex of the ubiquitin cascade and represents an important regulator of cellular protein homeostasis. Critical contributions of UBA1-dependent pathways to the regulation of homeostasis and degeneration in the nervous system are emerging, including specific disruption of UBA1 in spinal muscular atrophy (SMA) and Huntington's disease (HD). In this review we discuss recent findings that put UBA1 at the centre of cellular homeostasis and neurodegeneration, highlighting the potential for UBA1 to act as a promising therapeutic target for a range of neurodegenerative diseases.

Original languageEnglish
Pages (from-to)622-32
Number of pages11
JournalTrends in Molecular Medicine
Volume21
Issue number10
DOIs
Publication statusPublished - Oct 2015

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