期刊
TRENDS IN MOLECULAR MEDICINE
卷 21, 期 10, 页码 622-632出版社
ELSEVIER SCI LTD
DOI: 10.1016/j.molmed.2015.08.003
关键词
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资金
- Wellcome Trust [106098/Z/14/Z]
- SMA Trust
- AxonomiX network
- Euan MacDonald Centre for Motor Neurone Disease Research
- Muscular Dystrophy Campaign
- Wellcome Trust [106098/Z/14/Z] Funding Source: Wellcome Trust
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 El 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.
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