UBA1: At the Crossroads of Ubiquitin Homeostasis and Neurodegeneration

Ewout J N Groen; Thomas H Gillingwater

doi:10.1016/j.molmed.2015.08.003

UBA1: At the Crossroads of Ubiquitin Homeostasis and Neurodegeneration

Research output: Contribution to journal › Literature review › peer-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 language	English
Pages (from-to)	622-32
Number of pages	11
Journal	Trends in Molecular Medicine
Volume	21
Issue number	10
DOIs	https://doi.org/10.1016/j.molmed.2015.08.003
Publication status	Published - Oct 2015

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10.1016/j.molmed.2015.08.003

UBA1 At the Crossroads of Ubiquitin Homeostasis and Neurodegeneration
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1 Finished

Understanding the role of altered ubiquitin homeostasis in motor neuron diseases
Gillingwater, T. & Groen, E.
1/03/15 → 31/08/19
Project: Research

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title = "UBA1: At the Crossroads of Ubiquitin Homeostasis and Neurodegeneration",

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.",

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year = "2015",

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AU - Groen, Ewout J N

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N2 - 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.

AB - 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.

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DO - 10.1016/j.molmed.2015.08.003

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JO - Trends in Molecular Medicine

JF - Trends in Molecular Medicine

SN - 1471-4914

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UBA1: At the Crossroads of Ubiquitin Homeostasis and Neurodegeneration

Abstract

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Understanding the role of altered ubiquitin homeostasis in motor neuron diseases

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