The Role of Deubiquitinating Enzymes in Synaptic Function and Nervous System Diseases

Posttranslational modification of proteins by ubiquitin has emerged as a critical regulator of synapse development and function. Ubiquitination is a reversible modification mediated by the concerted action of a large number of specific ubiquitin ligases and ubiquitin proteases, called deubiquitinati...

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Main Authors: Jennifer R. Kowalski, Peter Juo
Format: Article
Language:English
Published: Wiley 2012-01-01
Series:Neural Plasticity
Online Access:http://dx.doi.org/10.1155/2012/892749
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author Jennifer R. Kowalski
Peter Juo
author_facet Jennifer R. Kowalski
Peter Juo
author_sort Jennifer R. Kowalski
collection DOAJ
description Posttranslational modification of proteins by ubiquitin has emerged as a critical regulator of synapse development and function. Ubiquitination is a reversible modification mediated by the concerted action of a large number of specific ubiquitin ligases and ubiquitin proteases, called deubiquitinating enzymes (DUBs). The balance of activity of these enzymes determines the localization, function, and stability of target proteins. While some DUBs counter the action of specific ubiquitin ligases by removing ubiquitin and editing ubiquitin chains, other DUBs function more generally to maintain the cellular pool of free ubiquitin monomers. The importance of DUB function at the synapse is underscored by the association of specific mutations in DUB genes with several neurological disorders. Over the last decade, although much research has led to the identification and characterization of many ubiquitin ligases at the synapse, our knowledge of the relevant DUBs that act at the synapse has lagged. This review is focused on highlighting our current understanding of DUBs that regulate synaptic function and the diseases that result from dysfunction of these DUBs.
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spelling doaj-art-d5e10e8780954f43ae074712617e4bbd2025-02-03T01:23:08ZengWileyNeural Plasticity2090-59041687-54432012-01-01201210.1155/2012/892749892749The Role of Deubiquitinating Enzymes in Synaptic Function and Nervous System DiseasesJennifer R. Kowalski0Peter Juo1Department of Biological Sciences, Butler University, 4600 Sunset Avenue, Indianapolis, IN 46208, USADepartment of Molecular Physiology and Pharmacology, Tufts University School of Medicine, 150 Harrison Avenue, Boston, MA 02111, USAPosttranslational modification of proteins by ubiquitin has emerged as a critical regulator of synapse development and function. Ubiquitination is a reversible modification mediated by the concerted action of a large number of specific ubiquitin ligases and ubiquitin proteases, called deubiquitinating enzymes (DUBs). The balance of activity of these enzymes determines the localization, function, and stability of target proteins. While some DUBs counter the action of specific ubiquitin ligases by removing ubiquitin and editing ubiquitin chains, other DUBs function more generally to maintain the cellular pool of free ubiquitin monomers. The importance of DUB function at the synapse is underscored by the association of specific mutations in DUB genes with several neurological disorders. Over the last decade, although much research has led to the identification and characterization of many ubiquitin ligases at the synapse, our knowledge of the relevant DUBs that act at the synapse has lagged. This review is focused on highlighting our current understanding of DUBs that regulate synaptic function and the diseases that result from dysfunction of these DUBs.http://dx.doi.org/10.1155/2012/892749
spellingShingle Jennifer R. Kowalski
Peter Juo
The Role of Deubiquitinating Enzymes in Synaptic Function and Nervous System Diseases
Neural Plasticity
title The Role of Deubiquitinating Enzymes in Synaptic Function and Nervous System Diseases
title_full The Role of Deubiquitinating Enzymes in Synaptic Function and Nervous System Diseases
title_fullStr The Role of Deubiquitinating Enzymes in Synaptic Function and Nervous System Diseases
title_full_unstemmed The Role of Deubiquitinating Enzymes in Synaptic Function and Nervous System Diseases
title_short The Role of Deubiquitinating Enzymes in Synaptic Function and Nervous System Diseases
title_sort role of deubiquitinating enzymes in synaptic function and nervous system diseases
url http://dx.doi.org/10.1155/2012/892749
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