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Original Paper

Inhibition of Pathogenic Mutant SOD1 Aggregation in Cultured Motor Neuronal Cells by Prevention of Its SUMOylation on Lysine 75

Dangoumau A.a · Marouillat S.a · Burlaud Gaillard J.b · Uzbekov R.b · Veyrat-Durebex C.a · Blasco H.a, d · Arnoult C.c · Corcia P.a, e · Andres C.R.a, d · Vourc'h P.a,b,d

Author affiliations

aUMR INSERM U930, bPST Analyse des Systèmes Biologiques and cCNRS UMR 7292, Université François-Rabelais, dCHRU de Tours, Laboratoire de Biochimie et de Biologie Moléculaire, and eCentre SLA, CHRU de Tours, Hôpital Bretonneau, Tours, France

Related Articles for ""

Neurodegener Dis 2016;16:161-171

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Article / Publication Details

First-Page Preview
Abstract of Original Paper

Received: April 10, 2015
Accepted: August 07, 2015
Published online: November 26, 2015
Issue release date: April 2016

Number of Print Pages: 11
Number of Figures: 5
Number of Tables: 0

ISSN: 1660-2854 (Print)
eISSN: 1660-2862 (Online)

For additional information: http://www.karger.com/NDD

Abstract

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by the selective death of motor neurons. Mutations in the SOD1 gene encoding the superoxide dismutase 1 are present in 15% of familial ALS cases and in 2% of sporadic cases. These mutations are associated with the formation of SOD1-positive aggregates. The mechanisms of aggregation remain unknown, but posttranslational modifications of SOD1 may be involved. Here, we report that NSC-34 motor neuronal cells expressing mutant SOD1 contained aggregates positive for small ubiquitin modifier-1 (SUMO-1), and in parallel a reduced level of free SUMO-1. CLEM (correlative light and electron microscopy) analysis showed nonorganized cytosolic aggregates for all mutations tested (SOD1A4V, SOD1V31A, and SOD1G93C). We next show that preventing the SUMOylation of mutant SOD1 by the substitution of lysine 75, the SUMOylation site of SOD1, significantly reduces the number of motor neuronal cells with aggregates. These results support the need for further research on the SUMOylation pathways, which may be a potential therapeutic target in ALS.

© 2015 S. Karger AG, Basel


Article / Publication Details

First-Page Preview
Abstract of Original Paper

Received: April 10, 2015
Accepted: August 07, 2015
Published online: November 26, 2015
Issue release date: April 2016

Number of Print Pages: 11
Number of Figures: 5
Number of Tables: 0

ISSN: 1660-2854 (Print)
eISSN: 1660-2862 (Online)

For additional information: http://www.karger.com/NDD


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