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Elongator subunit 3...
Elongator subunit 3 (ELP3) modifies ALS through tRNA modification.
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Bento-Abreu, Andre (author)
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- Jager, Gunilla (author)
- Umeå universitet,Institutionen för molekylärbiologi (Teknisk-naturvetenskaplig fakultet)
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Swinnen, Bart (author)
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Rué, Laura (author)
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Hendrickx, Stijn (author)
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Jones, Ashley (author)
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Staats, Kim A. (author)
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Taes, Ines (author)
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Eykens, Caroline (author)
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Nonneman, Annelies (author)
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Nuyts, Rik (author)
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Timmers, Mieke (author)
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Silva, Lara (author)
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Chariot, Alain (author)
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Nguyen, Laurent (author)
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Ravits, John (author)
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Lemmens, Robin (author)
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Cabooter, Deirdre (author)
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Van Den Bosch, Ludo (author)
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Van Damme, Philip (author)
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Al-Chalabi, Ammar (author)
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- Bystrom, Anders (author)
- Umeå universitet,Institutionen för molekylärbiologi (Teknisk-naturvetenskaplig fakultet)
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Robberecht, Wim (author)
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(creator_code:org_t)
- 2018-02-03
- 2018
- English.
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In: Human Molecular Genetics. - : Oxford University Press. - 0964-6906 .- 1460-2083. ; 27:7, s. 1276-1289
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Abstract
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- Amyotrophic lateral sclerosis (ALS) is a fatal degenerative motor neuron disorder of which the progression is influenced by several disease-modifying factors. Here, we investigated ELP3, a subunit of the elongator complex that modifies tRNA wobble uridines, as one of such ALS disease modifiers. ELP3 attenuated the axonopathy of a mutant SOD1, as well as of a mutant C9orf72 ALS zebrafish model. Furthermore, the expression of ELP3 in the SOD1G93A mouse extended the survival and attenuated the denervation in this model. Depletion of ELP3 in vitro reduced the modified tRNA wobble uridine mcm5s2U and increased abundance of insoluble mutant SOD1, which was reverted by exogenous ELP3 expression. Interestingly, the expression of ELP3 in the motor cortex of ALS patients was reduced and correlated with mcm5s2U levels. Our results demonstrate that ELP3 is a modifier of ALS and suggest a link between tRNA modification and neurodegeneration.
Subject headings
- NATURVETENSKAP -- Biologi -- Cellbiologi (hsv//swe)
- NATURAL SCIENCES -- Biological Sciences -- Cell Biology (hsv//eng)
- MEDICIN OCH HÄLSOVETENSKAP -- Medicinska och farmaceutiska grundvetenskaper -- Neurovetenskaper (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Basic Medicine -- Neurosciences (hsv//eng)
Publication and Content Type
- ref (subject category)
- art (subject category)
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- By the author/editor
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Bento-Abreu, And ...
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Jager, Gunilla
-
Swinnen, Bart
-
Rué, Laura
-
Hendrickx, Stijn
-
Jones, Ashley
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show more...
-
Staats, Kim A.
-
Taes, Ines
-
Eykens, Caroline
-
Nonneman, Anneli ...
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Nuyts, Rik
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Timmers, Mieke
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Silva, Lara
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Chariot, Alain
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Nguyen, Laurent
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Ravits, John
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Lemmens, Robin
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Cabooter, Deirdr ...
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Van Den Bosch, L ...
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Van Damme, Phili ...
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Al-Chalabi, Amma ...
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Bystrom, Anders
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Robberecht, Wim
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show less...
- About the subject
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- NATURAL SCIENCES
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NATURAL SCIENCES
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and Biological Scien ...
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and Cell Biology
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Basic Medicine
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and Neurosciences
- Articles in the publication
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Human Molecular ...
- By the university
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Umeå University