Evidence map›Paper›PMID 22819776›Full record

ArticleNeurobiology of disease2013

Mutant SOD1G93A triggers mitochondrial fragmentation in spinal cord motor neurons: neuroprotection by SIRT3 and PGC-1α.

Wenjun Song, Yuting Song, Brad Kincaid, Blaise Bossy, Ella Bossy-Wetzel

Registry-linked trialOpen access · greenAbstract read
In one paragraph

Article in Neurobiology of disease, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT04654689 (Impact of the Combined Treatment of Curcumin and Resveratrol Liposomed Polyphenols With G04CB02 on the Clinical Improvement of ALS Patients), which is not on this map. Cited by 128 papers.

0numbers the graph read from it
0cells of the map it votes in
128citing papers in PubMed
10.5field-weighted citation impact, top 1% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

NCT04654689 phase2completednot on this mapstarted 2021, after this paper: background citation

Impact of the Combined Treatment of Curcumin and Resveratrol Liposomed Polyphenols With G04CB02 on the Clinical Improvement of ALS Patients

TypeinterventionalSponsorFundación Universidad Católica de Valencia San Vicente MártirRan2021 to 2023Enrolled90ConditionsAmyotrophic Lateral SclerosisArmsLiposomed polyphenols resveratrol and curcumin, Placebo for liposomed resveratrol and curcumin, Isocaloric Diet, G04CB02, Placebo microcrystalline methylcellulose
3 · Its place in the literature

Who cites it

128 citing papers in PubMed, 203 citations in OpenAlex.

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  17. The role of SIRT3 in homeostasis and cellular health.Frontiers in cellular neuroscience · 2024
    Review
  18. Article
  19. Article
  20. Review

68 more citing papers are in PubMed but not listed here.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

5 authors at 1 institution in 1 country.

Wenjun SongBurnett School of Biomedical Sciences, College of Medicine, University of Central Florida, Orlando, FL 32816, USA.
Yuting Song
Brad Kincaid
Blaise Bossy
Ella Bossy-Wetzel
University of Central Florida · US

Funding

Mitochondrial Fission in Huntington's DiseaseR01NS055193 · NINDS · UNIVERSITY OF CENTRAL FLORIDA · PI BOSSY-WETZEL, ELLA R · 2008 to 2017
$2.8M
OPA1 Mutation and Retinal Ganglion Cell DegenerationR01EY016164 · NEI · UNIVERSITY OF CENTRAL FLORIDA · PI BOSSY-WETZEL, ELLA R · 2005 to 2009
$1.8M
NEI NIH HHS R01 EY016164NEI NIH HHS R01EY016164NINDS NIH HHS R01 NS055193NINDS NIH HHS R01NS055193
6 · The paper itself

Abstract

Mutations in the Cu/Zn Superoxide Dismutase (SOD1) gene cause an inherited form of ALS with upper and lower motor neuron loss. The mechanism underlying mutant SOD1-mediated motor neuron degeneration remains unclear. While defects in mitochondrial dynamics contribute to neurodegeneration, including ALS, previous reports remain conflicted. Here, we report an improved technique to isolate, transfect, and culture rat spinal cord motor neurons. Using this improved system, we demonstrate that mutant SOD1(G93A) triggers a significant decrease in mitochondrial length and an accumulation of round fragmented mitochondria. The increase of fragmented mitochondria coincides with an arrest in both anterograde and retrograde axonal transport and increased cell death. In addition, mutant SOD1(G93A) induces a reduction in neurite length and branching that is accompanied with an abnormal accumulation of round mitochondria in growth cones. Furthermore, restoration of the mitochondrial fission and fusion balance by dominant-negative dynamin-related protein 1 (DRP1) expression rescues the mutant SOD1(G93A)-induced defects in mitochondrial morphology, dynamics, and cell viability. Interestingly, both SIRT3 and PGC-1α protect against mitochondrial fragmentation and neuronal cell death by mutant SOD1(G93A). This data suggests that impairment in mitochondrial dynamics participates in ALS and restoring this defect might provide protection against mutant SOD1(G93A)-induced neuronal injury.

Indexed as

Amyotrophic Lateral SclerosisAnimalsAxonal TransportDisease Models, AnimalDynaminsImmunohistochemistryMiceMice, TransgenicMitochondriaMitochondrial DynamicsMotor NeuronsMutationPeroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alphaRatsRats, Sprague-DawleyRNA-Binding ProteinsDnm1l protein, ratDynaminsPeroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alphaPpargc1a protein, ratRNA-Binding ProteinsSirtuin 3Sod1 protein, mouseSod1 protein, ratSuperoxide DismutaseSuperoxide Dismutase-1Transcription Factors

Identifiers

PMID22819776
PMCPMC3992938
OpenAlexW2045337251

What OpenQuestion holds

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Registered trials

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.