Evidence map›Paper›PMID 41742309›Full record

ArticleActa neuropathologica communications2026

Fast and slow strains of misfolded mutant superoxide dismutase 1 in familial amyotrophic lateral sclerosis.

Guilian Xu, Kristy Dillon, Amanda Lopez, Selma Brkic, Susan Fromholt, Lyle W Ostrow, Jonathan D Glass, Paramita Chakrabarty, Jacob I Ayers, David R Borchelt

Abstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Acta neuropathologica communications, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
–field-weighted citation impact
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.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

  1. Article
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

10 authors.

Guilian XuDepartment of Neuroscience, Center for Translational Research in Neurodegenerative Disease, University of Florida, Box 100159, Gainesville, FL, 32610, USA.
Kristy DillonDepartment of Neuroscience, Center for Translational Research in Neurodegenerative Disease, University of Florida, Box 100159, Gainesville, FL, 32610, USA.
Amanda LopezDepartment of Neuroscience, Center for Translational Research in Neurodegenerative Disease, University of Florida, Box 100159, Gainesville, FL, 32610, USA.
Selma BrkicDepartment of Neuroscience, Center for Translational Research in Neurodegenerative Disease, University of Florida, Box 100159, Gainesville, FL, 32610, USA.
Susan FromholtDepartment of Neuroscience, Center for Translational Research in Neurodegenerative Disease, University of Florida, Box 100159, Gainesville, FL, 32610, USA.
Lyle W OstrowDepartments of Neurology and Neural Sciences, Lewis Katz School of Medicine at Temple University, Philadelphia, PA, 19140, USA.
Jonathan D GlassDepartment of Neurology, Emory University School of Medicine, Atlanta, GA, 30322, USA.
Paramita ChakrabartyDepartment of Neuroscience, Center for Translational Research in Neurodegenerative Disease, University of Florida, Box 100159, Gainesville, FL, 32610, USA.
Jacob I AyersDepartment of Neuroscience, Center for Translational Research in Neurodegenerative Disease, University of Florida, Box 100159, Gainesville, FL, 32610, USA. Jacob.Ayers@ucsf.edu.
David R BorcheltDepartment of Neuroscience, Center for Translational Research in Neurodegenerative Disease, University of Florida, Box 100159, Gainesville, FL, 32610, USA. drb1@ufl.edu.

Funding

Modeling the progression of SOD1-linked motor neuron diseaseR01NS092788 · NINDS · UNIVERSITY OF FLORIDA · PI PARAMITA CHAKRABARTY · 2015 to 2026
$3.6M
NIH HHS R01NS0927881NINDS NIH HHS R01 NS092788
6 · The paper itself

Abstract

Mutations in superoxide dismutase 1 (SOD1) account for ~ 10% of familial amyotrophic lateral sclerosis (fALS) cases. Most SOD1 ALS cases show a 2–5 year clinical course, but a subset of patients exhibit a slowly progressing illness lasting 10–20 years. Substantial evidence indicates that disease-causing mutations in SOD1 promote misfolding and aggregation. Spinal tissue homogenates from paralyzed transgenic mice containing misfolded mutant SOD1 accelerate paralysis when injected into the spine or sciatic nerve of young mutant SOD1 transgenic mice. Using this prion-like seeding model in G85R-SOD1:YFP transgenic mice to initiate the disease process, we show that human SOD1 variants associated with rapidly progressing ALS produce SOD1-ALS strains that cause paralysis earlier than mutations associated with slowly progressing disease. Our findings suggest that the heterogeneous clinical presentations of different SOD1 mutations in ALS could be linked to prion-like strain attributes that govern the templating and propagation kinetics of misfolded SOD1.

Indexed as

Amyotrophic Lateral SclerosisSuperoxide Dismutase-1AnimalsDisease Models, AnimalDisease ProgressionHumansMiceMice, TransgenicMutationProtein FoldingSpinal CordSOD1 protein, humanSod1 protein, mouseSuperoxide Dismutase-1Amyotrophic lateral sclerosisPrionpropagationStrainsSuperoxide dismutase-1

Identifiers

PMID41742309
PMCPMC13041472

What OpenQuestion holds

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LicenceCC BY-NC-ND
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Registered trials

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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.