Evidence map›Paper›PMID 37651231›Full record

ArticleCell reports2023

iPSC motor neurons, but not other derived cell types, capture gene expression changes in postmortem sporadic ALS motor neurons.

Aaron Held, Michelle Adler, Christine Marques, Charles Jourdan Reyes, Amey S Kavuturu, Ana R A A Quadros, I Sandra Ndayambaje, Erika Lara, Michael Ward, Clotilde Lagier-Tourenne and 1 more

Open access · goldAbstract read
In one paragraph

Article in Cell reports, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
21citing papers in PubMed, 1 pooled it
5.3field-weighted citation impact, top 4% 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.

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

21 citing papers in PubMed, 1 synthesis or guideline pooled it, 26 citations in OpenAlex.

  1. Pooled it
  2. Article
  3. Dysregulation of mitochondrial bioenergetics, RNA processing, and cellular stress responses in FUS-ALS: a multi-dataset transcriptomic perspective.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2026
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  15. Early disruption of the CREB pathway drives dendritic morphological alterations in FTD/ALS cortical neurons.Proceedings of the National Academy of Sciences of the United States of America · 2024
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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

11 authors at 4 institutions in 1 country.

Aaron HeldDepartment of Neurology, Sean M. Healey & AMG Center for ALS, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA.
Michelle AdlerDepartment of Neurology, Sean M. Healey & AMG Center for ALS, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA.
Christine MarquesDepartment of Neurology, Sean M. Healey & AMG Center for ALS, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA.
Charles Jourdan ReyesDepartment of Neurology, Sean M. Healey & AMG Center for ALS, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA; The Collaborative Center for X-Linked Dystonia-Parkinsonism, Department of Neurology, Massachusetts General Hospital, Charlestown, MA 02129, USA.
Amey S KavuturuDepartment of Neurology, Sean M. Healey & AMG Center for ALS, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA.
Ana R A A QuadrosDepartment of Neurology, Sean M. Healey & AMG Center for ALS, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA.
I Sandra NdayambajeDepartment of Neurology, Sean M. Healey & AMG Center for ALS, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA.
Erika LaraiPSC Neurodegenerative Research Initiative, Center for Alzheimer's and Related Dementias, National Institute on Aging, NIH, Bethesda, MD 20892, USA.
Michael WardNational Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD 20892, USA.
Clotilde Lagier-TourenneDepartment of Neurology, Sean M. Healey & AMG Center for ALS, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA; Broad Institute of Harvard University and MIT, Cambridge MA 02142, USA.
Brian J WaingerDepartment of Neurology, Sean M. Healey & AMG Center for ALS, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA; Broad Institute of Harvard University and MIT, Cambridge MA 02142, USA; Department of Anesthesiology, Critical Care and Pain Medicine, Massachusetts General Hospital, Boston MA 02114, USA; Harvard Stem Cell Institute, Cambridge MA 02138, USA. Electronic address: brian.wainger@mgh.harvard.edu.
Harvard University · USMassachusetts General Hospital · USNational Institute of Neurological Disorders and Stroke · USNational Institute on Aging · US

Funding

Determining stathmin-2 function and potential as a therapeutic target in ALS/FTDR01NS112503 · NINDS · UNIVERSITY OF CALIFORNIA, SAN DIEGO · PI CLEVELAND, DON W, LAGIER-TOURENNE, CLOTILDE · 2020 to 2024
$4.1M
A Human Stem Cell-Derived Neuromuscular Junction Model for Amyotrophic Lateral SclerosisDP2NS106664 · NINDS · MASSACHUSETTS GENERAL HOSPITAL · PI WAINGER, BRIAN JASON · 2017 to 2017
$2.6M
In vivo modelling and therapy development for stathmin-2 loss in TDP-43 proteinopathiesRF1NS124203 · NINDS · MASSACHUSETTS GENERAL HOSPITAL · PI CLEVELAND, DON W, LAGIER-TOURENNE, CLOTILDE · 2021 to 2021
$2.5M
Resolving the Role of Neuronal STING in Amyotrophic Lateral Sclerosis and Frontotemporal DementiaRF1NS127407 · NINDS · MASSACHUSETTS GENERAL HOSPITAL · PI LAGIER-TOURENNE, CLOTILDE, WAINGER, BRIAN JASON · 2023 to 2023
$2.1M
Defining the molecular connections between hyperexcitability and neurodegeneration in ALSF32NS114319 · NINDS · MASSACHUSETTS GENERAL HOSPITAL · PI HELD, AARON HARRY · 2020 to 2022
$136k
NINDS NIH HHS DP2 NS106664NINDS NIH HHS F32 NS114319NINDS NIH HHS R01 NS112503NINDS NIH HHS RF1 NS124203NINDS NIH HHS RF1 NS127407
6 · The paper itself

Abstract

Motor neuron degeneration, the defining feature of amyotrophic lateral sclerosis (ALS), is a primary example of cell-type specificity in neurodegenerative diseases. Using isogenic pairs of induced pluripotent stem cells (iPSCs) harboring different familial ALS mutations, we assess the capacity of iPSC-derived lower motor neurons, sensory neurons, astrocytes, and superficial cortical neurons to capture disease features including transcriptional and splicing dysregulation observed in human postmortem neurons. At early time points, differentially regulated genes in iPSC-derived lower motor neurons, but not other cell types, overlap with one-third of the differentially regulated genes in laser-dissected motor neurons from ALS compared with control postmortem spinal cords. For genes altered in both the iPSC model and bona fide human lower motor neurons, expression changes correlate between the two populations. In iPSC-derived lower motor neurons, but not other derived cell types, we detect the downregulation of genes affected by TDP-43-dependent splicing. This reduction takes place exclusively within genotypes known to involve TDP-43 pathology.

Indexed as

Amyotrophic Lateral SclerosisInduced Pluripotent Stem CellsDNA-Binding ProteinsGene ExpressionHumansMotor NeuronsDNA-Binding ProteinsALScell-type specificityCP: NeuroscienceCP: Stem cell researchdisease modelingnonsense-mediated decaysplicingTDP-43

Identifiers

PMID37651231
PMCPMC10622181
OpenAlexW4386277172

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC-ND
Read underepoch 390

Registered trials

None linked

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.