Evidence map›Paper›PMID 32423971›Full record

ArticleDisease models & mechanisms2020

Human embryoid bodies as a 3D tissue model of the extracellular matrix and α-dystroglycanopathies.

Alec R Nickolls, Michelle M Lee, Kristen Zukosky, Barbara S Mallon, Carsten G Bönnemann

Open access · goldAbstract read
In one paragraph

Article in Disease models & mechanisms, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.

0numbers the graph read from it
0cells of the map it votes in
11citing papers in PubMed
1.1field-weighted citation impact, top 24% 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

11 citing papers in PubMed, 15 citations in OpenAlex.

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  5. Sharing resources to advance translational research.Disease models & mechanisms · 2022
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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

5 authors at 1 institution in 1 country.

Alec R NickollsNational Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA.ORCID 0000-0002-7399-4304
Michelle M LeeNational Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA.
Kristen ZukoskyNational Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA.
Barbara S MallonNational Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA.ORCID 0000-0002-5292-098X
Carsten G BönnemannNational Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA carsten.bonnemann@nih.gov.ORCID 0000-0002-5930-2324
National Institutes of Health · US

Funding

Molecular and Clinical Manifestations of Matrix and Aggregate MyopathiesZIANS003129 · NINDS · NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE · PI BÖNNEMANN, CARSTEN · 2011 to 2025
$52.8M
Human pluripotent stem cellsZICNS003008 · NINDS · NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE · PI ROBEY, PAMELA · 2009 to 2025
$21.2M
6 · The paper itself

Abstract

The basal lamina is a specialized sheet of dense extracellular matrix (ECM) linked to the plasma membrane of specific cell types in their tissue context, which serves as a structural scaffold for organ genesis and maintenance. Disruption of the basal lamina and its functions is central to many disease processes, including cancer metastasis, kidney disease, eye disease, muscular dystrophies and specific types of brain malformation. The latter three pathologies occur in the α-dystroglycanopathies, which are caused by dysfunction of the ECM receptor α-dystroglycan. However, opportunities to study the basal lamina in various human disease tissues are restricted owing to its limited accessibility. Here, we report the generation of embryoid bodies from human induced pluripotent stem cells that model the basal lamina. Embryoid bodies cultured via this protocol mimic pre-gastrulation embryonic development, consisting of an epithelial core surrounded by a basal lamina and a peripheral layer of ECM-secreting endoderm. In α-dystroglycanopathy patient embryoid bodies, electron and fluorescence microscopy reveal ultrastructural basal lamina defects and reduced ECM accumulation. By starting from patient-derived cells, these results establish a method for the

Indexed as

Basement MembraneCase-Control StudiesCell Culture TechniquesCells, CulturedChildChild, PreschoolDystroglycansEmbryoid BodiesExtracellular MatrixFemaleGene Expression Regulation, DevelopmentalHuman Embryonic Stem CellsHumansInduced Pluripotent Stem CellsInfant, NewbornMaleDystroglycansRibitolDystroglycanExtracellular matrixMuscular dystrophyStem cells

Identifiers

PMID32423971
PMCPMC7328151
OpenAlexW3027295894

What OpenQuestion holds

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LicenceCC BY
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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.