Evidence map›Paper›PMID 39868835›Full record

ArticleCNS neuroscience & therapeutics2025

Morphological Characteristics and Extracellular Matrix Abnormalities in Astrocytes Derived From iPSCs of Children With Alexander Disease.

Huan Yi, Jie Zhang, Kai Gao, Wei Yan, Hongyuan Chu, Junjiao Zhang, Fan Zhang, Yuwu Jiang, Jingmin Wang, Ye Wu

Abstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in CNS neuroscience & therapeutics, 2025. 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.

Huan YiChildren's Medical Center, Department of Pediatric Neurology, Peking University First Hospital, Beijing, China.ORCID 0000-0003-2525-6937
Jie ZhangChildren's Medical Center, Department of Pediatric Neurology, Peking University First Hospital, Beijing, China.ORCID 0000-0002-4047-0897
Kai GaoChildren's Medical Center, Department of Pediatric Neurology, Peking University First Hospital, Beijing, China.
Wei YanChildren's Medical Center, Department of Pediatric Neurology, Peking University First Hospital, Beijing, China.
Hongyuan ChuChildren's Medical Center, Department of Pediatric Neurology, Peking University First Hospital, Beijing, China.
Junjiao ZhangChildren's Medical Center, Department of Pediatric Neurology, Peking University First Hospital, Beijing, China.
Fan ZhangChildren's Medical Center, Department of Pediatric Neurology, Peking University First Hospital, Beijing, China.
Yuwu JiangChildren's Medical Center, Department of Pediatric Neurology, Peking University First Hospital, Beijing, China.
Jingmin WangChildren's Medical Center, Department of Pediatric Neurology, Peking University First Hospital, Beijing, China.
Ye WuChildren's Medical Center, Department of Pediatric Neurology, Peking University First Hospital, Beijing, China.

Funding

Beijing Key Laboratory of Molecular Diagnosis and Study on Pediatric Genetic Diseases BZ0317Beijing Natural Science Foundation 7244416Key Project of Clinical Medicine Research of National Clinical Research NCRCCHD-2021-KP-02National High Level Hospital Clinical Research Funding (Scientific Research Seed Fund of Peking University First Hospital) 2023SF42National Key Research and Development Program of China 2020YFA0804000National Key Research and Development Program of China 2021YFC2401202National Natural Science Foundation of China 81901155National Natural Science Foundation of China 82171435National Natural Science Foundation of China 82171694National Natural Science Foundation of China U22A20339
6 · The paper itself

Abstract

aimsAlexander disease (AxD) is a leukodystrophy caused by mutations in the astrocytic filament gene GFAP. There are currently no effective treatments for AxD. Previous studies have rarely established AxD models with the patient's original GFAP mutations. In this study, we aimed to explore the morphological and transcriptomic characteristics of GFAP-mutant astrocytes via induced pluripotent stem cell (iPSC) models of AxD.

methodsFibroblasts from three AxD children were reprogrammed into iPSCs. Wild-type (WT) and AxD-iPSCs were differentiated into astrocytes. We compared the morphological and transcriptomic differences between WT- and AxD iPSC-derived astrocytes.

resultsAstrocytes induced from AxD-derived iPSCs exhibited the Rosenthal fibers (RFs), the main pathological phenotype of AxD. Compared with WT astrocytes, AxD astrocytes had shorter processes, more branches, and larger cell bodies. Transcriptomic analysis revealed that extracellular matrix (ECM) components, particularly chondroitin sulfate proteoglycans (CSPGs), were upregulated, and ECM-degrading enzymes were generally downregulated. These changes may lead to abnormalities in neurons and myelination.

conclusionsWe explored the morphological characteristics of AxD astrocytes via iPSC models and revealed the ECM, previously unexplored for AxD, may be an important new pathogenic mechanism of this disease.

Indexed as

Alexander DiseaseAstrocytesExtracellular MatrixInduced Pluripotent Stem CellsCell DifferentiationCells, CulturedChildFemaleGlial Fibrillary Acidic ProteinHumansMaleGFAP protein, humanGlial Fibrillary Acidic ProteinAlexander diseaseextracellular matrixGFAPinduced pluripotent stem cellsRosenthal fibers

Identifiers

PMID39868835
PMCPMC11770893

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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.