Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Enhanced thrombospondin-1 causes dysfunction of vascular endothelial cells derived from Fabry disease-induced pluripotent stem cells.

EBioMedicine - 1 Feb 2020

Do Hyo-Sang, Park Sang-Wook, Im Ilkyun, Seo Donghyuk, Yoo Han-Wook, Go Heounjeong, Kim Yoo Hyung, Koh Gou Young, Lee Beom-Hee, Han Yong-Mahn

Abstract excerpt

BACKGROUND: Fabry disease (FD) is a recessive X-linked lysosomal storage disorder caused by α-galactosidase A (GLA) deficiency. Although the mechanism is unclear, GLA deficiency causes an accumulation of globotriaosylceramide (Gb3), leading to vasculopathy. METHODS: To explore the relationship between the accumulation of Gb3 and vasculopathy, induced pluripotent stem cells generated from four Fabry patients...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to alpha-Galactosidase
Share to Endothelial Cells

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Endothelium, Vascular

Share to Fabry Disease

Share to Gene Editing

Share to Gene Knockout Techniques

Share to Induced Pluripotent Stem Cells

Share to Thrombospondin 1

Source

Open in PubMed Open full text

Identifiers

PMID: 31981984
DOI: 10.1016/j.ebiom.2020.102633
PMCID: PMC6992938

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Enhanced thrombospondin-1 causes dysfunction of vascular endothelial cells derived from Fabry disease-induced pluripotent stem cells.PMID 31981984Read ↗

Fasudil alleviates the vascular endothelial dysfunction and several phenotypes of Fabry disease.Semantic proximity Read ↗

CRISPR/Cas9-mediated suppression of A4GALT rescues endothelial cell dysfunction in a fabry disease vasculopathy model derived from human induced pluripotent stem cells.Semantic proximity Read ↗

Reversal of the Inflammatory Responses in Fabry Patient iPSC-Derived Cardiovascular Endothelial Cells by CRISPR/Cas9-Corrected Mutation.Semantic proximity Read ↗

CRISPR/Cas9-mediated suppression of A4GALT rescues endothelial cell dysfunction in a Fabry disease vasculopathy model derived from human induced pluripotent stem cellsSemantic proximity Read ↗

Clinical characteristics and induced pluripotent stem cells (iPSCs) disease model of Fabry disease caused by a novel GLA mutation.Semantic proximity Read ↗

Anderson–Fabry Disease: From Endothelial Dysfunction to Emerging TherapiesSemantic proximity Read ↗

Generation of a gene-corrected human isogenic iPSC line from a patient with Fabry disease carrying the GLA variant c.1069C>T using CRISPR/Cas9-mediated homology directed repair.Semantic proximity Read ↗

Harnessing iPSCs, 3D organoids, and multiomics to model rare vascular diseases: Emerging new approach methodologies.Semantic proximity Read ↗

Generation of human induced pluripotent stem cell line MHHi029-A from a male Fabry disease patient carrying c.959A > T mutation.Semantic proximity Read ↗

Generation of a GLA knock-out human-induced pluripotent stem cell line, KSBCi002-A-1, using CRISPR/Cas9.Semantic proximity Read ↗

Current and experimental therapeutics for Fabry diseaseSemantic proximity Read ↗

Generation of a CRISPR/Cas9-corrected-hiPSC line (DDLABi001-A) from Fabry disease (FD)-derived iPSCs having α-galactosidase (GLA) gene mutation (c.803_806del).Semantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.