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Article

NOTCH3 signal activation by its extracellular domain accumulation in an iPSC line newly established from a CADASIL patient.

Human cell - 24 May 2026

Sun Ruihua, Zhao Zhenxiang, Che Ningning, Liu Ning, Ji Xiaoyi, Li Anqi, Wang Fengyu, Shang Junkui, Zhang Jiewen

Abstract excerpt

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common monogenic cerebral small-vessel disease caused by NOTCH3 mutations, yet its pathogenic mechanisms remain incompletely understood due to limited disease models. The NOTCH3 R544C mutation is a prevalent hotspot in East Asian populations, but patient-derived iPSC models are lacking. Here, we...

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Topics

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Share to Basic Helix-Loop-Helix Proteins
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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Share to Receptor, Notch3

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Source

Identifiers

PMID: 42177738
DOI: 10.1007/s13577-026-01391-3

Genes

NOTCH3
HES1
HEY1
NANOG
TRA

Variants

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026.

Related research

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Research trail→

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NOTCH3 signal activation by its extracellular domain accumulation in an iPSC line newly established from a CADASIL patient.PMID 42177738Read ↗

Modeling CADASIL vascular pathologies with patient-derived induced pluripotent stem cellsSemantic proximity Read ↗

Generation and characterization of the human iPSC line IDISi001-A isolated from blood cells of a CADASIL patient carrying a NOTCH3 mutation.Semantic proximity Read ↗

NOTCH3 R545C mutation drives vascular-neuronal dysfunction and cognitive impairment in CADASIL pathogenesis.Semantic proximity Read ↗

CADASIL from Bench to Bedside: Disease Models and Novel Therapeutic ApproachesSemantic proximity Read ↗

Generation of human induced pluripotent stem cells (NIHTVBi004-A, NIHTVBi005-A, NIHTVBi006-A, NIHTVBi007-A, NIHTVBi008-A) from 5 CADASIL patients with NOTCH3 mutation.Semantic proximity Read ↗

Progress to Clarify How NOTCH3 Mutations Lead to CADASIL, a Hereditary Cerebral Small Vessel Disease.Semantic proximity Read ↗

NOTCH3 Mutations in CADASIL: a multicellular perspective on neurodegeneration.Semantic proximity Read ↗

Blood vessel organoids generated by base editing and harboring single nucleotide variation in Notch3 effectively recapitulate CADASIL-related pathogenesis.Semantic proximity Read ↗

Blood vessel organoids generated by base editing and harboring single nucleotide variation in Notch3 effectively recapitulate CADASIL-related pathogenesisSemantic proximity Read ↗

Human iPS cell-derived mural cells as an in vitro model of hereditary cerebral small vessel disease.Semantic proximity Read ↗

Novel human pathological mutations. Gene symbol: NOTCH3. Disease: cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL).Semantic proximity Read ↗

Novel human pathological mutations. Gene symbol: NOTCH3. Disease: cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL).Semantic proximity Read ↗

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