ArticleNeuropathology and applied neurobiology2015
Immunolocalization of platelet-derived growth factor receptor-β (PDGFR-β) and pericytes in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL).
Article in Neuropathology and applied neurobiology, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers.
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Who cites it
27 citing papers in PubMed, 47 citations in OpenAlex.
- 3D Vessels-on-Chip using isogenic hiPSC-derived VSMCs reveal NOTCH3-driven alterations in brain small vessel disease.Stem cell reports · 2026Article
- Pericytes in the optic nerve head.Progress in retinal and eye research · 2025Review
- Screening and Identification of Brain Pericyte-Selective Markers.CNS neuroscience & therapeutics · 2025Article
- Progress to Clarify HowBiomolecules · 2024Review
- A high-throughput single-cell RNA expression profiling method identifies human pericyte markers.Neuropathology and applied neurobiology · 2023Article
- ER stress induced immunopathology involving complement in CADASIL: implications for therapeutics.Acta neuropathologica communications · 2023Article
- Neuropathology of microbleeds in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL).Journal of neuropathology and experimental neurology · 2023Article
- Evidence of beta amyloid independent small vessel disease in familial Alzheimer's disease.Brain pathology (Zurich, Switzerland) · 2022Article
- Normal cerebrospinal fluid concentrations of PDGFRβ in patients with cerebral amyloid angiopathy and Alzheimer's disease.Alzheimer's & dementia : the journal of the Alzheimer's Association · 2022Article
- Charcot-Bouchard aneurysms revisited: clinicopathologic correlations.Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2021Article
- Notch3 Signaling and Aggregation as Targets for the Treatment of CADASIL and Other NOTCH3-Associated Small-Vessel Diseases.The American journal of pathology · 2021Review
- Loss with ageing but preservation of frontal cortical capillary pericytes in post-stroke dementia, vascular dementia and Alzheimer's disease.Acta neuropathologica communications · 2021Article
- The expression of B7-H3 isoforms in newly diagnosed glioblastoma and recurrence and their functional role.Acta neuropathologica communications · 2021Article
- Neural crest cell-derived pericytes act as pro-angiogenic cells in human neocortex development and gliomas.Fluids and barriers of the CNS · 2021Review
- The pericyte: A critical cell in the pathogenesis of CADASIL.Cerebral circulation - cognition and behavior · 2021Review
- Loss of capillary pericytes and the blood-brain barrier in white matter in poststroke and vascular dementias and Alzheimer's disease.Brain pathology (Zurich, Switzerland) · 2020Article
- Human iPS cell-derived mural cells as an in vitro model of hereditary cerebral small vessel disease.Molecular brain · 2020Article
- Insulin-Independent and Dependent Glucose Transporters in Brain Mural Cells in CADASIL.Frontiers in genetics · 2020Article
- Blood brain barrier leakage is not a consistent feature of white matter lesions in CADASIL.Acta neuropathologica communications · 2019Article
- Modeling CADASIL vascular pathologies with patient-derived induced pluripotent stem cells.Protein & cell · 2019Article
Corrections and comments
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Authors and funding
5 authors at 2 institutions in 2 countries.
Funding
Abstract
aimsCerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is identified by aggregates of NOTCH3 extracellular domain (N3ECD) along capillaries and the deposition of granular osmiophilic material (GOM). We assessed the pattern of distribution of pericytes in relation to N3ECD deposits in cerebral microvessels of CADASIL subjects.
methodsWe assessed post mortem brains from (n = 50) subjects with CADASIL, cerebral small vessel disease, and similar-age cognitively normal and older controls. Immunohistochemical and immunofluorescent staining methods were used to study the distribution and quantify immunoreactivities of the platelet-derived growth factor receptor-β (PDGFR-β) (for pericytes) and microvascular markers in the frontal cortex and white matter.
resultsPDGFR-β antibody stained cells typical of pericytes in capillaries and small arterioles in both the grey and white matter. PDGFR-β reactive pericytes adopted 'crescent' morphology wrapped closely around capillary walls readily evident in cross-sections. We noted considerable overlap between PDGFR-β and N3ECD imunoreactivities in capillaries. Quantitative analysis of PDGFR-β immunoreactivity revealed significant differences in PDGFR-β %A in CADASIL compared with young controls (P < 0.05). PDGFR-β %A was further positively correlated with the basement membrane marker collagen IV (r = 0.529, P = 0.009), but was not associated with GLUT-1, the marker for endothelial cells.
conclusionsOur results suggest increased expression of PDGFR-β immunoreactive pericytes in cerebral microvessels in CADASIL compared with similar age controls. While we cannot confirm whether PDGFR-β-expressing pericytes produce N3ECD and hence GOM, our findings demonstrate that up-regulation of pericyte-like cells is associated with microvascular changes, including loss of vascular smooth muscle cells in CADASIL.
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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.