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Updated: May 25, 2026
06:49
A High Output Method to Isolate Cerebral Pericytes from Mouse
Published on: January 14, 2020
Pericytes as a new target for pathological processes in CADASIL
Dorota Dziewulska1, Eliza Lewandowska
1Department of Neurology, Medical University ofWarsaw, Banacha Str.1a, 02-097 Warsaw, Poland. dorota.dziewulska@wum.edu.pl
Summary
Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) damages not only smooth muscle cells but also pericytes in brain capillaries. This pericyte damage contributes to microcirculation issues and white matter injury.
Area of Science:
- Neurology
- Vascular Biology
- Genetics
Background:
- Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) is a genetic angiopathy linked to NOTCH3 gene mutations.
- It primarily affects vascular smooth muscle cells (VSMC) in small arteries and arterioles, leading to degeneration and loss.
- The NOTCH3 receptor is also expressed on pericytes, suggesting potential pericyte involvement in CADASIL pathology.
Purpose of the Study:
- To investigate the hypothesis that pericytes and capillary vessels are damaged in CADASIL.
- To examine microvessels in autopsy brains and skin-muscle biopsies from CADASIL patients.
Main Methods:
- Microscopic examination of autopsy brain and skin-muscle biopsy samples from CADASIL patients.
- Analysis of pericyte morphology, endothelial-pericytic connections, and capillary lumen.
- Identification of granular osmiophilic material (GOM) deposits.
Main Results:
- Degeneration and loss of pericytes were observed in capillary vessels of CADASIL patients.
- Pericytes showed shrinkage, cytoplasmic vacuoles, and pathological mitochondria.
- Degenerative changes in endothelial-pericytic connections, including peg-and-socket junctions, were noted.
- Granular osmiophilic material (GOM) was found near pericyte membranes.
- Capillary endothelial cells exhibited degeneration, death, or swelling, leading to lumen narrowing or occlusion.
Conclusions:
- CADASIL severely damages not only VSMC but also pericytes within brain capillaries.
- Pericyte involvement can increase capillary permeability and disrupt cerebral microcirculation, contributing to white matter injury.
- Degeneration of pericytes, which regulate capillary contractility, may explain early defective vasomotor reactivity in CADASIL.
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