Related Experiment Video

Updated: Jul 13, 2026

A Visual Approach for Inducing Dolichoectasia in Mice to Model Large Vessel-Mediated Cerebrovascular Dysfunction
04:29

A Visual Approach for Inducing Dolichoectasia in Mice to Model Large Vessel-Mediated Cerebrovascular Dysfunction

Published on: May 17, 2024

[Study of the familiar form of vascular dementia (CADASIL)]

Keikichi Takahashi1, Kaichi Yoshizaki

  • 1National Institute for Longevity Sciences, National Center for Geriatrics and Gerontology, Gengo 36-3, Morioka-cho, Obu, 474-8522 Japan. takahsi@nils.go.jp

Nihon Shinkei Seishin Yakurigaku Zasshi = Japanese Journal of Psychopharmacology
|July 19, 2007
PubMed

Insights

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is not caused by Notch3 receptor defects. This study found no differences in Notch3 processing or signaling in CADASIL patients.

Area of Science:

  • Neurology
  • Genetics
  • Molecular Biology

Background:

  • Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a genetic disorder.
  • It is characterized by recurrent strokes, dementia, and migraines.
  • CADASIL is caused by mutations in the Notch3 receptor gene.

Purpose of the Study:

  • To investigate the underlying mechanisms of pathological alterations in CADASIL.
  • To determine if Notch3 receptor processing, trafficking, or signaling is impaired in CADASIL.

Main Methods:

  • Comparison of wild-type (WT) and mutant Notch3 receptors.
  • Analysis of receptor trafficking, processing, ligand binding, and signal transduction.

Main Results:

  • No significant differences were observed in receptor trafficking between WT and mutant Notch3.
  • No differences were found in receptor processing, ligand binding specificity, or signal transduction.
  • Mutant Notch3 receptors function similarly to WT receptors in these aspects.

Conclusions:

  • Pathological alterations in CADASIL are not due to defects in Notch3 receptor processing or signaling.
  • The precise molecular mechanisms causing CADASIL remain to be elucidated.
  • Further research is needed to identify the exact cause of CADASIL.

More Related Videos

A Mouse Model for Vascular Cognitive Impairment and Dementia Based on Needle-guided Asymmetric Bilateral Common Carotid Artery Stenosis05:12

A Mouse Model for Vascular Cognitive Impairment and Dementia Based on Needle-guided Asymmetric Bilateral Common Carotid Artery Stenosis

Published on: November 22, 2024

Using Retinal Imaging to Study Dementia09:17

Using Retinal Imaging to Study Dementia

Published on: November 6, 2017

Related Concept Videos

Dementia l: Introduction01:22

Dementia l: Introduction

Dementia is an acquired, progressive syndrome characterized by a decline in multiple cognitive domains severe enough to impair daily functioning and reduce independence. Although memory loss is a central feature, the diagnosis requires additional deficits involving language, executive function, visuospatial skills, judgment, calculation, or abstract reasoning. These cognitive impairments reflect underlying neurodegenerative or vascular processes that gradually disrupt neuronal networks...
Dementia01:30

Dementia

Dementia is a collective term for cognitive disorders primarily affecting memory, thinking, and reasoning. It is not a specific disease but a syndrome, with Alzheimer's disease being the most common cause, accounting for approximately 60-80% of cases. Other types include vascular dementia, Lewy body dementia, and frontotemporal dementia. Dementia affects millions worldwide, particularly older adults, though it is not a normal part of aging.
The progression of dementia is generally gradual.
Alzheimer Disease l: Introduction01:29

Alzheimer Disease l: Introduction

Alzheimer disease is a chronic, progressive, and irreversible neurodegenerative disorder and the most common cause of dementia in older adults. It leads to gradual neuronal loss, causing cognitive decline, behavioral changes, and loss of functional independence.Risk Factors and EtiologyThe disease is multifactorial. Age is the strongest risk factor, with prevalence doubling every 5 years after age 65. Genetic factors include mutations in genes such as APP, PSEN1, and PSEN2, which are associated...
Alzheimer Disease ll: Pathophysiology01:23

Alzheimer Disease ll: Pathophysiology

Alzheimer disease involves structural changes in the brain that begin long before symptoms appear. The most distinctive features are extracellular neuritic plaques and intracellular neurofibrillary tangles.Neuritic plaques form in the cerebral cortex and around blood vessels. These plaques contain a dense core of beta-amyloid (Aβ)—a toxic protein fragment that clumps outside neurons. The core is surrounded by damaged neuronal extensions, as well as reactive astrocytes and microglia. Abnormal...
Alzheimer's Disease: Overview01:26

Alzheimer's Disease: Overview

Alzheimer's Disease (AD) is a continually advancing neurodegenerative disorder, distinguished by escalating memory loss, cognitive dysfunction, and dementia. The disease unfolds in three stages: preclinical, mild cognitive impairment (MCI), and dementia. Its onset is insidious, and the progression gradual, with the cause not well explained by other disorders.
The clinical diagnosis of AD hinges on the presence of memory and other cognitive impairments. Biomarkers, such as changes in Aβ and tau...
Coronary Artery Disease I: Introduction01:30

Coronary Artery Disease I: Introduction

Coronary Artery Disease (CAD): An Overview with Scientific InsightsCoronary Artery Disease (CAD), often referred to as C-A-D, is a prevalent blood vessel disorder classified under the broader category of atherosclerosis. Atherosclerosis is a pathological process characterized by the hardening and narrowing of arteries due to the accumulation of atherosclerotic plaques. These plaques are composed of cholesterol, fatty substances, inflammatory cells, calcium, and fibrin, reducing blood flow to...