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Current Understanding of Pathology and Therapeutic Status for CADASIL

  • Suning Ping
  • Li-Ru ZhaoEmail author
Chapter
Part of the Springer Series in Translational Stroke Research book series (SSTSR)

Abstract

Recently, it has been drawn an increased attention on a hereditary form of stroke and vascular dementia named cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). Because of poor understanding of the pathogenesis of this disease, the treatment that specifically delay or stop the pathological progression of CADASIL has not yet been developed. This chapter provides an update on CADASIL research. The pathological features and possible molecular mechanisms of CADASIL are outlined, the involvement of vascular endothelial cells in pathological progression of CADASIL is introduced, and emerging studies in treatment research for CADASIL is reviewed and discussed.

Keywords

CADASIL Notch3 mutation Vascular smooth muscle cells Endothelial cells Capillary thrombosis Pathology Treatment 

Abbreviations

CADASIL

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy

EGFrs

Epidermal growth factor-like repeats

G-CSF

Granulocyte colony stimulating factor

GOM

Granular osmiophilic materials

MA

Migraine with aura

N3ECD

NOTCH3 N-terminal extracellular domain

N3TMIC

NOTCH3 transmembrane intracellular domain

SCF

Stem cell factor

VSMCs

Vascular smooth muscle cells

Notes

Acknowledgements

This work was supported by American Heart Association (15GRNT25700284).

Funding information This work was supported by American Heart Association (15GRNT25700284).

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© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Neurosurgery, State University of New YorkUpstate Medical UniversitySyracuseUSA

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