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Current Understanding of Pathology and Therapeutic Status for CADASIL

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Cerebral Ischemic Reperfusion Injuries (CIRI)

Part of the book series: Springer Series in Translational Stroke Research ((SSTSR))

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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.

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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

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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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Authors and Affiliations

  1. Department of Neurosurgery, State University of New York, Upstate Medical University, Syracuse, NY, USA

    Suning Ping & Li-Ru Zhao

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  1. Suning Ping
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  2. Li-Ru Zhao
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Correspondence to Li-Ru Zhao .

Editor information

Editors and Affiliations

  1. Department of Vascular Neurosurgery, PLA Rocket Force General Hospital, Beijing, China

    Weijian Jiang

  2. Department of Neurology, University of California Irvine, Irvine, California, USA

    Wengui Yu

  3. Department of Neurosurgery, Tangdu Hospital of Air Force Medical University, Xi’an, China

    Yan Qu

  4. Department of Neurosurgery, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China

    Zhongsong Shi

  5. Department of Neurology, The First Affiliated Hospital School of Medicine, Zhejiang University, Hangzhou, Zhejiang, China

    Benyan Luo

  6. Department of Anesthesiology, Loma Linda University, Loma Linda, California, USA

    John H. Zhang

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Ping, S., Zhao, LR. (2018). Current Understanding of Pathology and Therapeutic Status for CADASIL. In: Jiang, W., Yu, W., Qu, Y., Shi, Z., Luo, B., Zhang, J. (eds) Cerebral Ischemic Reperfusion Injuries (CIRI). Springer Series in Translational Stroke Research. Springer, Cham. https://doi.org/10.1007/978-3-319-90194-7_12

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  • DOI: https://doi.org/10.1007/978-3-319-90194-7_12

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