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Brain Arteriovenous Malformation Modeling, Pathogenesis, and Novel Therapeutic Targets

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Abstract

Patients harboring brain arteriovenous malformation (bAVM) are at life-threatening risk of rupture and intracranial hemorrhage (ICH). The pathogenesis of bAVM has not been completely understood. Current treatment options are invasive, and ≈ 20 % of patients are not offered interventional therapy because of excessive treatment risk. There are no specific medical therapies to treat bAVMs. The lack of validated animal models has been an obstacle for testing hypotheses of bAVM pathogenesis and testing new therapies. In this review, we summarize bAVM model development and bAVM pathogenesis and potential therapeutic targets that have been identified during model development.

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Abbreviations

bAVM:

Brain arteriovenous malformation

ICH:

Intracranial hemorrhage

HHT:

Hereditary hemorrhagic telangiectasia

ENG:

Endoglin

ALK1:

Activin-like kinase 1

VEGF:

Vascular endothelial growth factor

PDGFB:

Platelet-derived growth factor-B

AV:

shunt Arteriovenous shunt

f:

Allele with two loxP sites flanking the target sequence

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Acknowledgments

We thank Voltaire Gungab for assistance with manuscript preparation and members of the UCSF BAVM Study Project (http://avm.ucsf.edu) for their support. The principal investigator, Hua Su, is supported by grants from the National Institutes of Health (R01 NS027713 and P01 NS044155).

Compliance with Ethics Requirements

This review article does not contain any studies with human or animal subjects. All cited studies describe ethical standards in cited manuscripts.

Conflict of Interest

Wanqiu Chen, Eun-Jung Choi, Cameron M. McDougall, and Hua Su declare that they have no conflict of interest.

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  1. Center for Cerebrovascular Research, Department of Anesthesia and Perioperative Care, University of California, San Francisco, 1001 Potrero Avenue, Box 1363, San Francisco, CA, 94110, USA

    Wanqiu Chen, Eun-Jung Choi, Cameron M. McDougall & Hua Su

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  1. Wanqiu Chen
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  2. Eun-Jung Choi
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Correspondence to Hua Su.

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Wanqiu Chen and Eun-Jung Choi contributed equally to this review.

This article is dedicated in memory of our collaborator and mentor William L. Young, MD, for his seminal contributions to brain AVM research.

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Chen, W., Choi, EJ., McDougall, C.M. et al. Brain Arteriovenous Malformation Modeling, Pathogenesis, and Novel Therapeutic Targets. Transl. Stroke Res. 5, 316–329 (2014). https://doi.org/10.1007/s12975-014-0343-0

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