Learn About Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy

What is the definition of Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy?

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy, usually called CADASIL, is an inherited condition that causes stroke and other impairments. This condition affects blood flow in small blood vessels, particularly cerebral vessels within the brain. The muscle cells surrounding these blood vessels (vascular smooth muscle cells) are abnormal and gradually die. In the brain, the resulting blood vessel damage (arteriopathy) can cause migraines, often with visual sensations or auras, or recurrent seizures (epilepsy).

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What are the causes of Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy?

Mutations in the NOTCH3 gene cause CADASIL. The NOTCH3 gene provides instructions for producing the Notch3 receptor protein, which is important for the normal function and survival of vascular smooth muscle cells. When certain molecules attach (bind) to Notch3 receptors, the receptors send signals to the nucleus of the cell. These signals then turn on (activate) particular genes within vascular smooth muscle cells.

How prevalent is Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy?

CADASIL is likely a rare condition; however, its prevalence is unknown.

Is Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy an inherited disorder?

This condition is inherited in an autosomal dominant pattern, which means one copy of the altered NOTCH3 gene in each cell is sufficient to cause the disorder.

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Who are the sources who wrote this article ?

Published Date: April 01, 2019Published By: National Institutes of Health

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