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In page Canavan disease:

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Canavan disease is caused by a defective ASPA gene which is responsible for the production of the enzyme aspartoacylase. Decreased aspartoacylase activity prevents the normal breakdown of N-acetylaspartate, wherein the accumulation of N-acetylaspartate, or lack of its further metabolism interferes with the growth of the myelin sheath of the nerve fibers of the brain. The myelin sheath is the fatty covering that surrounds nerve cells and acts as an insulator, allowing for efficient transmission of nerve impulses.[citation needed][2]