Definitions

Wilson disease

Wilson disease

or hepatolenticular degeneration

Recessive hereditary defect (see recessiveness) that impairs one's ability to metabolize copper. In affected persons, copper accumulates in the basal ganglia (see ganglion) of the brain (involved in control of movement), causing progressive degeneration; forms a brownish ring at the margin of the cornea of the eye; and is deposited in the liver, gradually leading to cirrhosis. Other symptoms include tremor, lack of coordination, and personality changes. The disease usually appears in the person's teen years or twenties. Early diagnosis and treatment with a high-protein, low-copper diet and a substance to chelate copper can reverse the effects and prevent permanent brain and liver damage.

Learn more about Wilson disease with a free trial on Britannica.com.

Wilson disease protein (also called ATP7B) is an ATPase that transports copper.

See also

External links

References

Further reading

Search another word or see wilson diseaseon Dictionary | Thesaurus |Spanish
Copyright © 2014 Dictionary.com, LLC. All rights reserved.
  • Please Login or Sign Up to use the Recent Searches feature
FAVORITES
RECENT

;