• Home

Home > Medical Reference > Encyclopedia (English)

Toggle: English / Spanish

Menkes syndrome

• Overview
• Symptoms
• Treatment
• Prevention

Definition:

Menkes syndrome is an inborn error of metabolism in which cells in the body cannot absorb enough copper.

Alternative Names:

Steely hair disease; Menkes kinky hair syndrome; Kinky hair disease

Causes, incidence, and risk factors:

Menkes syndrome is caused by a defect in the ATP7A gene. The defect makes it hard for body to proper distribute and absorb copper. As a result, the brain and other parts of the body do not get enough copper. Insufficient copper levels can affect the structure of bone, skin, hair, and blood vessels and interfere with nerve function. In the meantime, copper builds up in the small intestine and kidneys.

Menkes syndrome is inherited, which means it runs in families. It is inherited in an x-linked recessive condition.

• Review Date: 5/22/2007
• Reviewed By: Brian Kirmse, M.D., Department of Human Genetics, Mount Sinai School of Medicine, New York, NY. Review provided by VeriMed Healthcare Network.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is the first of its kind, requiring compliance with 53 standards of quality and accountability, verified by independent audit. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial process . A.D.A.M. is also a founding member of Hi-Ethics (www.hiethics.com) and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 1997-2007 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.