Steely Hair Syndrome

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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 the body to distribute and absorb copper. As a result, the brain and other parts of the body do not get enough copper.

Low copper levels can affect the structure of bone, skin,...

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