Journal article

Menkes protein contributes to the function of peptidylglycine alpha-amidating monooxygenase

TC Steveson, GD Ciccotosto, XM Ma, GP Mueller, RE Mains, BA Eipper

Endocrinology | ENDOCRINE SOC | Published : 2003

Abstract

Menkes protein (ATP7A) is a P-type ATPase involved in copper uptake and homeostasis. Disturbed copper homeostasis occurs in patients with Menkes disease, an X-linked disorder characterized by mental retardation, neurodegeneration, connective tissue disorders, and early childhood death. Mutations in ATP7A result in malfunction of copper-requiring enzymes, such as tyrosinase and copper/zinc superoxide dismutase. The first step of the two-step amidation reaction carried out by peptidylglycine alpha-amidating monooxygenase (PAM) also requires copper. We used tissue from wild-type rats and mice and an ATP7A-specific antibody to determine that ATP7A is expressed at high levels in tissues expressin..

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