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Menke’s kinky hair syndrome

Hair serves to eliminate toxic materials (e.g., lead) and metabolites from the body, and may be used to monitor environmental contamination. For example, copper deficiency is a cause of Menke s kinky hair syndrome protein deficiency leads to hair loss and discoloration. Hair keratin carries a strong negative charge and binds inorganic materials it becomes prone to... [Pg.185]

Copper is an essential element to most life forms. In humans it is the third most abundant trace element only iron and zinc are present in higher quantity. Utilization of copper usually involves a protein active site which catalyzes a critical oxidation reaction, e.g., cytochrome oxidase, amine oxidases, superoxide dismutase, ferroxidases, dopamine-/ -hydrox-ylase, and tyrosinase. Accordingly, animals exhibit unique homeostatic mechanisms for the absorption, distribution, utilization, and excretion of copper (J). Moreover, at least two potentially lethal inherited diseases of copper metabolism are known Wilson s Disease and Menkes s Kinky Hair Syndrome (I). [Pg.265]

Danks, D. M., Campbell, P, E., Stevens, B. J., Mayne, V., and Cartwright, E, (1972a) Menkes s kinky hair syndrome. An inherited defect in copper absorption with widespread effects. Pediatrics 50 188. [Pg.133]

Copper Anaemia Menkes (kinky hair) syndrome Cardiac abnormalities/heart disease Wilson s disease Hepatic injury and jaundice Headache, vomiting Haemolytic shock... [Pg.66]

Packman S, O Toole C, Price DC, Thaler MM (1983) Cadmium, zinc, and copper metabolism in the mottled mouse, an animal model for Menkes kinky hair syndrome. J Inorg Biochem 19 203-211... [Pg.118]

Garnica, A. D., and Fletcher, S. R, (1975) Parenteral copper in Menkes kinky-hair syndrome. Lancet 2 659,... [Pg.134]

Menke s syndrome (kinky hair disease) + + +/- Males with wormian bones, low serum copper and tortuous intracranial vessels... [Pg.171]


See other pages where Menke’s kinky hair syndrome is mentioned: [Pg.667]    [Pg.667]    [Pg.385]    [Pg.590]    [Pg.118]    [Pg.418]    [Pg.170]   
See also in sourсe #XX -- [ Pg.2 , Pg.185 ]

See also in sourсe #XX -- [ Pg.2 , Pg.185 ]




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