ascorbic-acid and Menkes-Kinky-Hair-Syndrome

ascorbic-acid has been researched along with Menkes-Kinky-Hair-Syndrome* in 3 studies

Reviews

1 review(s) available for ascorbic-acid and Menkes-Kinky-Hair-Syndrome

Article	Year
Copper transport: an overview. Proceedings of the Society for Experimental Biology and Medicine. Society for Experimental Biology and Medicine (New York, N.Y.), 1991, Volume: 196, Issue:2 Topics: Animals; Ascorbic Acid; Biological Transport, Active; Ceruloplasmin; Copper; Glutathione; Histidine; Intestines; Menkes Kinky Hair Syndrome; Metallothionein; Mice; Rats; Serum Albumin	1991

Other Studies

2 other study(ies) available for ascorbic-acid and Menkes-Kinky-Hair-Syndrome

Article	Year
Vitamin C treatment in Menkes' disease: failure to affect biochemical and clinical parameters. Journal of inherited metabolic disease, 1989, Volume: 12 Suppl 2 Topics: Ascorbic Acid; Brain Diseases, Metabolic; Copper; Humans; Infant; Male; Menkes Kinky Hair Syndrome	1989
Menkes disease: is vitamin C treatment effective? Brain & development, 1985, Volume: 7, Issue:5 The pathogenesis of Menkes disease seems to be linked to metallothionein which binds to copper trapped within cells in some tissues. The only known therapy for this disease is parenteral administration of copper, but the effects are equivocal. We treated a patient with Menkes disease by giving vitamin C orally. The clinical manifestation and bone changes improved and the plasma copper and ceruloplasmin levels gradually increased. Vitamin C may prevent the binding of copper and metallothionein by its reducing effect, and excess copper would be released from the cells. Vitamin C treatment is a simple and physiological method, and should aid in clarifying the pathogenesis of the disease. Topics: Ascorbic Acid; Brain Diseases, Metabolic; Humans; Infant; Male; Menkes Kinky Hair Syndrome; Tomography, X-Ray Computed	1985

Article

Year

Vitamin C treatment in Menkes' disease: failure to affect biochemical and clinical parameters.

Journal of inherited metabolic disease, 1989, Volume: 12 Suppl 2

Topics: Ascorbic Acid; Brain Diseases, Metabolic; Copper; Humans; Infant; Male; Menkes Kinky Hair Syndrome

1989

Menkes disease: is vitamin C treatment effective?

Brain & development, 1985, Volume: 7, Issue:5

The pathogenesis of Menkes disease seems to be linked to metallothionein which binds to copper trapped within cells in some tissues. The only known therapy for this disease is parenteral administration of copper, but the effects are equivocal. We treated a patient with Menkes disease by giving vitamin C orally. The clinical manifestation and bone changes improved and the plasma copper and ceruloplasmin levels gradually increased. Vitamin C may prevent the binding of copper and metallothionein by its reducing effect, and excess copper would be released from the cells. Vitamin C treatment is a simple and physiological method, and should aid in clarifying the pathogenesis of the disease.

Topics: Ascorbic Acid; Brain Diseases, Metabolic; Humans; Infant; Male; Menkes Kinky Hair Syndrome; Tomography, X-Ray Computed

1985