Abstract
Menkes' (Kinky Hair) Disease, an inborn error of Cu-metabolism, is detectable in in vitro cultured skin fibroblasts. Such cells accumulate significant more 64-Cu from the medium than control cells: the results of 9 otherwise verified patients and of 24 controls illustrate this (see Figure). Some unusual results of patients will be mentioned, among which one confirmed Menkes patient, which had unusual biochemical characteristics and a patient which had an unknown Cu-deficiency, different from Menkes' Disease. Some complicating factors of the 64-Cu uptake will be discussed: the influence of confluency of the cultures, Cu content of the medium and the presence of such Cu binding ligands as albumin. Incorporated 64-Cu by human skin fibroblast cultures (o =from controls, Δ =from patients, each symbol represents one culture,
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