Abstract
Menkes FB demonstrate a greater accumulation and a reduced efflux of copper than normal FB. As shown by DNA synthesis, copper was toxic to Menkes FB even at 2 μg/ml which was found stimulatory to normal FB. Treatment of labeled FB with trypsin did not affect the level of 64Cu accumulation. Most of the 64Cu incorporated was bound to a molecule with a MW of approximately 10,000. The amount of radioactivity bound to this molecule was greater in Menkes than in normal FB. A small amount of radioactivity, proportionally greater in normal than in Menkes FB, was bound to a molecule(s) with a MW > 100,000. 64Cu incubated with lysates of normal and Menkes FB bound preferentially to a molecule with a MW of approximately 10,000. By incubating increasing amounts of 64Cu with a constant amount of FB protein, an increasing amount of radioactivity was bound to MW species > 100,000. in a mixture of 0.12μg 64Cu with 4 mg Menkes FB protein, all the radioactivity was bound to the small MW species, whereas in a similar mixture with normal FB, only 70% of the 64Cu was bound to the small MW form, and the remainder to the large MW molecule(s). Even at saturating 64Cu concentrations, the radioactivity bound to the 10,000 MW species was still greater in the Menkes FB. Mixtures of fractions of the labeled large and low MW species with lysates from normal and Menkes FB showed interconversion of the radioactivity between these molecular forms. These data indicate that copper is preferentially bound to a small molecule and that Menkes FB bind more copper than normal FB.
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Labodle, G., Beratis, N., Price, P. et al. 532 SPECIFIC 64cu BINDING IN MENKES AND NORMAL SKIN FIBRO-BLASTS (FB). Pediatr Res 12 (Suppl 4), 452 (1978). https://doi.org/10.1203/00006450-197804001-00537
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DOI: https://doi.org/10.1203/00006450-197804001-00537