1122 copper-binding (cub) by proteins from kidneys and livers of brindled heterozygotes (brh)


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ABSTRACT The Br (CeH/MoBrJ) mouse is a model for Menkes Kinky Hair Synd., a Cu metabolic disease. Extracts of Cu-binding protein (CuBP) were prepared from livers(Li) and kidneys(Ki) of adult


Cu-loaded(CuL) and non Cu-loaded(NCuL) nl(CeH/HeJ) and BrH mice. S.C. CuSO4 soln., 35μg/kg Cu/dx20d was given. Extracts were applied to Sephadex G-75-120; profiles were monitored by


absorbance at 215, 250, 280 nm, [Cu], and [Cd]. Eight protein peaks (PP) were obtained, 6 Cu-containing. PP A, m.w. 37,000D, was present in Li only after CuL. Thus, PP A in Li may be


Cuinduced. CuL seems to induce Cd-binding to PP A, except in Ki of BrH. PP B, m.w. 29,000D, is found only in Ki of NCuL BrH. PP C, m.w. 15,700D, is present in all extracts. A major PP in Li,


the [Cu]:A215 is low in Li and Ki of nl and Li of CuL nl. PP D, est. m.w. 5,250D, is absent in Ki: in NCuL it is present only in Li of BrH. PP E from Li of nl is P-rich with minor CuB; it


appears to bind Cd in Li of nl and BrH after CuL. PP F was observed only in Li of nl. We cannot say whether P induction has taken place. The increase in CuB relative to PP suggests that


excess Cu is bound to existing P. Our CuL data suggest that CuB in Li and Ki of BrH is similar to Br hemizygotes; these data indicate CuB among soluble P of nl and BrH is different. Question


remains whether abnormal Cu content at tissue level in BrH can be explained only on the basis of soluble PBCu at a cellular level. ARTICLE PDF AUTHOR INFORMATION AUTHORS AND AFFILIATIONS *


Dept. of Pediatrics, U. of Fla. College of Medicine, Gainesville, Fla. A Garnica & S M T Chang Authors * A Garnica View author publications You can also search for this author inPubMed 


Google Scholar * S M T Chang View author publications You can also search for this author inPubMed Google Scholar RIGHTS AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE THIS


ARTICLE Garnica, A., Chang, S. 1122 COPPER-BINDING (CuB) BY PROTEINS FROM KIDNEYS AND LIVERS OF BRINDLED HETEROZYGOTES (BrH). _Pediatr Res_ 15 (Suppl 4), 629 (1981).


https://doi.org/10.1203/00006450-198104001-01148 Download citation * Issue Date: 01 April 1981 * DOI: https://doi.org/10.1203/00006450-198104001-01148 SHARE THIS ARTICLE Anyone you share the


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