Fig. 2.

Atp7b^ΔHep mice accumulate copper and lack holo-CP. A: copper levels in the Atp7b^ΔHep livers compared with Atp7b^Lox/Lox controls (n = 5–6). B: copper excretion in the urine of Atp7b^ΔHep mice and Atp7b^Lox/Lox littermates (n = 5–6). C: copper accumulation in Atp7b^ΔHep livers differs in males and females; sex dependence was not apparent in Atp7b^−/− and Atp7b^+/+ animals (n = 5–8 for each genotype and each sex; P value determined using 2-tailed unpaired t-test). D: ceruloplasmin in the liver and serum of Atp7b^ΔHep mice and control littermates. Top: total ceruloplasmin in the liver is unchanged in Atp7b^ΔHep mice. Middle: analysis of ceruloplasmin in a serum under denaturing conditions (Serum Denat) shows lower levels of CP in Atp7b^ΔHep mice compared with control Atp7b^Lox/Lox animals. Bottom: ratio of holo-CP and apo-CP in a serum under nondenaturing conditions (Serum Non Denat). Atp7b^+/+ and Atp7b^−/− were used as positive (holo-CP) and negative (apo-CP) controls.