Fig. 4.

Atp7a^int/Y mice exhibit signatures of Cu deficiency. A: copper-dependent ceruloplasmin (Cp) activity in the plasma of Atp7a^wt/Y and Atp7a^int/Y mice at day P16. Values are expressed as means ± SE; ***P < 0.001. B: blood hemoglobin (Hb) levels (mean ± SE) in Atp7a^wt/Y and Atp7a^int/Y mice at day P16. No significant differences. C: cardiac hypertrophy in Atp7a^int/Y mice at P16. Values are expressed as average ratio of heart to body weight ± SE; ***P < 0.001. D: tyrosinase activity is reduced in the skin of Atp7a^int/Y mice at P16. In-gel oxidation of l-3,4-dihydroxyphenylalanine (l-DOPA) was used to assay tyrosinase activity in 10 μg of skin lysates fractionated by use of nonreducing SDS-PAGE. Immunoblot detection of actin was used to indicate protein loading. E: microscopic analysis of hair taken from the flanks of Atp7a^wt/Y and Atp7a^int/Y mice at P16. Note the progressive loss of pigmentation toward the base of the hair in Atp7a^int/Y mice consistent with a depletion of copper stores during neonatal growth.