• homozygotes showed elevated serum ferritin

• aceruloplasminemic mice showed normal copper gastrointestinal absorption, hepatic uptake, and biliary copper excretion

• the copper content of brain, heart, spleen, and kidney was normal, and homozygotes exhibited normal copper-zinc superoxide dismutase activity in these tissues

• notably, hepatic copper content was significantly elevated in aceruloplasminemic mice

• ferrokinetic studies showed normal rates of iron absorption, initial tissue iron distribution, and plasma iron turnover

• no differences were detected in cellular iron uptake; however, homozygotes displayed a significant impairment in hepatocyte iron efflux

• at 1 year of age, there was no evidence of diabetes, anemia, or neurological deficits despite iron accumulation in these sites

• progressive accumulation of parenchymal iron, reaching a 3- to 6-fold increase in the iron content of the spleen by 1 year of age

• the spleen displayed normal cellular architecture with abundant iron in reticuloendothelial cells

• progressive accumulation of parenchymal iron, reaching a 3- to 6-fold increase in the iron content of the liver by 1 year of age (J:57730)

• the liver displayed normal cellular architecture with abundant iron in reticuloendothelial cells (J:57730)

• hepatic iron displayed a 3.5-fold increase in aceruloplasminemic mice due to loss of ferroxidase function (J:71807)

• the hemoglobin concentration remained normal relative to wild-type (J:57730)

• progressive accumulation of parenchymal iron, reaching a 3- to 6-fold increase in the iron content of the spleen by 1 year of age

• the spleen displayed normal cellular architecture with abundant iron in reticuloendothelial cells

• aceruloplasminemic mice showed normal copper gastrointestinal absorption, hepatic uptake, and biliary copper excretion

• the copper content of brain, heart, spleen, and kidney was normal, and homozygotes exhibited normal copper-zinc superoxide dismutase activity in these tissues

• notably, hepatic copper content was significantly elevated in aceruloplasminemic mice

• progressive accumulation of parenchymal iron, reaching a 3- to 6-fold increase in the iron content of the liver by 1 year of age (J:57730)

• the liver displayed normal cellular architecture with abundant iron in reticuloendothelial cells (J:57730)

• hepatic iron displayed a 3.5-fold increase in aceruloplasminemic mice due to loss of ferroxidase function (J:71807)

• progressive accumulation of parenchymal iron, reaching a 3- to 6-fold increase in the iron content of the spleen by 1 year of age

• the spleen displayed normal cellular architecture with abundant iron in reticuloendothelial cells

• retinas exhibit increased iron at 5-6 months of age, with highest levels in the RPE and photoreceptor outer segments (J:92620)

• levels of the cytosolic iron storage protein ferritin are increased in retinas (J:92620)

• choroidal thinning (J:92620)

• retinas exhibit increased iron at 5-6 months of age, with highest levels in the RPE and photoreceptor outer segments (J:92620)

• age dependent subretinal neovascularization (J:92620)

• local photoreceptor degeneration (J:92620)

• age dependent retinal epithelium hypertrophy, hyperplasia and necrosis (J:92620)

• retinas of mutants surviving 6-9 months exhibit focal areas of hypopigmentation in the midperipheral retina (J:92620)

• age dependent retinal epithelium hyperplasia (J:92620)

• mutants surviving 6-9 months exhibit retinal degeneration (J:92620)

• age dependent subretinal neovascularization (J:92620)

• local photoreceptor degeneration (J:92620)

• age dependent retinal epithelium hypertrophy, hyperplasia and necrosis (J:92620)

• retinas of mutants surviving 6-9 months exhibit focal areas of hypopigmentation in the midperipheral retina (J:92620)

• age dependent retinal epithelium hyperplasia (J:92620)