A copper deficient diet prevents hepatic copper accumulation and dysfunction in Long-Evans Cinnamon (LEC) rats with an abnormal copper metabolism and hereditary hepatitis

1994 ◽  
Vol 69 (2) ◽  
pp. 137-140 ◽  
Author(s):  
Naoki Sugawara ◽  
Chieko Sugawara
Keyword(s):  
Copper Metabolism ◽  
Copper Accumulation ◽  
Deficient Diet ◽  
Hepatic Copper ◽  
Lec Rats ◽  
Long Evans

Hepatic Copper Accumulation Induces DNA Strand Breaks in the Liver Cells of Long-Evans Cinnamon Strain Rats

2000 ◽  
Vol 276 (1) ◽  
pp. 174-178 ◽  
Author(s):  
Masanobu Hayashi ◽  
Tomoko Kuge ◽  
Daiji Endoh ◽  
Kenji Nakayama ◽  
Jiro Arikawa ◽  
...  
Keyword(s):  
Dna Strand Breaks ◽  
Liver Cells ◽  
Copper Accumulation ◽  
Strand Breaks ◽  
Hepatic Copper ◽  
Long Evans ◽  
Dna Strand

scholarly journals Soy Protein Isolate Enhances Hepatic Copper Accumulation and Cell Damage in LEC Rats

2003 ◽  
Vol 133 (5) ◽  
pp. 1250-1254 ◽  
Author(s):  
Kayo Yonezawa ◽  
Sachiko Nunomiya ◽  
Mitsue Daigo ◽  
Yasumitsu Ogra ◽  
Kazuo T. Suzuki ◽  
...  
Keyword(s):  
Soy Protein ◽  
Cell Damage ◽  
Soy Protein Isolate ◽  
Protein Isolate ◽  
Copper Accumulation ◽  
Hepatic Copper ◽  
Lec Rats

Pleiotropic effect of LEC mutation: a rodent model of Wilson's disease

1994 ◽  
Vol 266 (5) ◽  
pp. G907-G913 ◽  
Author(s):  
M. L. Schilsky ◽  
R. J. Stockert ◽  
I. Sternlieb
Keyword(s):  
Copper Concentration ◽  
Copper Metabolism ◽  
Subcellular Fractionation ◽  
Pleiotropic Effect ◽  
Subcellular Fractions ◽  
Maximal Velocity ◽  
Normal Delivery ◽  
Hepatic Copper ◽  
Lec Rats ◽  
And Control

Metabolic studies with 67Cu were undertaken to identify the site of the cellular defect in copper metabolism in the Long-Evans Cinnamon (LEC) rat. The apparent rate of copper uptake by LEC primary hepatocytes was increased [maximal velocity (Vmax) = 259 pmol.min-1.mg protein-1] compared with controls (Vmax = 161 pmol.min-1.mg protein-1); however, Michaelis-Menten constant (Km) values were comparable (11.8 and 12.7 microM, LEC and control, respectively). Efflux of copper from LEC and control hepatocytes was similar from 0 to 15 min, but was reduced from 15 to 60 min in the former. Although hepatic copper contents were markedly elevated in LEC rats compared with controls (658 +/- 199 vs. 21.5 +/- 6.6 micrograms/g dry wt), biliary copper concentration was reduced in LEC rats compared with controls (0.187 vs. 1.39 +/- 0.66 microgram/ml). Subcellular fractionation of LEC liver homogenates revealed approximately 75% of copper to be present in cytosol, with gradients of copper concentration from cytosol to either lysosome or microsomal subcellular fractions. LEC rat bile and hepatic microsome and lysosome fractions contained smaller fractions of 67Cu administered intravenously as cupric acetate compared with control rats. However, recovery of 67Cu in bile and in lysosomal subcellular fractions were similar for LEC and controls following administration of 67Cu-labeled asialoceruloplasmin, which is targeted to lysosomes. This discordance suggests a possible defect in the entry of copper into lysosomes but normal delivery of lysosomal copper to bile. Based on these findings, we conclude that the mutation in LEC rats alters copper transport at more than one cellular site.

scholarly journals Copper Supplementation, A Challenge in Cattle

Animals ◽  
2020 ◽  
Vol 10 (10) ◽  
pp. 1890
Author(s):  
Marta López-Alonso ◽  
Marta Miranda
Keyword(s):  
Copper Deficiency ◽  
Copper Toxicity ◽  
Copper Metabolism ◽  
Copper Accumulation ◽  
Hepatic Copper ◽  
Copper Status ◽  
Large Numbers ◽  
Copper Supplementation ◽  
Key Points ◽  
Copper Storage

Ensuring adequate copper supplementation in ruminants is a challenging task due to the complexity of copper metabolism in these animals. The three-way interaction between copper, molybdenum and sulphur (Cu-Mo-S) in the rumen makes ruminants, particularly cattle, very susceptible to suffering from secondary copper deficiency. Paradoxically, excessive copper storage in the liver to prevent deficiency becomes a hazard when ruminants are fed copper-supplemented diets even slightly above requirements. While cattle were traditionally thought to be relatively tolerant of copper accumulation, and reports of copper poisoning were until recently somewhat rare, in recent years an increased number of episodes/outbreaks of copper toxicity in cattle, particularly in dairy cattle, have been reported worldwide. The growing number of lethal cases reported seems to indicate that copper intoxication is spreading silently in dairy herds, urging the development of strategies to monitor herd copper status and improve farmers’ awareness of copper toxicity. In fact, monitoring studies carried out on numerous samples collected from culled animals in slaughterhouses and/or diagnostic laboratories have demonstrated that large numbers of animals have hepatic copper concentrations well above adequate levels in many different countries. These trends are undoubtedly due to copper supplementation aimed at preventing copper deficiency, as dietary copper intake from pasture alone is unlikely to cause such high levels of accumulation in liver tissue. The reasons behind the copper overfeeding in cattle are related both to a poor understanding of copper metabolism and the theory of “if adding a little produces a response, then adding a lot will produce a better response”. Contrary to most trace elements, copper in ruminants has narrow margins of safety, which must also be formulated considering the concentrations of copper antagonists in the diet. This review paper aims to provide nutritionists/veterinary practitioners with the key points about copper metabolism in cattle to guarantee an adequate copper supply while preventing excessive hepatic copper loading, which requires à la carte copper supplementation for each herd.

scholarly journals Expression of the Wilson disease gene is deficient in the Long-Evans Cinnamon rat

1994 ◽  
Vol 301 (1) ◽  
pp. 1-4 ◽  
Author(s):  
Y Yamaguchi ◽  
M E Heiny ◽  
N Shimizu ◽  
T Aoki ◽  
J D Gitlin
Keyword(s):  
Wilson Disease ◽  
Molecular Basis ◽  
Disease Gene ◽  
Genetic Disorder ◽  
Copper Accumulation ◽  
Hepatic Copper ◽  
Examine Gene Expression ◽  
Bona Fide ◽  
Rat Strain ◽  
Long Evans

Long-Evans Cinnamon rats develop a necrotizing hepatitis characterized by excessive hepatic copper accumulation, defective holoceruloplasmin biosynthesis and impaired biliary copper excretion. To elucidate the molecular basis of this defect, a cDNA clone encoding the rat Wilson disease gene was isolated and used to examine gene expression in selected tissues from normal and Long-Evans Cinnamon rats. Although this cDNA readily detects Wilson transcripts in liver and other tissues from normal rats, such transcripts are entirely absent from tissues derived from the Long-Evans Cinnamon rat strain. These data therefore identify the Long-Evans Cinnamon rat as the first bona fide animal model of Wilson disease and suggest that this rat strain may be a valuable resource in the study of this genetic disorder.

DIFFERENTIAL RESPONSES OF COPPER(I) AND COPPER(II) ON LIVER COPPER ACCUMULATION IN THE LONG-EVANS CINNAMON (LEC) RATS STUDIED BY PIXE DETERMINATION

2000 ◽  
Vol 10 (03n04) ◽  
pp. 155-159 ◽  
Author(s):  
A. Ohta ◽  
G. Bu ◽  
T. Matsubayashi ◽  
H. Mihara ◽  
N. Nemoto ◽  
...  
Keyword(s):  
Early Stage ◽  
Copper Accumulation ◽  
Body Sway ◽  
X Ray ◽  
Liver Copper ◽  
Supplementation Period ◽  
Lec Rats ◽  
Long Evans ◽  
Determination Of Copper

Effects of supplementation by copper(I) and copper (II) in Long-Evans Cinnamon(LEC) rats and life survival of rats are given. Long-Evans Cinnamon(LEC) strain rats were used at age of 8 weeks. The rats were randomly assigned to either copper(I) chloride or copper (II) chloride group. We have performed x-ray emissivite determination of copper in liver of LEC rats using PIXE and detected increase in copper in liver at early stage which could be supplemented by copper(I) and copper (II). During the supplementation period, clinical deterioration continued accompanied by daily body sway. The rats lost an undetermined amount of body weight and died approximately fourteen days later.

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