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Dr.M.Siva Kumar Reddy MDDepartment of Biochemistry
Sri Venkateswara Medical College
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Copper is an essential trace element (i.e., micronutrient) copper is essential to the proper functioning of organs
and metabolic processes. Body contains about 100 mg copper distributed in
different organ. Copper is incorporated into a variety
of proteins and metalloenzymes which perform essential metabolic functions.
Copper deficiency and toxicity can be either of genetic or non-genetic origin.
Introduction
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Biochemical functions
Biochemical functions of copper
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Cu- constituent of several enzymes
Cytochrome
oxidase
catalase
tyrosinase
Super oxide
dismutase
Monoamine oxidase
Ascorbic acid
oxidase
Phenol oxidase
and uricase
ALA synthase
copper
Due to its presence in a wide variety ofenzymes, copper is involved in many metabolic reaction.
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Copper - hemoglobin
Copper is necessary for the synthesis ofHemoglobin( Cu is a constituent of ALA synthase,needed for heme synthesis).
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Elastin &collagen - copper
Lysine Residues of collagen and elastin to allysin.
Lysyl oxidase( a copper-containing enzyme)
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Ceruloplasmin exhibits a copper-dependent oxidase activity.
oxidation of Fe2+ (ferrous iron) into Fe3+ (ferric iron)
Ceruloplasmin assist iron transport in the plasma in association with transferrin, which can carry iron only in the ferric state.
Ceruloplasmin and iron transport
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Melanin and phospholipids
Copper is necessary for the synthesis ofmelanin and phospholipids……..
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HEMOCYANIN
Hemocyanin, a copper protein complex in invertebrates, functions like hemoglobin for 0xygen transport.
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COPPER-CONTAINING NON-ENZYMATIC PROTEINS
HEMOCUPREIN
CEREBROCUPREIN
HEPATOCUPREIN
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Development of bone and nervous system
Bone development
Nervous system development
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•Adults2-3mg/d
•children0.5-2mg/d
Daily requirements
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Sources of copper
Liver kidney
Meat
Egg yolk cereals Green veggies
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Milk is poor source of copper
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Copper absorption
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Copper absorption
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Facilitators vs Inhibitors
1. Phytate
2. Zinc
3. molybdenum
1. Metallothionein
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Demineralization of bones, Demyelination of neural tissue, Anemia, Fragility of arteries, Myocardial fibrosis, Hypopigmentation of skin, Greying of hair.
Copper deficiency
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Plasma copperPlasma copper
100-200mg/dl
95% tightly bound to
ceruloplasmin
5% loosely bound to Albumin
Normal concentration of serum ceruloplasmin is 25-50 mg/dl.
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The symptoms of Menke's disease include decreased copper in plasma and anemia and depigmentation of hair.
Defect in the intestinal absorption of copper
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Menke’s disease
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Wilson’s disease
Copper is deposited in abnormal amounts in liver and lenticular nucleus of brain. This may lead to hepatic cirrhosis and brain necrosis
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Kayser fleischer ring
KF ring
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Low levels of copper and ceruloplasmin in plasma with increased excretion of copper in urine.
Copper deposition in kidney causes renal damage. This leads to increased excretion of amino acids, glucose, peptides and hemoglobin in urine.
Intestinal absorption of copper is very high, about 4-6 times higher than normal.
Wilson’s disease
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Treatment of Wilson's disease
penicillamine treatment relies on its binding to accumulated copper and elimination through urine
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Thank you