Dr.M.Siva Kumar Reddy MDDepartment of Biochemistry

Sri Venkateswara Medical College

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

Biochemical functions

Biochemical functions of copper

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.

Copper - hemoglobin

Copper is necessary for the synthesis ofHemoglobin( Cu is a constituent of ALA synthase,needed for heme synthesis).

Elastin &collagen - copper

Lysine Residues of collagen and elastin to allysin.

Lysyl oxidase( a copper-containing enzyme)

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

Melanin and phospholipids

Copper is necessary for the synthesis ofmelanin and phospholipids……..

HEMOCYANIN

Hemocyanin, a copper protein complex in invertebrates, functions like hemoglobin for 0xygen transport.

COPPER-CONTAINING NON-ENZYMATIC PROTEINS

HEMOCUPREIN

CEREBROCUPREIN

HEPATOCUPREIN

Development of bone and nervous system

Bone development

Nervous system development

•Adults2-3mg/d

•children0.5-2mg/d

Daily requirements

Sources of copper

Liver kidney

Meat

Egg yolk cereals Green veggies

Milk is poor source of copper

Copper absorption

Copper absorption

Facilitators vs Inhibitors

1. Phytate

2. Zinc

3. molybdenum

1. Metallothionein

Demineralization of bones, Demyelination of neural tissue, Anemia, Fragility of arteries, Myocardial fibrosis, Hypopigmentation of skin, Greying of hair.

Copper deficiency

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.

The symptoms of Menke's disease include decreased copper in plasma and anemia and depigmentation of hair.

Defect in the intestinal absorption of copper

Menke’s disease

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

Kayser fleischer ring

KF ring

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

Treatment of Wilson's disease

penicillamine treatment relies on its binding to accumulated copper and elimination through urine

Thank you