Copper is essential for maintaining the strength of the skin, blood vessels, epithelial and connective tissue.

1. J.THOUFIGA KATHIJA
I –BNYS “A” SECTION
BIOCHEMISTRY

2. COPPER is an essential trace element that is
Vital to the health of all living things (humans,
plants, animals, and microorganisms).
In humans, copper is essential to the proper
functioning of organs and metabolic processes.
A 70 Kg human adult body contain
approximately 80 mg of copper.
Highest concentrations are found in *Liver
*kidneys
Significant amount in *Cardiac Muscles
*skeletal Muscles
*Bone

3. SHELLFISH YOLK
DARK
CHOCOLATE
LEGUMES
DIETARY FOOD SOURCES
*Recommended Dietary Allowance = 2 to 3 mg per day

4. Poor source of copper

5. Ceruloplasmic(ferroxidase)
Cytochrome oxidase
Superoxide dismutase
Dopamine b-hudroxylase
Tyrosinase
Tryptophan dioxygenase
Lysyl oxidase
COPPER CONTAINING ENZYMES

6. 1.MOBILIZATION OF IRON (IRON TRANSPORT):
#Cu is an integral part of
CERULOPLASMIN which catalyses the
conversion of Fe2+ Fe3+
#Iron can be transported in
Fe3+ form
2.SYNTHESIS OF HEMOGLOBIN
(As a constitute of ALA synthase)
FUNCTIONS

7. 3. Formation of COLLAGEN, ELASTIN
and CROSS LINKING as a constitute of
LYSYL OXIDASE
4. Synthesis of MELANIN as a
constituent of TYROSINASE
5. CATECHOLAMINE synthesis is a
constituent of DOPAMINE OXIDASE
6. ANTIOXIDANT function

8. COPPER METABOLISM
•Copper absorbed from Duodenum
•Metallothionein is a transport protein that
facilitates copper absorption
•Phylates , Zinc and Molybdenum decreases
Copper uptake
•Excretion is mainly through Bile
•Urine doesn’t contain Copper in normal
circumstances.

9. ABSORPTION AND EXCRETION

10. DEFICIENCY MANIFESTATIONS
#NEUTROPENIA (decreased number of
neutrophils) and HYPOCHROMIC ANEMIA in early
stages
#OSTEOPOROSIS and various bone and joint
abnormalities
#Decreased pigmentation of SKIN
#Laterstages,NEUROLOGICAL ABMORMALITIES

11. INBORN ERROR OF
COPPER METABOLISM
Menkes syndrome Wilson’s disease

12. MENKES SYNDROME OR KINKY – HAIR DISEASE
•It is very #Rare
#Fatal
#X – linked recessive disorder
•Genetic defect in absorption of copper from
intestine
•Both serum copper and ceruloplasmic and liver
copper content are low.

13. Clinical manifestations occur early in
life & include :
Kinky or Twisted brittle hair due to loss of
copper catalyzed disulfide bond formation.
Depigmentation of the skin and hair
Seizures
Mental retardation
Vascular defects (lesion of the blood vessels)

15. s
WILSON’S DISEASE
 Inborn error of copper metabolism.
 Autosomal recessively inherited disorder caused by a
mutation in the gene ATP7B that codes for cation
transporting enzymes involved in copper transport which
leads to impaired :
#Copper excretion into bile.
#Reabsorption of copper in the kidney
#Hepatic incorporation of copper
into ceruloplasmic

16. COPPER TOXICITY occurs due to copper deposition in Liver,
Brain and Kidney
Copper deposits in the eye can sometimes be seen as yellow or
brown pigment around the iris, Kayser – Fleischer rings
Excessive deposition of copper in the brain leads to neurological
symptoms, #in liver leads to cirrhosis
#in kidney leads to Renal tubular damage.
MANIFESTATIONS

18. TREATMENT
By administration of a chelating agent, Penicillamine
to promote urinary copper excretion.
Penicallamine for life
Liver transplantation may be considered, particularly
in young patients with severe diseases