This document summarizes key information about copper, including its distribution and role in the body, requirements, biochemical functions, excess effects, and metabolic defects like Wilson's disease. It notes that copper is necessary for iron absorption, incorporation into hemoglobin, cellular respiration, connective tissue synthesis, antioxidant protection, and vitamin C requiring reactions. Wilson's disease results from a genetic defect impairing copper excretion from the liver, leading to accumulation and toxicity. The document also briefly discusses manganese, fluoride, and iodine requirements and effects.

1. COPPER
Prof. Dr. Viyatprajna Acharya
IMS & SUM Hospital

2. A case of Wilson’s disease
11 yr male child with irrelevant talks responded
to Risperidone……

3. Total body copper= 100mg
• Distributed in mm., liver, bone marrow, brain,
kidney, heart & hair
• Enzymes-
Ceruloplasmin
Cytochrome C
Cytochrome oxidase
ALA synthase
Lysyl oxidase
MAO
SOD
Phenol oxidase

4. Whole
blood=
100mg/dl
Cu
95% in RBC as
Erythrocuprein
Plasma= Ceruloplasmin
10% bound loosely
to albumin in
plasma- transport
form
Excretion-Through Bile

5. Requirement- 1.5-3 mg/day

6. Biochemical role of copper
• Necessary for iron absorption- ceruloplasmin
(oxidation of fe2+- Fe3+)
• Incorporation of iron into Hb
• Cellular respiration- Cytochrome c oxidase
• Connective tissue synthesis- Lysyl oxidase
• Protection against oxidants- SOD
• Necessary for Tyrosinase activity
• Co-factor for Vitamin C requiring
hydroxylations

7. Cu excess- oxidation of lipids and
proteins, Free radical generation
• Taken to liver by albumin
• 50% is absorbed in stomach and upper small
intestine
• Excreted in feces
• Partly excreted in bile
• Cu leaves the liver bound to Ceruloplasmin

8. Metabolic defect of Copper
• Wilson’s disease
• Copper deficiency anemia- ALA synthase
• CVS disorders- lysyl oxidase
• Menke’s kinky hair syndrome- Cu binding
ATPase defect
• Hypopigmentation- Tyrosinase

9. Wilson’s disease- Hepatolenticular
degeneration- ↓ Ceruloplasmin
Genetic defect of copper
binding ATPase gene
(Mutation in ATP7B protein)
↓
Copper elimination from liver
hampered
↓
Accumulates in liver and brain
Kayser-Fleischer ring
•Excess Cu- binds to apoceruloplasmin
•↓Ceruloplasmin in circulation
•CLD (Cirrhosis, hepatitis), Hemolytic anemia, Neurologic syndrome

11. Menke’s kinky hair syndrome
•X-linked defect, in boys
•Defect in Cu binding P type ATPase
•Cu is not absorbed from intestine
•Involves Nervous system, connective tissue and vasculature
•Fatal in infancy
•NO HEPATIC involvement

12. Copper excess- diarrhoea, green
discolouration of saliva
Cu poisoning- hemolysis, hemoglobinuria,
proteinuria, renal failure

14. Manganese
• D/R- 5mg/day
• Many enzymes are
Mn dependent or Mn
is an integral part of
them
• Deficiency- Impaired
growth and skeletal
deformities

15. Mn dependent enzymes
• Hexokinase
• Phosphoglucomutase
• Pyruvate carboxylase

16. FLOURIDE
• Known to prevent caries & tooth decay
• Flouride ions get incorporated into apatite crystal
surface making it resistant to bacterial attack
• 1ppm in water is safe limit
• Flourosis (>20ppm) – Excessive flourine intake
through water- Nalgonda, Nellore, Patiala, Angul
• Chr. Intestinal upset, gastroenteritis, loss of appetite
and loss of weight, Bone deformities like Genu
Valgum
• Alternate areas of osteosclerosis and osteoporosis

17. IODINE
• Known for its deficiency
• 235 districts in India- Iodine deficiency
endemic area
• D/R- 150-200 mg/d
• Iodine level in blood- 5-10 mg/dl
• Iodised salt – boon or bane??

19. FOR MORE PPT ON MEDICAL
BIOCHEMISTRY PLEASE VISIT MY
WEBSITE
http://www.vpacharya.com/