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Metabolism	of	Copper	and	its	clinical	significance
Dr.	RohiniC	Sane
Copper	Metabolism	
• Total	body	copper	content	: 1oomg	
• Daily	dietary	requirement	: 1.5-3.0	mg/day	
• Occurrence :	muscles	,bones,	brain,	heart	,hair,	kidney
• Dietary	sources:	cereals,	meat	,	liver,	nuts,	green	vegetables	
• Enzyme	containing	Copper	:	Ceruloplasmin	,Tyrosinase	,Cytochrome	
oxidase	,ALA	synthtase	,Super	oxide	dismutase,	Phenol	oxidase
• Enzymes	containing	protein		:
Copper	containg pigment Tissue	
Cerebrocupreine Brain
Hepatocupreine Liver
Hemocupreine RBC
Erythrocupreine Bone	marrow	
Hemocyanine Oxygen	carrying	blue	pigment	in	crustacean
Copper Metabolism
Copper	Metabolism	
•
Functions	of	Copper
qFunctions	of	Copper	:
1. iron	absorption	
2. Incorporation	of	iron	into	Hb
3. Cofactor	:	hydroxylation	of	vitamin	C	
4. Protects	heart	by	increased	LDL	
5. Ferroxidase (	Ceruloplasmin)
Fe²⁺																										Fe³⁺
(	reduced	)											(	oxidized	)
Functions	of		Copper
Copper	Metabolism	
• Absorption	:	duodenum	
• Excretion	:	bile	(	under	normal	condition	urine	dose	not	contain	
copper	.
BLOOD
100microgram	/dl		
5%	Plasma	Ceruloplasmin	
(	blue	color	glycoprotein	)95%	RBC
Copper Metabolism
Copper Metabolism
1.COPPER	STORAGE	FORM
-CERULOPLASMIN
2.COPPER	TRANSPOORT	FORM
-ALBUMIN
Copper	as	an	antioxidant	
Copper	functions	as	a	cofactor	for	anti	oxidant	enzyme	
super	oxide	dismutase
Copper	ATP	ases
Symptoms	of	Copper	Toxicity
Causes	of	copper	
toxicity:
a) Hemolysis
b) Haemoglobinuria
c) Proteinuria	
d) Renal	failure
Management	of		Copper	Toxicity
Copper Metabolism-Wilson’s	Disease
Copper	Metabolism-Wilson’s	Disease
Menke’s Disease	and	Copper	Metabolism
Copper Metabolism-Menke’s Disease
Genetic	mutation	associated	with	Menke’s Disease
Symptoms	of	Menkes syndrome	of Copper	Metabolism	
qSymptoms	of	Menkes syndrome	are:
1. Bone	spurs
2. Brittle,	kinky	hair
3. Feeding	difficulties
4. Irritability
5. Lack	of	muscle	tone,	floppiness
6. Low	body	temperature
7. Mental	deterioration
8. Pudgy,	rosy	cheeks
9. Seizures
10. Skeletal	changes
Diagnostic	Tests	and	treatment	for	Menke’s Disease
qDiagnostic	Tests	for	Menke’s Disease	include:
1. Serum	ceruloplasmin	(molecule	 that	transports	copper	in	the	blood)
2. Serum	copper	level
3. Skin	cell	culture
4. X-ray	of	the	skeleton	or	x-ray	of	the	skull
5. Genetic	testing	may	show	a	change	(mutation)	in	the	ATP7A gene.
qManagement	of	Menke’s disease
• Treatment	usually	only	helps	when	started	very	early	in	the	course	of	the	
disease.	Injections	of	copper	into	a	vein	or	under	the	skin	have	been	used	with	
mixed	results.
Comparison	of	Wilson’s	Disease	and	Menke’s Disease
Wilson’s		and	Menke’s Disease	are	Hereditary	are	associated	with	Copper	Metabolism
Comparison	of	Wilson’s		and	Menke’s Disease		of			Copper	Metabolism
Role	of	Cholesterol	Apo	A	,Apo	E	in	Copper	Metabolism
Copper metabolism and its clinical significance
Copper metabolism and its clinical significance

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