MetabolismofCopperanditsclinicalsignificanceDr.Rohini CSane

CopperMetabolism• Totalbodycoppercontent: 1oomg• Dailydietaryrequirement: 1.5-3.0mg/day• Occurrence :muscles,bones,brain,heart,hair,kidney• Dietarysources:cereals,meat,liver,nuts,greenvegetables• EnzymecontainingCopper:Ceruloplasmin,Tyrosinase,Cytochromeoxidase,ALAsynthtase,Superoxidedismutase,Phenoloxidase• Enzymescontainingprotein:

Coppercontaing pigment Tissue

Cerebrocupreine Brain

Hepatocupreine Liver

Hemocupreine RBC

Erythrocupreine Bonemarrow

Hemocyanine Oxygencarryingbluepigment incrustacean

CopperMetabolism

CopperMetabolism

•

FunctionsofCopper

qFunctionsofCopper:

1. ironabsorption2. IncorporationofironintoHb3. Cofactor:hydroxylationofvitaminC4. ProtectsheartbyincreasedLDL5. Ferroxidase (Ceruloplasmin)

Fe²⁺Fe³⁺(reduced)(oxidized)

FunctionsofCopper

CopperMetabolism• Absorption:duodenum• Excretion:bile(undernormalconditionurinedosenotcontaincopper.

BLOOD100microgram/dl

5%PlasmaCeruloplasmin(bluecolorglycoprotein)95%RBC

CopperMetabolism

CopperMetabolism

1.COPPERSTORAGEFORM-CERULOPLASMIN

2.COPPERTRANSPOORTFORM

-ALBUMIN

Copperasanantioxidant

Copperfunctionsasacofactorforantioxidantenzyme

superoxidedismutase

CopperATPases

SymptomsofCopperToxicityCausesofcoppertoxicity:a) Hemolysis

b) Haemoglobinuria

c) Proteinuria

d) Renalfailure

ManagementofCopperToxicity

CopperMetabolism-Wilson’sDisease

CopperMetabolism-Wilson’sDisease

Menke’s DiseaseandCopperMetabolism

CopperMetabolism-Menke’s Disease

GeneticmutationassociatedwithMenke’s Disease

SymptomsofMenkes syndromeof CopperMetabolism

qSymptomsofMenkes syndromeare:

1. Bonespurs2. Brittle,kinkyhair3. Feedingdifficulties4. Irritability5. Lackofmuscletone,floppiness6. Lowbodytemperature7. Mentaldeterioration8. Pudgy,rosycheeks9. Seizures10. Skeletalchanges

DiagnosticTestsandtreatmentforMenke’s DiseaseqDiagnosticTestsforMenke’s Diseaseinclude:

1. Serumceruloplasmin(molecule thattransportscopperintheblood)

2. Serumcopperlevel

3. Skincellculture

4. X-rayoftheskeletonorx-rayoftheskull

5. Genetictestingmayshowachange(mutation)intheATP7A gene.

qManagementofMenke’s disease

• Treatmentusuallyonlyhelpswhenstartedveryearlyinthecourseofthedisease.Injectionsofcopperintoaveinorundertheskinhavebeenusedwithmixedresults.

ComparisonofWilson’sDiseaseandMenke’s Disease

Wilson’sandMenke’s DiseaseareHereditaryareassociatedwithCopperMetabolism

ComparisonofWilson’sandMenke’s DiseaseofCopperMetabolism

RoleofCholesterolApoA,ApoEinCopperMetabolism