copper metabolism and its clinical significance
TRANSCRIPT
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
FunctionsofCopper
qFunctionsofCopper:
1. ironabsorption2. IncorporationofironintoHb3. Cofactor:hydroxylationofvitaminC4. ProtectsheartbyincreasedLDL5. Ferroxidase (Ceruloplasmin)
Fe²⁺Fe³⁺(reduced)(oxidized)
CopperMetabolism• Absorption:duodenum• Excretion:bile(undernormalconditionurinedosenotcontaincopper.
BLOOD100microgram/dl
5%PlasmaCeruloplasmin(bluecolorglycoprotein)95%RBC
SymptomsofCopperToxicityCausesofcoppertoxicity:a) Hemolysis
b) Haemoglobinuria
c) Proteinuria
d) Renalfailure
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.