Copper Flashcards
The daily copper dietary requirement is between
2-6mg which is mainly obtained from red meat, cocoa, shell-fish, water pumped through copper pipes and chocolates.
Absorbed and transport by
Typically 40-60% copper is absorbed by the duodenum and is transported via metalloenzymes e.g. ascorbic acid oxidase.
Transport in plasma?
90% is bound to a conjugated metalloenzyme known as ceruloplasmin, 9% is bound to carrier proteins such as albumin and only 1% is free Cu²+.
Body content of copper is about
80-120mg.
Copper function?
Copper is involved in the process of erythropoiesis
Copper is critical for energy production in the cells
Copper is closely related to estrogen metabolism
Copper stimulates production of the neurotransmitters epinephrine, norepinephrine and dopamine.
Hypocuperaemia is associated with
- Anemia in infants
- Malnutrition in infants
- Menkes’ kinky-hair syndrome….genetic disorder where copper absorption -leads to a brain
- disease which then cause characteristic wiry-steel hair.
Hypercuperaemia is associated with:
-Pregnancy − Estrogen therapy − Thelassemia and anemia − Leukemia and Lymphoma particularly Hodgkin’s disease − Rheumatoid arthritis
Wilson’s disease:
- Autosomal recessive genetic disorder with onset in the 2nd or 3rd decade
– Onset in children aged 4-5yrs is also reported but rarely
– Serum ceruloplasmin and serum copper is low
– Increased urine and tissue copper due to excessive deposition of copper in
tissues particularly in the hepatocytes and basal ganglia of the brain leading to their steady degeneration.
Symptom of Wilson’s disease in liver
liver dysfunction) Jaundice, weakness, partial hypertension, cirrhosis, anorexia
Symptoms of Wilson’s disease CNS
- In the CNS: (neurological disorder) Tremor, in-coordination, ataxia, rigidity, dysphagia
Symptoms of Wilson’s disease in cornea
- In cornea: Kayser-Fleischer rings (green-brown discoloration) caused by copper deposition
Lab findings for wialsins disease
Low ceruloplasmin, low serum and fecal copper Increased tissue, urinary and CSF copper.
Treatment of Wilson’s disease
-Lifelong treatment is recommended
-Low-copper diet - avoid shellfish, chocolate, liver, mushrooms, nuts,
and various other foods.
-Vitamin B6 supplements (pyridoxine)
-Anti-copper agents * Zinc acetate (a chelating agent for Cu, balances Cu levels) * Trientine hydrochloride * Penicillamine and D-penicillamine
Copper Type of specimen ?
Serum or urine, however the collection MUST BE in metal free containers
– Hair sample can also be used particularly from patient with Menkes’
kinky-hair syndrome.
- 2-5cm long hair should be washed with acetone and digested with 70% pure nitric acid.