Iron Copper- Skildog Flashcards
What enzyme is needed in the apical membrane BEFORE Fe or Cu can be transported into an intestinal epithelial cell?
A brush border reductase
What transporter does Fe use to enter the enterocyte? How is it exported out the basolateral side?
Enters through DMT1 (divatlent metal transporter)
Leaves through Ferroportin channel
Why does iron inhibit zinc absorption?
They compete for the DMT1 transporter (on luminal side)
In what state is Fe transported in the blood?
Needs to be Fe3+
Binds to transferrin
What enzyme is responsible for oxidizing Iron so it can be transported in the blood? What other mineral is required?
Hephaestin (aka ceruloplasmin)
requires cooper
What protein regulates ferroportin on the basolateral side? (thus regulate blood Fe)
Hepsidin!
- High iron in liver
- transcription/translation of hepsidin
- Hepcidin binds ferroportin and causes its degredation
Where is iron stored?
Liver
What happens during iron toxicity?
Accumulates in tissue and acts as a free radical and causes oxidative damage.
What is chronic hemochromotosis?
- Inherited mutation in hepcidin
- Causes IRON OVERLOAD
What transporter does cooper use on the apical membrane? How does it get exported out of the basolateral side?
CTR1
ATP7A channel
Menke’s kinky hair syndrome is a mutation in what?
Mutation in ATP7A
Copper transporter on basolateral side
Causes Copper deficiency
Hypothermia Hypotonia Poor Feeding Failure to thrive Seizures FINE BRITTLE HAIR
Menke’s Kinky hair syndrome - mutation in ATP7A
Copper deficiency
Wilson Disease is a mutation in what?
ATP7B = copper transporter found on the liver
Is responsible for transporting excess cooper to the bile for excretion.
What are Kayser-Fleisher rings? What are they associated with?
Copper colored rings in around the eye
Associated with Wilson Disease
What is Gitelman Syndrome?
Mutation of SLC12A3
Na/Cl transporter in the DCT
Causes hypomagnesemia, hypokalemia, and hpocalciuria
