Hemoglobin and Iron Metabolism — Flashcards

_____, probably acting through an aporepressor molecule, acts as a negative regulator of the synthesis of ALA Synthase-1 in the hepatic biosynthesis of Heme

Some of the iron absorbed in the duodenum is taken up by _____ for storage as ferritin

-aminolevulinic acid is converted to _____ via the enzyme -aminolevulinic acid dehydratase (heme synthesis)

In the first step of heme synthesis, _____ and succinyl-CoA are combined to form -aminolevulinic acid via the enzyme -aminolevulinic acid synthase (with vitamin B6 as a cofactor)

-thalassemia due to a _____ gene deletion is characterized by formation of 4 tetramers, known as Hb Barts

The _____ form of hemoglobin has a high affinity for O2

-aminolevulinic acid synthase is regulated via negative feedback by _____ and heme

The two embryonic globins are _____ and epsilon ()

_____ iron (meat-derived) is readily absorbed and enters the duodenal cell via the heme transporter

Primary _____ is due to mutations in the HFE gene on chromosome 6, leading to increased expression of DMT1 and decreased synthesis of Hepcidin