Conversion of AAs to Specialized Products Flashcards
What are the 3 sources of our AA pool?
1) Diet
2) Actual body protein
3) Synthesis of non-essential AAs that we make
What is the most common porphyrin in humans?
Heme
What is a porphyrin?
Cyclic compounds that binds metal ions (usually Fe2+ or Fe3+)
What many roles does heme have?
Hemoglobin, myoglobin, cytochromes, cytochrome P450 monooxygenase system, catalase, NO synthase and peroxidase
What are the major sites of heme biosynthesis?
Liver and erythrocyte-producing cells of bone marrow
What specific AA do we use for the making of heme?
Glycine
-Glycine is combined w/ succinyl CoA
What is the most important, rate-limiting, committed step of the making of heme?
ALAS1
What is heme biosynthesis inhibited by?
Heme
What is the 2nd step of heme biosynthesis (ALA to Porphobilinogen) inhibited by?
Lead
What is the last enzyme of heme biosynthesis?
Ferrochelastase (mitochondrial enzyme)
-Protoporphyrin IX -> Heme (iron is just added to center with N’s pointing in attached to it)
What is the last step of heme biosynthesis inhibited by?
Lead
What is porphyrias?
Rare, inherited defects in heme synthesis -> accumulation and increased excretion of porphyrins or porphyrin precursors
How long does a RBC live? Where is it degraded?
120 days
-Degraded in liver and spleen
What is the first step of heme degradation?
An old heme is picked up by a macrophage in spleen -> Heme oxygenase breaks open the heme (using NADPH) -> Biliverdin (green pigment)
What happens to biliverdin?
Reduced to bilirubin (red-orange) by biliverdin reductase (using NADPH)