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Quiz on
Heme Synthesis
Reading:
Textbook Reading: Voet & Voet, Biochemistry, 3rd Edition, p. 813 & 1013-1022.
Some recent articles
(optional reading):
H. L. Schubert, E. Raux, K. S. Wilson & M. J. Warren (1999) "Common chelatase
design in the branched tetrapyrrole pathways of heme and anaerobic cobalamin
synthesis," Biochemistry 38: 10660-10669.
G. A. Hunter & G. C. Ferreira (1999) "Pre-steady-state reaction of
5-Aminolevulinate Synthase," J. Biol. Chem. 274: 12222-12228.
S. W. Ryter & R. M. Tyrrell (2000) "The heme synthesis and degradation pathways:
Role in oxidant sensitivity" Free Radical Biol. & Med. 28: 289-309.
M. A. A. Mathews, H. L. Schubert, F. G. Whitby, K. J. Alexander, K. Schadick, H.
A. Bergonia, J. D. Phillips & C. P. Hill (2001) "Crystal structure of human
uroporphrinogen III synthase," EMBO J. 20: 5832-5839.
E. K. Jaffe, J. Martins, J. Li, J. Kervinen & R. L. Dunbrack (2001) "The
molecular mechanism of lead inhibition of human Porphobilinogen Synthase," J.
Biol. Chem. 276: 1531-1537.
Frére, F., Schubert,
W., Stauffer, F., Frankenberg, N., Jahn, R. Neier D., Heinz, D. (2002)
"Structure of porphobilinogen synthase from Pseudomonas aeruginosa in
complex with 5-fluorolevulinic acid suggests a double Schiff base mechanism,"
J. Mol. Biol. 320: 237-247.
P. T. Erskine, L. Coates, D. Butler, J. H. Youell, A. A. Brindley, S. P. Wood,
M. J. Warren, P. M. Shoolingin-Jordan & J. B. Cooper (2003) "X-ray structure of
a putative reaction intermediate of 5-aminolaevulinic acid dehydratase," Biochem.
J. 373: 733-738.
K. E. Anderson, J. R. Bloomer, H. L. Bonkovsky, J. P. Kushner, C. A. Pierach, N.
R. Pimstone & R. J. Desnick (2005) "Recommendations for the diagnosis and
treatment of the acute porphyrias," Ann. Intern. Med. 142: 439-450.
H. Fukuda, A. Casas & A. Batlle (2005) "Aminolevulinic acid: from its unique
biological function to its star role in photodynamic therapy," Internat. J.
Biochem. & Cell Biol. 37: 272-276.
R. S. Ajioka, J. D. Phillips & J. P. Kushner (2006) "Biosynthesis of heme in
mammals," Biochim. Biophys. Acta 1763: 723-736.
G. O. Latunde-Dada, A. T.
McKie & R. J. Simpson (2006) "Animal models with enhanced erythropoiesis and
iron absorption," Biochim. Biophys. Acta 1762: 414-423.
A. Pietrangelo (2006) "Hereditary hemochromatosis," Biochim. Biophys. Acta 1763:
700-710.
1.a. Write
out the reaction leading to formation of d-aminolevulinate
(ALA). Include names and structures of substrates and product of the reaction
(mechanism not required). What vitamin-derived cofactor participates in the
reaction?
b. Write out the reaction catalyzed by Porphobilinogen
Synthase (PBG Synthase), giving names and structures of reactants and product.
How are the substrates of the PBG Synthase reaction initially held at the active site?
Explain the effect of Pb++ on the PBG Synthase reaction. How are
effects of lead poisoning on the brain explained?
c. Summarize and explain the effect of genetic deficiency of
PBG Synthase on synthesis of ALA.
Copyright © 2000-2008 by Joyce J. Diwan. All rights reserved.
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