XVIII
Contents
27.9
Biosynthesis of Pyrimidine
Nucleotides
637
Salvage Pathways
638
De Novo
Synthesis
638
Formation of UMP
638
Formation of Other Pyrimidine
Nucleotides
639
Synthesis of Cytidine Nucleotides
640
Synthesis of Thymidine Nucleotides
640
Pyrimidine Analogues
641
Regulation of
de Novo
Pyrimidine
Biosynthesis
641
27.10 Coordination of Purine and Pyrimidine
Nucleotide Biosynthesis
642
27.11 Catabolism of Pyrimidine
Nucleotides
643
27.12 Abnormalities of Pyrimidine
Metabolism
644
Supplemental Readings
and References
644
CHAPTER
28
Hemoglobin
28.1
Structure of Hemoglobins
645
Globin Chains
645
Heme Group
646
28.2
Functional Aspects of Hemoglobin
646
Oxygen Transport
646
Mechanism of Oxygenation
650
Function, Metabolism, and Regulation of Organic
Phosphates in Erythrocytes
652
CO
2
Transport
655
Nitric Oxide (NO) Binding to Hemoglobin
656
Erythropoietin
656
28.3
Inherited Disorders of Hemoglobin
Structure and Synthesis
657
Normal Hemoglobins
657
Thalassemias
659
a-Thalassemias
660
Thalassemias of the /3-Globin Gene Family
662
8-/3
Thalassemias, Lepore Hemoglobins, and
Hereditary Persistence of Fetal Hemoglobin
(HPFH)
662
Hemoglobinopathies
664
Molecular Pathology
664
Unstable Hemoglobins
667
Secondary Polycythemia Syndromes
667
Congenital Methemoglobinemias and
Cyanosis
668
Hemoglobin S and Sickling Disorders
668
y-
and S-Globin Mutants
670
Screening and Prenatal Diagnosis
670
28.4
Derivatives of Hemoglobin
671
Carbon Monoxide-Hemoglobin
671
Carbaminohemoglobin
672
Methemoglobin
672
Sulfhemoglobin
673
Cyanmethemoglobin
673
Glycated Hemoglobins
674
Supplemental Readings
and References
674
CHAPTER
29
Metabolism of Iron and Heme
29.1
Iron Metabolism
675
Absorption of Iron from the Diet
675
Plasma Iron Transport
679
Storage of Iron
679
Coordinate Regulation of Iron Uptake and Storage
in Non-Erythroid Cells
679
Alterations of Plasma Transferrin
Concentration
680
Disorders of Iron Metabolism
681
Iron Deficiency Anemia
681
Iron-Storage Disorders
682
Hereditary Hemochromatosis
683
29.2
Heme Biosynthesis
684
Formation of
8
-Aminolevulinic Acid
684
