Johann Schaller, Department of Chemistry & Biochemistry, University of Bern, Switzerland.Simon Gerber, Department of Chemistry & Biochemistry, University of Bern, Switzerland.Urs Kaempfer, Department of Chemistry & Biochemistry, University of Bern, Switzerland.Sofia Lejon, Department of Chemistry & Biochemistry, University of Bern, Switzerland.Christian Trachsel, Department of Chemistry & Biochemistry, University of Bern, Switzerland.

• Preface xi1 Introduction 1References 3Part I 52 Blood Components 72.1 Introduction 72.2 Short History 72.3 Blood Components 82.3.1 Blood cells 82.3.2 Blood plasma 122.3.3 The blood group system 13References 15Data Sheet 163 Blood Plasma Proteins 173.1 Introduction 173.2 Short History 173.3 Classification of Blood Plasma Proteins 17References 20Part II 214 Domains, Motifs and Repeats 234.1 Introduction 234.2 The Epidermal Growth Factor (EGF)-Like Domain 244.3 The Kringle Domain 264.4 The g-Carboxyglutamic Acid-Rich (Gla) Domain 274.5 The PAN/Apple Domain 284.6 The Sushi/CCP/SCR Domain 304.7 The Fibronectin Type I, the Fibronectin Type II Collagen-Binding and the Fibronectin Type III Domains 314.8 The Coagulation Factors 5/8 Type C Domain (FA58C) 324.9 The Thrombospondin Type I Repeat (TSP1) 344.10 The VWFA, VWFC and VWFD Domains 354.11 The Cystatin-Like Domain 364.12 The LDL-Receptor Class A (LDLRA) and Class B (LDLRB) Domains 364.13 The C-Terminal Cystine Knot (CTCK) Structure 384.14 The Anaphylatoxin Domain 404.15 The CUB Domain 404.16 The NTR Domain 414.17 The C-Type Lectin (CTL) Domain 424.18 The Ig-Like Domain 43References 445 Protein Families 475.1 Introduction 475.2 Serine Proteases: The Trypsin Family 475.3 Serine Protease Inhibitors (Serpins) 485.4 The a2-Macroglobulin Family 505.5 The Serum Albumin Family 525.6 The Pancreatic Trypsin Inhibitor (Kunitz) Family Signature 525.7 The Kazal Serine Protease Inhibitors Family Signature 535.8 The Multicopper Oxidase Family 545.9 The Lipocalin Family 545.10 The Globin Family 555.11 The Glucagon/Gastric Inhibitory Polypeptide (GIP)/Secretin/ Vasoactive Intestinal Peptide (VIP) Family 565.12 The Glycoprotein Hormone Family 585.13 Membrane Attack Complex Components/Perforin Signature (The Complement C6/C7/C8/C9 Family) 595.14 The Lipase Family 605.15 Hormone Families and Signatures 615.15.1 The erythropoietin/thrombopoietin signature 615.15.2 The corticotropin-releasing factor family 615.15.3 The gonadotropin-releasing hormone signature 625.15.4 The calcitonin/CGRP/IAPP family 625.15.5 The gastrin/cholecystokinin family 625.15.6 The insulin family 625.16 Growth Factor Families 645.16.1 The platelet-derived growth factor family 645.16.2 The transforming growth factor-b family 645.17 Cytokine Families 655.17.1 The tumor necrosis factor family 655.17.2 The interferon a, b and d family 665.18 Interleukin Families and Signatures 665.18.1 The interleukin-1 signature 665.18.2 The interleukin-2 signature 675.18.3 The interleukin-4/-13 signature 685.18.4 The interleukin-6/granulocyte colony-stimulating factor/myelomonocytic growth factor family 685.18.5 The interleukin-7 and -9 signature 695.18.6 Interleukin-10 family 695.19 The Small Cytokine (Intercrine/Chemokine) Family and Signature 70References 726 Posttranslational Modifications 756.1 Introduction 756.2 Databases for Posttranslational Modifications (PTMs) 756.3 Disulfide Bridges 756.4 Glycosylation 776.5 Phosphorylation 796.5.1 Ser/Thr phosphorylation sites 806.5.2 Tyr phosphorylation site 816.6 Hydroxylation 816.6.1 Aspartic acid and asparagine hydroxylation site 816.7 Sulfation 826.8 Acylation and Alkylation 846.9 Amidation 846.10 Carboxylation 846.11 Crosslinks 856.11.1 The isoglutamyl cysteine thioester bond 856.11.2 The isopeptide bond 85References 86Part III 897 Blood Coagulation and Fibrinolysis 917.1 Introduction 917.1.1 Definition of haemostasis 917.2 Primary Haemostasis 927.3 The Coagulation Cascade 957.4 The Fibrinolytic System 1097.5 The Regulation of Blood Coagulation and Fibrinolysis 1147.6 The Kinin and Angiotensin/Renin Systems 116References 119Data Sheets 1218 The Complement System 1518.1 Introduction 1518.1.1 Nomenclature 1528.2 The Classical Pathway 1528.2.1 The anaphylatoxins C3a, C4a and C5a 1608.3 The Alternative Pathway 1608.4 The Terminal Components of the Complement System 1628.5 Components of Complement Activation 1658.6 Regulation of the Complement System 167References 171Data Sheets 1729 The Immune System 1959.1 Introduction 1959.2 Immunoglobulins 1969.3 Antibody Diversity 2039.4 Major Histocompatibility Complex (MHC) Proteins 2059.5 Innate Immune System 2099.6 Other Proteins 213References 214Data Sheets 21510 Enzymes 23110.1 Introduction 23110.2 EC 1: Oxidoreductases 23110.2.1 EC 1.11: peroxidases 23310.3 EC 2: Transferases 23610.3.1 EC 2.3: acyltransferases 23610.3.2 EC 2.6: transfer of nitrogenous group 23710.3.3 EC 2.7: transfer of phosphorous-containing groups 23710.4 EC 3: Hydrolases 23910.4.1 EC 3.1: esterases (ester bond) 23910.4.2 EC 3.2: glycosylases (sugars) 24210.4.3 EC 3.4: peptidases (peptide bonds) 243EC 3.4.17: metallocarboxypeptidases 243EC 3.4.21: serine endopeptidases (serine proteases) 243EC 3.4.22: cysteine endopeptidases (cysteine proteases) 250EC 3.4.23: aspartic endopeptidases (aspartic proteases) 25310.5 EC 5: Isomerases 25510.5.1 EC 5.3: oxidoreductases (intramolecular) 256References 257Data Sheets 25811 Inhibitors 28311.1 Introduction 28311.2 Serine Protease Inhibitors (Serpins) 28311.2.1 Introduction 28311.2.2 Structural properties 28411.2.3 Inhibitory reaction 28611.3 Blood Coagulation and Fibrinolysis 28611.3.1 Blood coagulation 28711.3.2 Fibrinolysis 29111.4 The Complement System 29211.5 General Inhibitors 29311.6 Other Serpins 29411.7 Other Inhibitors 294References 298Data Sheets 29912 Lipoproteins 31712.1 Introduction 31712.2 Enzymes in Lipoprotein Metabolism 31812.3 Apolipoproteins 32012.4 LDL and VLDL Receptors 32412.5 Serum Amyloid A Proteins 325References 326Data Sheets 32713 Hormones 34313.1 Introduction 34313.2 Pancreatic Hormones 34313.3 Gastrointestinal Hormones 34613.4 Calcium-Related Hormones 34713.5 Hormone-Releasing Factors, Their Trophic Hormones and Related Hormones 34913.6 Vasopressin and Oxytocin 35513.7 Natural Opioid Peptides 35613.8 Vasoactive Peptides 35713.9 Erythropoietin and Thrombopoietin 35913.10 Others 36013.10.1 Adiponectin 36013.10.2 Islet amyloid polypeptide 360References 361Data Sheets 36214 Cytokines and Growth Factors 39314.1 Introduction 39314.2 Interleukin Families 39514.2.1 Interleukin-1 family 39514.2.2 Interleukin-2 family 39714.2.3 Interleukin-3 family 39714.2.4 Interleukin-4/interleukin-13 family 39814.2.5 Interleukin-5 family 39814.2.6 Interleukin-6 family 40014.2.7 Interleukin-7/interleukin-9 family 40214.2.8 Interleukin-10 family 40214.2.9 Interleukin-15 family 40414.2.10 Granulocyte–macrophage colony-stimulating factor family 40414.3 Tumour Necrosis Factor Family 40514.4 The Interferon Family 40614.4.1 The interferon a/b family 40614.4.2 The interferon g family 40814.5 Chemokines 40814.5.1 The intercrine a/chemokine CxC family 40914.5.2 The intercrine b/chemokine CC family 41014.6 The Platelet-Derived Growth Factor/Vascular Endothelial Growth Factor Family 41314.7 Transforming Growth Factor-b Family 41414.8 Miscellaneous 41514.8.1 The nerve growth factor-b family 41514.8.2 Epidermal growth factor 41714.8.3 Insulin-like growth factor binding proteins 418References 418Data Sheets 41915 Transport and Storage 45115.1 Introduction 45115.2 The Serum Albumin Family 45115.3 The Globin Family 45215.3.1 Haptoglobin 45615.4 Iron Transport and Storage 45715.4.1 Iron oxidation: ceruloplasmin 45715.4.2 Iron transport proteins: transferrins 45915.4.3 Iron storage 46215.4.4 Heme transport and iron recovery: hemopexin 46215.5 Transport of Hormones, Steroids and Vitamins 46315.5.1 Hormone transport proteins 46415.5.2 Steroid transport proteins 46715.5.3 Vitamin A transport protein 46715.6 Other Transport Proteins in Plasma 46815.6.1 Selenoproteins 468References 468Data Sheets 46916 Additional Proteins 48716.1 Introduction 48716.2 Additional Blood Plasma Proteins 487Data Sheets 492Appendix 503Glossary 515Index 521

"A major addition to the protein library, with an attractive and authoritative display of critical information about each plasma protein and a color schema of its three-dimensional structure from PDB." (The Quarterly Review of Biology, December 2009)