The X-linked lymphoproliferative syndrome: molecular and cellular basis of the disease

Thesis

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Titlepage

Contents

CHAPTER 1 X-Linked Lymphoproliferative Disease, A Progressive Immunodeficiency

Part 1. Structural characterization of the XLP gene SAP and its homologue EAT-2

CHAPTER 2 Characterization of SAP / SH2D1A missense mutations identified in X-linked lymphoproliferative disease patients

CHAPTER 3 Structural basis for the interaction of the free SH2 domain EAT-2 with SLAM receptors in hematopoietic cells

Part 2. Functional characterization of humoral defects in the XLP syndrome

CHAPTER 4 Alterations of the X-linked lymphoproliferative disease gene SAP/SH2D1A in the Common Variable Immunodeficiency syndrome.

CHAPTER 5 Signaling pathways involving the adapter SAP are essential for both T helper cell and B cell responses

CHAPTER 6 CONCLUSIONS

APPENDICES

Appendix 1 Summary

Appendix 2 Samenvatting

Appendix 3 ABBREVIATIONS

Appendix 4 ACKNOWLEDGEMENTS

Appendix 5 Curriculum Vitae

Appendix 6 BIBLIOGRAPHY

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