Paroxysmal nocturnal hemoglobinuria and the glycosylphosphatidylinositol-linked proteins:
Gespeichert in:
| Format: | Buch |
|---|---|
| Sprache: | English |
| Veröffentlicht: |
San Diego [u.a.]
Academic Press
2000
|
| Schlagworte: | |
| Online-Zugang: | Inhaltsverzeichnis |
| Beschreibung: | NT: PNH and the GPI-linked proteins |
| Beschreibung: | XII, 279 S. Ill., graph. Darst. |
| ISBN: | 0127729402 |
Internformat
MARC
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| 245 | 1 | 0 | |a Paroxysmal nocturnal hemoglobinuria and the glycosylphosphatidylinositol-linked proteins |c ed. by Neal S. Young ... |
| 246 | 1 | 3 | |a PNH and the GPI-linked proteins |
| 264 | 1 | |a San Diego [u.a.] |b Academic Press |c 2000 | |
| 300 | |a XII, 279 S. |b Ill., graph. Darst. | ||
| 336 | |b txt |2 rdacontent | ||
| 337 | |b n |2 rdamedia | ||
| 338 | |b nc |2 rdacarrier | ||
| 500 | |a NT: PNH and the GPI-linked proteins | ||
| 650 | 4 | |a Cellular signal transduction | |
| 650 | 4 | |a Glycoproteins | |
| 650 | 4 | |a Glycosylphosphatidylinositols | |
| 650 | 4 | |a Hemoglobinuria, Paroxysmal | |
| 650 | 4 | |a Membrane proteins | |
| 650 | 4 | |a Paroxysmal hemoglobinuria | |
| 650 | 4 | |a Phosphoinositides | |
| 650 | 0 | 7 | |a Paroxysmale nächtliche Hämoglobinurie |0 (DE-588)4609767-3 |2 gnd |9 rswk-swf |
| 650 | 0 | 7 | |a Glykoproteine |0 (DE-588)4071905-4 |2 gnd |9 rswk-swf |
| 689 | 0 | 0 | |a Paroxysmale nächtliche Hämoglobinurie |0 (DE-588)4609767-3 |D s |
| 689 | 0 | 1 | |a Glykoproteine |0 (DE-588)4071905-4 |D s |
| 689 | 0 | |5 DE-604 | |
| 700 | 1 | |a Young, Neal S. |e Sonstige |4 oth | |
| 856 | 4 | 2 | |m HBZ Datenaustausch |q application/pdf |u http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=009066207&sequence=000002&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA |3 Inhaltsverzeichnis |
| 999 | |a oai:aleph.bib-bvb.de:BVB01-009066207 | ||
Datensatz im Suchindex
| _version_ | 1804128066733080576 |
|---|---|
| adam_text | Contents
Contributors xi
Preface xiii
A Brief History of PNH
Wendell Rosse
The Diagnosis of PNH 8
Summary 15
References 16
2
Genetics of PNH
Lucio Luzzatto and Khedoudja Nafa
Biochemical Genetics of PNH in Somatic Cells 21
The PIG A Gene 23
v
The PIG A Protein 24
Detecting Mutations in the PIG A Gene 27
Germ Line PIG A Mutations 32
The Spectrum of Somatic Mutations in the PIG A
Gene 33
PIG A Mutations without PNH 40
Structure Function Relationships 41
Correlation between Phenotypes and Genotypes 42
Comparison of the Spectrum of Mutations in Three X Linked
Genes: PIG A, FVIII, and G6PD 42
Conclusion 43
References 44
3
Hemolysis in PNH
Charles J. Parker
Introduction 49
The Classical Pathway of Complement 55
The Alternative Pathway of Complement 61
Phenotypic Mosaicism 64
Functional Basis of the Abnormal Sensitivity of the
Erythrocytes of PNH to Complement Mediated
Lysis 68
Treatment of Hemolysis 83
Summary and Conclusions 86
References 89
4
Thrombotic Complications in PNH
Elaine M. Sloand and Neal S. Young
Introduction 101
Incidence of Thrombosis in PNH 102
Factors Associated with Venous Thrombosis 103
Platelet Abnormalities in PNH 104
Role of Red Cell and Platelet Microvesicles 107
Urokinase Activator Receptor (uPAR) and Cell Mediated
Fibrinolysis 108
Treatment of Thrombosis in PNH 109
Summary 109
References 110
5
Bone Marrow Failure in PNH
Daniel E. Dunn, Johnson M. Lin, and Neal S. Young
Introduction 113
Pathophysiology of Marrow Failure in PNH 115
An Immune Model of PNH Pathogenesis 120
Clinical Course and Treatment of Marrow Failure in
PNH 127
Summary 130
References 131
6
Animal Models of PNH
Taroh Kinoshita, Monica Bessler, and Junji Takeda
Introduction 139
Mouse Piga Gene 141
In Vitro Differentiation of PIGA( ) ES Cells 143
Chimeric Mice Bearing PIGA( ) Hematopoietic Stem
Cells 149
Hematopoiesis by Cells from Patients with PNH in SCID
Mice 154
Conclusions and Perspectives 155
References 155
7
The Function of GPI Anchored Proteins
Ian Okazaki and Joel Moss
Introduction 159
GPI Linked Proteins and Cell Signaling 161
(
Role of GPI Anchored Proteins in Insulin Action 166
GPI Anchored Proteins Function as Receptors 166
GPI Anchors and Membrane Structure 168
Summary 170
References 171
8
GPI in Lower Animals
Louis Schofield
Introduction 179 :
Structures of Protozoal GPIs 180
Evidence for GPI Mediated Signaling in Mammalian Cell
Biology 182
Signal Transduction in Host Cells by Protozoal
GPIs 185
Parasite GPIs as Functional Toxins and Pathogenicity
Factors 187
Regulation of Macrophage Function by Glycoconjugates of
Leishmania 188
Regulation of Host Cells by Ceramide Containing GPIs of
T. cruzi 189
Insulin Mimetic Activities of Protozoal GPIs 189
CD Id Restricted NK T Cells and Immunity to GPI Anchored
Proteins 190
Conclusions 191
References 192
9
Synthesis of the GPI Anchor
Daniel Sevlever, Rui Chen, and M. Edward Medof
Introduction 199
GPI Biosynthetic Pathway 200
Mammalian and Yeast GPI Anchor Defective
Mutants 203
GPI Anchor Addition to Proteins 207
Biological Implications of GPI Anchoring 210
Conclusions 213
References 214
10
Functional and Structural Organization
of GPI Anchored Proteins in
Cellular Membranes
Satyajit Mayor
Introduction 221
GPi Anchor Function and the Raft Hypothesis 221
Lateral Heterogeneity in Cell Membranes 222
Detergent Resistant Membranes and Their Connection with
Rafts 223
Evidence for Rafts in Living Cell Membranes 227
Involvement of Rafts in Biosynthetic and Endocytic
Sorting 230
Rafts and Signaling by GPIAPs 232
In Conclusion 233
References 233
11
Structure and Function of
GPI Specific Phospholipases
Martin G. Low
Introduction: GPI Anchors and Phospholipases 239
Biochemical Properties of Plasma GPI PLD 245
Distribution of GPI PLD in Tissues and Cells 252
Physiological Role of GPI PLD 257
References 262
Appendix: Sequence of the Coding Region
of the Human PIG A Gene 269
Index 271
|
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| id | DE-604.BV013298514 |
| illustrated | Illustrated |
| indexdate | 2024-07-09T18:43:20Z |
| institution | BVB |
| isbn | 0127729402 |
| language | English |
| oai_aleph_id | oai:aleph.bib-bvb.de:BVB01-009066207 |
| oclc_num | 43913628 |
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| physical | XII, 279 S. Ill., graph. Darst. |
| publishDate | 2000 |
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| publishDateSort | 2000 |
| publisher | Academic Press |
| record_format | marc |
| spelling | Paroxysmal nocturnal hemoglobinuria and the glycosylphosphatidylinositol-linked proteins ed. by Neal S. Young ... PNH and the GPI-linked proteins San Diego [u.a.] Academic Press 2000 XII, 279 S. Ill., graph. Darst. txt rdacontent n rdamedia nc rdacarrier NT: PNH and the GPI-linked proteins Cellular signal transduction Glycoproteins Glycosylphosphatidylinositols Hemoglobinuria, Paroxysmal Membrane proteins Paroxysmal hemoglobinuria Phosphoinositides Paroxysmale nächtliche Hämoglobinurie (DE-588)4609767-3 gnd rswk-swf Glykoproteine (DE-588)4071905-4 gnd rswk-swf Paroxysmale nächtliche Hämoglobinurie (DE-588)4609767-3 s Glykoproteine (DE-588)4071905-4 s DE-604 Young, Neal S. Sonstige oth HBZ Datenaustausch application/pdf http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=009066207&sequence=000002&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA Inhaltsverzeichnis |
| spellingShingle | Paroxysmal nocturnal hemoglobinuria and the glycosylphosphatidylinositol-linked proteins Cellular signal transduction Glycoproteins Glycosylphosphatidylinositols Hemoglobinuria, Paroxysmal Membrane proteins Paroxysmal hemoglobinuria Phosphoinositides Paroxysmale nächtliche Hämoglobinurie (DE-588)4609767-3 gnd Glykoproteine (DE-588)4071905-4 gnd |
| subject_GND | (DE-588)4609767-3 (DE-588)4071905-4 |
| title | Paroxysmal nocturnal hemoglobinuria and the glycosylphosphatidylinositol-linked proteins |
| title_alt | PNH and the GPI-linked proteins |
| title_auth | Paroxysmal nocturnal hemoglobinuria and the glycosylphosphatidylinositol-linked proteins |
| title_exact_search | Paroxysmal nocturnal hemoglobinuria and the glycosylphosphatidylinositol-linked proteins |
| title_full | Paroxysmal nocturnal hemoglobinuria and the glycosylphosphatidylinositol-linked proteins ed. by Neal S. Young ... |
| title_fullStr | Paroxysmal nocturnal hemoglobinuria and the glycosylphosphatidylinositol-linked proteins ed. by Neal S. Young ... |
| title_full_unstemmed | Paroxysmal nocturnal hemoglobinuria and the glycosylphosphatidylinositol-linked proteins ed. by Neal S. Young ... |
| title_short | Paroxysmal nocturnal hemoglobinuria and the glycosylphosphatidylinositol-linked proteins |
| title_sort | paroxysmal nocturnal hemoglobinuria and the glycosylphosphatidylinositol linked proteins |
| topic | Cellular signal transduction Glycoproteins Glycosylphosphatidylinositols Hemoglobinuria, Paroxysmal Membrane proteins Paroxysmal hemoglobinuria Phosphoinositides Paroxysmale nächtliche Hämoglobinurie (DE-588)4609767-3 gnd Glykoproteine (DE-588)4071905-4 gnd |
| topic_facet | Cellular signal transduction Glycoproteins Glycosylphosphatidylinositols Hemoglobinuria, Paroxysmal Membrane proteins Paroxysmal hemoglobinuria Phosphoinositides Paroxysmale nächtliche Hämoglobinurie Glykoproteine |
| url | http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=009066207&sequence=000002&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA |
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