Science 26 February 1993: Vol. 259. no. 5099, pp. 1318 - 1320 DOI: 10.1126/science.7680492

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Articles

How I treat paroxysmal nocturnal hemoglobinuria.

R. A. Brodsky (2009)
Blood 113, 6522-6527
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Narrative Review: Paroxysmal Nocturnal Hemoglobinuria: The Physiology of Complement-Related Hemolytic Anemia.

R. A. Brodsky (2008)
Ann Intern Med 148, 587-595
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Paroxysmal Nocturnal Hemoglobinuria: An Historical Overview.

C. J. Parker (2008)
Hematology 2008, 93-103
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The Pathophysiology of Disease in Patients with Paroxysmal Nocturnal Hemoglobinuria.

M. Bessler and J. Hiken (2008)
Hematology 2008, 104-110
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Changes in molecular species profiles of glycosylphosphatidylinositol anchor precursors in early stages of biosynthesis.

T. Houjou, J. Hayakawa, R. Watanabe, Y. Tashima, Y. Maeda, T. Kinoshita, and R. Taguchi (2007)
J. Lipid Res. 48, 1599-1606
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Thematic review series: Lipid Posttranslational Modifications. GPI anchoring of protein in yeast and mammalian cells, or: how we learned to stop worrying and love glycophospholipids.

P. Orlean and A. K. Menon (2007)
J. Lipid Res. 48, 993-1011
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Inducible and Reversible Transgene Expression in Human Stem Cells After Efficient and Stable Gene Transfer.

B. Y. Zhou, Z. Ye, G. Chen, Z. P. Gao, Y. A. Zhang, and L. Cheng (2007)
Stem Cells 25, 779-789
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Molecular basis of clonal expansion of hematopoiesis in 2 patients with paroxysmal nocturnal hemoglobinuria (PNH).

N. Inoue, T. Izui-Sarumaru, Y. Murakami, Y. Endo, J.-I. Nishimura, K. Kurokawa, M. Kuwayama, H. Shime, T. Machii, Y. Kanakura, et al. (2006)
Blood 108, 4232-4236
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The mutation rate in PIG-A is normal in patients with paroxysmal nocturnal hemoglobinuria (PNH).

D. J. Araten and L. Luzzatto (2006)
Blood 108, 734-736
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GUP1 of Saccharomyces cerevisiae Encodes an O-Acyltransferase Involved in Remodeling of the GPI Anchor.

R. Bosson, M. Jaquenoud, and A. Conzelmann (2006)
Mol. Biol. Cell 17, 2636-2645
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New Insights into Paroxysmal Nocturnal Hemoglobinuria.

R. A. Brodsky (2006)
Hematology 2006, 24-28
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Gpi19, the Saccharomyces cerevisiae Homologue of Mammalian PIG-P, Is a Subunit of the Initial Enzyme for Glycosylphosphatidylinositol Anchor Biosynthesis.

H. A. Newman, M. J. Romeo, S. E. Lewis, B. C. Yan, P. Orlean, and D. E. Levin (2005)
Eukaryot. Cell 4, 1801-1807
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A Quantitative Measurement of the Human Somatic Mutation Rate.

D. J. Araten, D. W. Golde, R. H. Zhang, H. T. Thaler, L. Gargiulo, R. Notaro, and L. Luzzatto (2005)
Cancer Res. 65, 8111-8117
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Accelerated telomere shortening in glycosylphosphatidylinositol (GPI)-negative compared with GPI-positive granulocytes from patients with paroxysmal nocturnal hemoglobinuria (PNH) detected by proaerolysin flow-FISH.

F. Beier, S. Balabanov, T. Buckley, K. Dietz, U. Hartmann, M. Rojewski, L. Kanz, H. Schrezenmeier, and T. H. Brummendorf (2005)
Blood 106, 531-533
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PIG-A mutations in normal hematopoiesis.

R. Hu, G. L. Mukhina, S. Piantadosi, J. P. Barber, R. J. Jones, and R. A. Brodsky (2005)
Blood 105, 3848-3854
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GPI-anchor deficiency in myeloid cells causes impaired Fc{gamma}R effector functions.

W. L. W. Hazenbos, B. E. Clausen, J. Takeda, and T. Kinoshita (2004)
Blood 104, 2825-2831
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Enhanced Responses of Glycosylphosphatidylinositol Anchor-Deficient T Lymphocytes.

W. L. W. Hazenbos, Y. Murakami, J.-i. Nishimura, J. Takeda, and T. Kinoshita (2004)
J. Immunol. 173, 3810-3815
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GPI-defective monocytes from paroxysmal nocturnal hemoglobinuria patients show impaired in vitro dendritic cell differentiation.

G. Ruggiero, G. Terrazzano, C. Becchimanzi, M. Sica, C. Andretta, A. M. Masci, L. Racioppi, B. Rotoli, S. Zappacosta, and F. Alfinito (2004)
J. Leukoc. Biol. 76, 634-640
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In vitro and in vivo evidence of PNH cell sensitivity to immune attack after nonmyeloablative allogeneic hematopoietic cell transplantation.

Y. Takahashi, J. P. McCoy Jr, C. Carvallo, C. Rivera, T. Igarashi, R. Srinivasan, N. S. Young, and R. W. Childs (2004)
Blood 103, 1383-1390
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Immune-Mediated Hemolytic Anemia.

W. F. Rosse, P. Hillmen, and A. D. Schreiber (2004)
Hematology 2004, 48-62
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SETH1 and SETH2, Two Components of the Glycosylphosphatidylinositol Anchor Biosynthetic Pathway, Are Required for Pollen Germination and Tube Growth in Arabidopsis.

E. Lalanne, D. Honys, A. Johnson, G. H. H. Borner, K. S. Lilley, P. Dupree, U. Grossniklaus, and D. Twell (2004)
PLANT CELL 16, 229-240
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Glycosyl Phosphatidylinositol-Anchored Proteins in Chemosensory Signaling: Antisense Manipulation of Paramecium tetraurelia PIG-A Gene Expression.

J. Yano, V. Rachochy, and J. L. Van Houten (2003)
Eukaryot. Cell 2, 1211-1219
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Primary prophylaxis with warfarin prevents thrombosis in paroxysmal nocturnal hemoglobinuria (PNH).

C. Hall, S. Richards, and P. Hillmen (2003)
Blood 102, 3587-3591
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Severe telomere shortening in patients with paroxysmal nocturnal hemoglobinuria affects both GPI- and GPI+ hematopoiesis.

A. Karadimitris, D. J. Araten, L. Luzzatto, and R. Notaro (2003)
Blood 102, 514-516
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The spectrum of PIG-A gene mutations in aplastic anemia/paroxysmal nocturnal hemoglobinuria (AA/PNH): a high incidence of multiple mutations and evidence of a mutational hot spot.

Y. Mortazavi, B. Merk, J. McIntosh, J. C. W. Marsh, H. Schrezenmeier, and T. R. Rutherford (2003)
Blood 101, 2833-2841
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The effect of GPI-anchor deficiency on apoptosis in mice carrying a Piga gene mutation in hematopoietic cells.

S. Kulkarni and M. Bessler (2002)
J. Leukoc. Biol. 72, 1228-1233
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Inefficient response of T lymphocytes to glycosylphosphatidylinositol anchor-negative cells: implications for paroxysmal nocturnal hemoglobinuria.

Y. Murakami, H. Kosaka, Y. Maeda, J.-i. Nishimura, N. Inoue, K. Ohishi, M. Okabe, J. Takeda, and T. Kinoshita (2002)
Blood 100, 4116-4122
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Using the P{wHy} Hybrid Transposable Element to Disrupt Genes in Region 54D-55B in Drosophila melanogaster.

S. E. Mohr and W. M. Gelbart (2002)
Genetics 162, 165-176
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Genes for Glycosylphosphatidylinositol Toxin Biosynthesis in Plasmodium falciparum.

M. Delorenzi, A. Sexton, H. Shams-Eldin, R. T. Schwarz, T. Speed, and L. Schofield (2002)
Infect. Immun. 70, 4510-4522
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Decreased susceptibility of leukemic cells with PIG-A mutation to natural killer cells in vitro.

S. Nagakura, S. Ishihara, D. E. Dunn, J.-i. Nishimura, T. Kawaguchi, K. Horikawa, M. Hidaka, T. Kagimoto, N. Eto, H. Mitsuya, et al. (2002)
Blood 100, 1031-1037
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Paroxysmal nocturnal haemoglobinuria: Nature's gene therapy?.

R J Johnson and P Hillmen (2002)
Mol. Pathol. 55, 145-152
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Mutation analysis of the PIG-A gene in Korean patients with paroxysmal nocturnal haemoglobinuria.

J H Yoon, H I Cho, S S Park, Y H Chang, and B K Kim (2002)
J. Clin. Pathol. 55, 410-413
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Long-term support of hematopoiesis by a single stem cell clone in patients with paroxysmal nocturnal hemoglobinuria.

J.-i. Nishimura, T. Hirota, Y. Kanakura, T. Machii, T. Kageyama, S. Doi, H. Wada, T. Masaoka, Y. Kanayama, H. Fujii, et al. (2002)
Blood 99, 2748-2751
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GATA1-Cre mediates Piga gene inactivation in the erythroid/megakaryocytic lineage and leads to circulating red cells with a partial deficiency in glycosyl phosphatidylinositol-linked proteins (paroxysmal nocturnal hemoglobinuria type II cells).

M. Jasinski, P. Keller, Y. Fujiwara, S. H. Orkin, and M. Bessler (2001)
Blood 98, 2248-2255
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Efficient retrovirus-mediated PIG-A gene transfer and stable restoration of GPI-anchored protein expression in cells with the PNH phenotype.

J.-i. Nishimura, K. L. Phillips, R. E. Ware, S. Hall, L. Wilson, T. L. Gentry, T. A. Howard, Y. Murakami, M. Shibano, T. Machii, et al. (2001)
Blood 97, 3004-3010
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Glycophosphatidylinositol-anchored Protein Deficiency as a Marker of Mutator Phenotypes in Cancer.

R. Chen, J. R. Eshleman, R. A. Brodsky, and M. E. Medof (2001)
Cancer Res. 61, 654-658
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Cloning of murine glycosyl phosphatidylinositol anchor attachment protein, GPAA1.

Y. Hiroi, R. Chen, H. Sawa, T. Hosoda, S. Kudoh, Y. Kobayashi, H. Aburatani, K. Nagashima, R. Nagai, Y. Yazaki, et al. (2000)
Am J Physiol Cell Physiol 279, C205-C212
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Mapping of a syndrome of X-linked thrombocytopenia with thalassemia to band Xp11-12: further evidence of genetic heterogeneity of X-linked thrombocytopenia.

W. H. Raskind, K. K. Niakan, J. Wolff, M. Matsushita, T. Vaughan, G. Stamatoyannopoulos, C. Watanabe, J. Rios, and H. D. Ochs (2000)
Blood 95, 2262-2268
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New Insights into the Pathophysiology of Acquired Cytopenias.

N. S. Young, J. L. Abkowitz, and L. Luzzatto (2000)
Hematology 2000, 18-38
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Paroxysmal Murine Hemoglobinuria(?): A Model for Human PNH.

L. Luzzatto (1999)
Blood 94, 2941-2944
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Increased Sensitivity to Complement and a Decreased Red Blood Cell Life Span in Mice Mosaic for a Nonfunctional Piga Gene.

G. Tremml, C. Dominguez, V. Rosti, Z. Zhang, P.P. Pandolfi, P. Keller, and M. Bessler (1999)
Blood 94, 2945-2954
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Different Roles of Glycosylphosphatidylinositol in Various Hematopoietic Cells as Revealed by a Mouse Model of Paroxysmal Nocturnal Hemoglobinuria.

Y. Murakami, T. Kinoshita, Y. Maeda, T. Nakano, H. Kosaka, and J. Takeda (1999)
Blood 94, 2963-2970
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Immobilization of glycosylphosphatidylinositol-anchored proteins inhibits T cell growth but not function.

M. D. Marmor, M. F. Bachmann, P. S. Ohashi, T. R. Malek, and M. Julius (1999)
Int. Immunol. 11, 1381-1393
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GPI1 Stabilizes an Enzyme Essential in the First Step of Glycosylphosphatidylinositol Biosynthesis.

Y. Hong, K. Ohishi, R. Watanabe, Y. Endo, Y. Maeda, and T. Kinoshita (1999)
J. Biol. Chem. 274, 18582-18588
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X inactivation and somatic cell selection rescue female mice carrying a Piga-null mutation.

P. Keller, G. Tremml, V. Rosti, and M. Bessler (1999)
PNAS 96, 7479-7483
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Deletion of GPI7, a Yeast Gene Required for Addition of a Side Chain to the Glycosylphosphatidylinositol (GPI) Core Structure, Affects GPI Protein Transport, Remodeling, and Cell Wall Integrity.

A. Benachour, G. Sipos, I. Flury, F. Reggiori, E. Canivenc-Gansel, C. Vionnet, A. Conzelmann, and M. Benghezal (1999)
J. Biol. Chem. 274, 15251-15261
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Resistance of Paroxysmal Nocturnal Hemoglobinuria Cells to the Glycosylphosphatidylinositol-Binding Toxin Aerolysin.

R. A. Brodsky, G. L. Mukhina, K. L. Nelson, T. S. Lawrence, R. J. Jones, and J. T. Buckley (1999)
Blood 93, 1749-1756
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The PIG-A Mutation and Absence of Glycosylphosphatidylinositol-Linked Proteins Do Not Confer Resistance to Apoptosis in Paroxysmal Nocturnal Hemoglobinuria.

R. E. Ware, J.-i. Nishimura, M. A. Moody, C. Smith, W. F. Rosse, and T. A. Howard (1998)
Blood 92, 2541-2550
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Circulating Primitive Stem Cells in Paroxysmal Nocturnal Hemoglobinuria (PNH) Are Predominantly Normal in Phenotype But Granulocyte Colony-Stimulating Factor Treatment Mobilizes Mainly PNH Stem Cells.

R. J. Johnson, A. C. Rawstron, S. Richards, G. J. Morgan, D. R. Norfolk, and S. O'C. a. P. Hillmen (1998)
Blood 91, 4504-4508
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The Glycosylphosphatidylinositol-Anchored Form and the Transmembrane Form of CD58 Associate with Protein Kinases.

D. Itzhaky, N. Raz, and N. Hollander (1998)
J. Immunol. 160, 4361-4366
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The affected gene underlying the class K glycosylphosphatidylinositol (GPI) surface protein defect codes for the GPI transamidase.

J. Yu, S. Nagarajan, J. J. Knez, S. Udenfriend, R. Chen, and M. E. Medof (1997)
PNAS 94, 12580-12585
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Structure, Function and Expression on Blood and Bone Marrow Cells of the Urokinase-Type Plasminogen Activator Receptor, uPAR.

T. Plesner, N. Behrendt, and M. Ploug (1997)
Stem Cells 15, 398-408
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Resistance to apoptosis caused by PIG-A gene mutations in paroxysmal nocturnal hemoglobinuria.

R. A. Brodsky, M. S. Vala, J. P. Barber, M. E. Medof, and R. J. Jones (1997)
PNAS 94, 8756-8760
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Tissue-specific knockout of the mouse Pig-a gene reveals important roles for GPI-anchored proteins in skin development.

M. Tarutani, S. Itami, M. Okabe, M. Ikawa, T. Tezuka, K. Yoshikawa, T. Kinoshita, and J. Takeda (1997)
PNAS 94, 7400-7405
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Relationship Between the Phenotypes of Circulating Erythrocytes and Cultured Erythroblasts in Paroxysmal Nocturnal Hemoglobinuria.

T. Shichishima, Y. Saitoh, T. Terasawa, K. Ogawa, and Y. Maruyama (1997)
Blood 90, 435-443
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Expression Cloning of PIG-L, a Candidate N-Acetylglucosaminyl-phosphatidylinositol Deacetylase.

N. Nakamura, N. Inoue, R. Watanabe, M. Takahashi, J. Takeda, V. L. Stevens, and T. Kinoshita (1997)
J. Biol. Chem. 272, 15834-15840
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A Patient With Paroxysmal Nocturnal Hemoglobinuria Bearing Four Independent PIG-A Mutant Clones.

J.-i. Nishimura, N. Inoue, H. Wada, E. Ueda, P. Pramoonjago, T. Hirota, T. Machii, T. Kageyama, A. Kanamaru, J. Takeda, et al. (1997)
Blood 89, 3470-3476
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Gpi1, a Saccharomyces cerevisiae Protein That Participates in the First Step in Glycosylphosphatidylinositol Anchor Synthesis.

S. D. Leidich and P. Orlean (1996)
J. Biol. Chem. 271, 27829-27837
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PIG-A and PIG-H, Which Participate in Glycosylphosphatidylinositol Anchor Biosynthesis, Form a Protein Complex in the Endoplasmic Reticulum.

R. Watanabe, T. Kinoshita, R. Masaki, A. Yamamoto, J. Takeda, and N. Inoue (1996)
J. Biol. Chem. 271, 26868-26875
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Carboxyl-terminal Targeting and Novel Post-translational Processing of JAW1, a Lymphoid Protein of the Endoplasmic Reticulum.

T. W. Behrens, G. M. Kearns, J. J. Rivard, H. D. Bernstein, J. W. Yewdell, and L. M. Staudt (1996)
J. Biol. Chem. 271, 23528-23534
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Natural History of Paroxysmal Nocturnal Hemoglobinuria.

P. Hillmen, S.M. Lewis, M. Bessler, L. Luzzatto, and J. V. Dacie (1995)
N. Engl. J. Med. 333, 1253-1259
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Temperature-sensitive Yeast GPI Anchoring Mutants gpi2 and gpi3 Are Defective in the Synthesis of N-Acetylglucosaminyl Phosphatidylinositol.

S. D. Leidich, Z. Kostova, R. R. Latek, L. C. Costello, D. A. Drapp, W. Gray, J. S. Fassler, and P. Orlean (1995)
J. Biol. Chem. 270, 13029-13035
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Protein lipidation in cell signaling.

P. Casey (1995)
Science 268, 221-225
 |  Abstract »  |  PDF »

Abnormalities of PIG-A Transcripts in Granulocytes from Patients with Paroxysmal Nocturnal Hemoglobinuria.

T. Miyata, N. Yamada, Y. Iida, J. Nishimura, J. Takeda, T. Kitani, and T. Kinoshita (1994)
N. Engl. J. Med. 330, 249-255
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