Blood Cells, Molecules, and Diseases

article

Commentary

Yves Najean

Blood Cells, Molecules and Diseases > 1997 > 23 > 1 > i

article

Molecular Genetics of Glucose-6-Phosphate Dehydrogenase Deficiency in Mexico

Maria Dolores Medina, Gerardo Vaca, Beatriz Lopez-Guido, Beryl Westwood, more

Blood Cells, Molecules and Diseases > 1997 > 23 > 1 > 88-94

ABSTRACT: Several studies carried out between 1965 and 1985 showed that G-6-PD deficiency in Mexico is heterogeneous at the biochemical level and that the G-6-PD A- phenotype is relatively common. We have now investigated the molecular basis of G-6-PD deficiency in Mexico. Up-to-date 60 chromosomes with G6PD mutations have been studied, 16 in previous studies and 44 in the present work. Molecular...

article

Hematologically Important Mutations: Glanzmann Thrombasthenia

Deborah L. French, Barry S. Coller

Blood Cells, Molecules and Diseases > 1997 > 23 > 1 > 39-51

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Cationic Phosphonolipids as non Viral Vectors for DNA Transfection in Hematopoietic Cell Lines and CD34+ Cells

Virginie Floch, Gwenaëlle Le Bolc'h, Marie-Pierre Audrézet, Jean-Jacques Yaouanc, more

Blood Cells, Molecules and Diseases > 1997 > 23 > 1 > 69-87

ABSTRACT: The ability to transfer genes into a hematopoietic stem cell and to achieve regulation of their expression in lymphoid or myeloid lineages should open many new therapeutic opportunities. Besides gene transfer mediated by virus vectors like retrovirus or adenovirus, non viral systems have the theoretical advantage of being safe and easy to manage. We developed a new family of cationic lipids...

article

Red Cell-Mediated Therapy: Opportunities and Challenges

Allen Krantz

Blood Cells, Molecules and Diseases > 1997 > 23 > 1 > 58-68

ABSTRACT: Red blood cells have unique properties that offer attractive therapeutic possibilities. It is proposed that by covalently linking drug pharmacophores to red cell surface proteins, significant enhancement of pharmacokinetics can be achieved. Specific anchoring to erythrocytes can be accomplished in principle, using unique, affinity labeling, combinatorial libraries targeting the surface protein,...

article

T-cell Receptor β Chain Variability in Bone Marrow and Peripheral Blood in Severe Acquired Aplastic Anemia

Chantal Y. Manz, Pierre-Yves Dietrich, Valérie Schnuriger, Catherine Nissen, more

Blood Cells, Molecules and Diseases > 1997 > 23 > 1 > 110-122

ABSTRACT: Aplastic anemia (AA) is characterized by multilineage bone marrow failure of unknown etiology. In order to assess the role of immune-mediated mechanisms in hematopoietic suppression, we examined the diversity of T lymphocyte repertoire in terms of variable (V) gene segment usage of the T cell receptor (TCR) β chain in bone marrow and peripheral blood of six patients with severe untreated...

article

Commentary

Virgil F. Fairbanks

Blood Cells, Molecules and Diseases > 1997 > 23 > 1 > ii-iii

article

Evidence for an Uncommon Microsatellite Instability on Mouse Chromosomes 2 and 4 and its Possible Role in Radiation Leukemogenesis

K. Noy Rithidech, John J. Dunn, Chris R. Gordon, Eugene P. Cronkite, more

Blood Cells, Molecules and Diseases > 1997 > 23 > 1 > 99-109

ABSTRACT: Although microsatellite instability (MSI), usually detected by DNA length polymorphisms, has been implicated in the induction of solid tumors in both humans and animals, its role in leukemogenesis is unclear. The goal of this study was to investigate whether there is an association between MSI and radiation leukemogenesis in CBA/Ca mice. Microsatellite lengths at 55 loci, mapped to eight...

article

Commentary

Ernest Beutler, Carol West, Terri Gelbart, Vincent J. Felitti

Blood Cells, Molecules and Diseases > 1997 > 23 > 1 > A145-A146

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HLA-H Mutations in the Ashkenazi Jewish Population

Ernest Beutler, Terri Gelbart

Blood Cells, Molecules and Diseases > 1997 > 23 > 1 > 95-98

ABSTRACT: Hereditary hemochromatosis is a common disorder in people of European origin. The HLA-H gene has been found to have two mutations that apparently cause hemochromatosis. The principal mutation, 845G->A (C282Y), is believed to have arisen relatively recently in the Celtic population. To determine the incidence of this mutation and the other hemochromatosis-associated mutation, 187C->G...

article

A Hemophilia Model in Zebrafish: Analysis of Hemostasis

Pudur Jagadeeswaran, Yuan C. Liu

Blood Cells, Molecules and Diseases > 1997 > 23 > 1 > 52-57

ABSTRACT: We have developed artificial hemophilia in zebrafish by treating them with copper and measured their clotting function by a newly developed sensitive clotting time assay. The clotting function can be detected rapidly and reliably in 30 hr larvae and in adult fish by measuring the blood clotting time. We have used this assay to screen wild type zebrafish and identified fish with prolonged...

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Expression of Transcription Factors During Sodium Phenylacetate Induced Erythroid Differentiation in K562 Cells

Armin V. Rath, Gerd E. Schmahl, Charlotte M. Niemeyer

Blood Cells, Molecules and Diseases > 1997 > 23 > 1 > 27-38

ABSTRACT: During 15 days of treatment of K562 cells with sodium phenylacetate, we observed an increase in the cellular hemoglobin concentration with a similar increase in the expression of γ-globin mRNA. Morphological studies demonstrated characteristic features of erythroid differentiation and maturation. At the same time there was no change in the level of expression of the cell surface antigenes...

article

Hematologically Important Mutations: Gaucher Disease

Ernest Beutler, Terri Gelbart

Blood Cells, Molecules and Diseases > 1997 > 23 > 1 > 2-7

article

EDITORIAL

Blood Cells, Molecules and Diseases > 1997 > 23 > 1 > 1

article

Genetic and Clinical Description of Hemochromatosis Probands and Heterozygotes: Evidence That Multiple Genes Linked to the Major Histocompatibility Complex Are Responsible for Hemochromatosis

James C. Barton, Wendy W. H. Shih, Ritsuko Sawada-Hirai, Ronald T. Acton, more

Blood Cells, Molecules and Diseases > 1997 > 23 > 1 > 135-145

ABSTRACT: We evaluated Alabama hemochromatosis probands (n= 74) and normal control subjects (n= 142) for expression of the hemochromatosis-associated mutations nt 845G->A (845A; Cys282Tyr) and nt 187C->G (His63Asp) in a gene linked to the major histocompatibility complex (MHC). We also tabulated parameters of iron metabolism and iron overload in probands and in obligate heterozygote family members...

article

Modulatory Subdomains of the HS2 Enhancer Differentially Regulate Enhancer Activity in Erythroid Cells at Different Developmental Stages

Resy Cavallesco, Dorothy Tuan

Blood Cells, Molecules and Diseases > 1997 > 23 > 1 > 8-26

ABSTRACT: The HS2 enhancer in the locus control region of human β-like globin genes displays developmental-stage-independent enhancer function. The mechanism by which it regulates the transcription of the globin genes in erythroid cells throughout development is not fully understood. In this paper we dissect the HS2 enhancer into an enhancer core and five modulatory subdomains M1 to M5. The enhancer...

article

Crystal Structure of NMC-4 Fab anti-von Willebrand Factor A1 Domain

Reha Celikel, Madhusudan, Kottayil I. Varughese, Midori Shima, more

Blood Cells, Molecules and Diseases > 1997 > 23 > 1 > 123-134

ABSTRACT: We have solved the crystal structure of the Fab fragment of NMC-4, a mouse monoclonal antibody that binds to the A1 domain of von Willebrand factor (vWF). Two Asp and three Tyr residues in the complementarity determining regions 1 and 3 of the heavy chain exhibited a spatial orientation suggestive of a dominant role in establishing contact with the antigen. A cluster of Asp and Tyr residues...

INFONA - science communication portal

Blood Cells, Molecules, and Diseases

Commentary

Molecular Genetics of Glucose-6-Phosphate Dehydrogenase Deficiency in Mexico

Hematologically Important Mutations: Glanzmann Thrombasthenia

Cationic Phosphonolipids as non Viral Vectors for DNA Transfection in Hematopoietic Cell Lines and CD34+ Cells

Red Cell-Mediated Therapy: Opportunities and Challenges

T-cell Receptor β Chain Variability in Bone Marrow and Peripheral Blood in Severe Acquired Aplastic Anemia

Commentary

Evidence for an Uncommon Microsatellite Instability on Mouse Chromosomes 2 and 4 and its Possible Role in Radiation Leukemogenesis

Commentary

HLA-H Mutations in the Ashkenazi Jewish Population

A Hemophilia Model in Zebrafish: Analysis of Hemostasis

Expression of Transcription Factors During Sodium Phenylacetate Induced Erythroid Differentiation in K562 Cells

Hematologically Important Mutations: Gaucher Disease

EDITORIAL

Genetic and Clinical Description of Hemochromatosis Probands and Heterozygotes: Evidence That Multiple Genes Linked to the Major Histocompatibility Complex Are Responsible for Hemochromatosis

Modulatory Subdomains of the HS2 Enhancer Differentially Regulate Enhancer Activity in Erythroid Cells at Different Developmental Stages

Crystal Structure of NMC-4 Fab anti-von Willebrand Factor A1 Domain

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