Separation of the acetylcholinesterase-deficient red cells in paroxysmal nocturnal hemoglobinuria

Chow, FL; Hall, SE; Rosse, WF; Telen, Marilyn J.

doi:10.1182/blood.v67.4.893.bloodjournal674893

Cited by 19 publications

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“…Erythrocytes from PNH patients were obtained and separated into populations of type I (normal) and type III (markedly sensitive to complement) cells as previously described, using cobra-venom lysis of type III cells to obtain type I cells (9) and anti -acetylcholinesterase affinity chromatography to isolate type III cells (10) . None of the patients studied had appreciable numbers of type II cells (with intermediate sensitivity to complement).…”

Section: Telen Et Al Brief Definitive Reportmentioning

confidence: 99%

Identification of human erythrocyte blood group antigens on decay- accelerating factor (DAF) and an erythrocyte phenotype negative for daf

Telen¹,

Hall²,

Green³

et al. 1988

The Journal of Experimental Medicine

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147

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Decay accelerating factor (DAF) is a glycoprotein present on the surfaces of many types ofcells in contact with plasma, including erythrocytes, leukocytes, and platelets (reviewed in reference 1). A small amount ofDAF is also present in serum. Numerous investigators have demonstrated that DAF inhibits the action of C3 convertases on cell surfaces, and its absence has been shown to be at least partially responsible for the abnormal sensitivity to lysis by complement exhibited by erythrocytes of patients with the acquired stem cell disorder paroxysmal nocturnal hemoglobinuria (PNH) (2). Hereditary absence of DAF has not been previously described .Tca and Cra are high-frequency human erythrocyte antigens . These antigens are part of a family of blood group antigens, designated Cromer related, which are all absent from the null phenotype cell IFC -, or Inab (3). Recently, Spring and colleagues (4) have identified two monoclonal antibodies which bound to high frequency red cell antigens absent from the Inab phenotype. They also demonstrated that these antibodies, as well as several human antisera to Cromer-related antigens, bound to a 70-kD glycoprotein when used to stain immunoblots of human erythrocyte membrane proteins . Because the wide tissue distribution of mAb reactivity, along with some of the biochemical characterization and immunoblotting data, was similar to that of DAF, we investigated whether the Cromer-related antigens Cra and Tca resided on the DAF molecule . Volume 167 June 1988 1993-1998 Materials and Methods Brief Definitive Report DAF was purified from random donor human erythrocytes by the method of NicholsonWeller (5), as modified by Sugita et al . (6). Resultant material was homogeneous as analyzed by SDS-PAGE and silver staining, and C3 convertase inhibitory activity was intact, as measured by the ability of purified DAF to inhibit lysis of sheep erythrocytes sensitized with antibody and bearing human complement components 1 and 4b (EACI, 4b) (5).

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Section: Telen Et Al Brief Definitive Reportmentioning

confidence: 99%

Identification of human erythrocyte blood group antigens on decay- accelerating factor (DAF) and an erythrocyte phenotype negative for daf

Telen¹,

Hall²,

Green³

et al. 1988

The Journal of Experimental Medicine

Self Cite

147

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Paroxysmal nocturnal hemoglobinuria due to hereditary nucleotide deletion in the HRF20 (CD59) gene

et al. 1992

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HRF20 (CD59) is a membrane glycoprotein which protects cells from the membrane attack reaction of homologous complement. A patient who is completely deficient in HRF20 expression and is suffering from paroxysmal nocturnal hemoglobinuria (PNH) was studied. His parents are cousins and both have decreased HRF20 expression, suggesting that the deficiency is genetic. We established a cultured cell line (NCU1) which is HRF20 deficient from the patient's lymphocytes by Epstein-Barr-virus (EBV) infection. Northern blot analysis revealed HRF20 mRNA signals, indicating that HRF20 mRNA were transcribed. HRF20 cDNA was amplified by the polymerase chain reaction (PCR) method. Sequencing of the cDNA from the NCU1 showed two single-base deletions at amino acid 16 and 96 from the N terminus of the mature protein. Deletion in the genomic DNA of peripheral blood lymphocytes was confirmed by the DNA sequence of an HRF20 open reading frame containing amino acid 16. Furthermore, the patient's parents and sister possessed both intact and deleted genomic HRF20 DNA while his brother's DNA was intact. These findings demonstrate that the HRF20 deficiency was genomic in origin, and that complete deletion was brought about by a homozygous abnormality in the HRF20 gene. The base deletion caused a codon frame shift resulting in failure to produce intact HRF20 protein in the patient.

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Expression of Human Erythrocyte Membrane Protein Antigens during Erythroid Differentiation

Telen

1990

Blood Cell Biochemistry

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Separation of the acetylcholinesterase-deficient red cells in paroxysmal nocturnal hemoglobinuria

Cited by 19 publications

References 0 publications

Identification of human erythrocyte blood group antigens on decay- accelerating factor (DAF) and an erythrocyte phenotype negative for daf

Identification of human erythrocyte blood group antigens on decay- accelerating factor (DAF) and an erythrocyte phenotype negative for daf

Paroxysmal nocturnal hemoglobinuria due to hereditary nucleotide deletion in the HRF20 (CD59) gene

Expression of Human Erythrocyte Membrane Protein Antigens during Erythroid Differentiation

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