An Inherited RBC Characteristic, NOR, Resulting in Erythrocyte Polyagglutination

Harris, Philip A.; Roman, G.K.; Moulds, John J.; Bird, Golding; Shah, Nishith

doi:10.1111/j.1423-0410.1982.tb01083.x

Cited by 36 publications

(19 citation statements)

References 16 publications

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“…Recently a second case of NOR polyagglutination has been identified in a two-generation Polish family (3). We found that polyagglutination of NOR erythrocytes, besides having the features described by Harris et al (2), was enhanced by sialidase and abolished by ␣-galactosidase treatment. Several lines of evidence were obtained indicating that NOR erythrocytes possessed unique neutral glycosphingolipids.…”

supporting

confidence: 67%

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Structure of a Neutral Glycosphingolipid Recognized by Human Antibodies in Polyagglutinable Erythrocytes from the Rare NOR Phenotype

Duk¹,

Reinhold²,

Reinhold³

et al. 2001

Journal of Biological Chemistry

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NOR is a rare inheritable polyagglutination phenomenon that has been described in two families. Our recent studies on these erythrocytes showed they contained at least two unique neutral glycosphingolipids, and based on their reactivity with Griffonia simplicifolia IB4 (GSL-IB4) isolectin (Kusnierz-Alejska, G., Duk, M., Storry, J. R., Reid, M. E., Wiecek, B., Seyfried, H., and Lisowska, E. (1999) Transfusion 39, 32-38), both oligosaccharide chains terminated with an ␣-galactose residue. The reactivity with GSL-IB4 suggested that these oligosaccharide chains terminated with a Gal␣133Gal-sequence and that anti-NOR agglutinins were common human anti-Gal␣133Gal xenoantibodies. In this report we describe the structure of one NOR component (NOR1) that migrated on thinlayer chromatographic plates in the region of pentaglycosylceramides. Treatment of this sample with ␣-galactosidase and ␤-N-acetylhexosaminidase was followed by highperformance thin-layer chromatography with product detection by lectins and the anti-Gb 4 monoclonal antibody. The results suggested that NOR1 was an ␣-galactosylated Gb 4 Cer with a ␤-N-acetylhexosaminidase-resistant GalNAc residue. Gas phase disassembly by ion trap mass spectrometry analysis showed the sequence to be Hex134HexN133Hex134Hex134Hex linked to a ceramide composed of C 18 sphingosine and a C 24 monounsaturated fatty acid. Together these data indicate NOR1 to be a novel Gal␣134GalNAc␤133Gal␣134Gal␤134 Glc-Cer structure. Additionally it has been shown that NOR glycolipids are recognized by human antibodies that were distinct from the known anti-Gal␣133Gal xenoantibodies.

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confidence: 67%

“…10). 2 Each sample was disassembled, and the terminal disaccharide was isolated (MS 3 ). Resonance activation of this ion, m/z 486, and spectral comparison of the products (MS 4 ) (Fig.…”

Section: Fig 5 Isolation and Msmentioning

confidence: 99%

Structure of a Neutral Glycosphingolipid Recognized by Human Antibodies in Polyagglutinable Erythrocytes from the Rare NOR Phenotype

Duk¹,

Reinhold²,

Reinhold³

et al. 2001

Journal of Biological Chemistry

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“…These findings and other experimental data strongly suggested that the P k and P1 antigens are synthesized by the same enzyme (14). Recently, a C/T polymorphism within exon 2a of the A4GALT gene was shown to predict the P 1 or P 2 genotypes and give rise to a novel open reading frame encoding a 28-amino acid peptide (15). However, no report has yet described a possible role for this peptide or a molecular link between the described mutation and P 1 /P 2 status.…”

mentioning

confidence: 69%

“…Rare inheritable NOR polyagglutination derives its name from Norton, VA, where the first case was reported (1). A similar phenotype was then described in the Polish T.S.…”

mentioning

confidence: 78%

A Single Point Mutation in the Gene Encoding Gb3/CD77 Synthase Causes a Rare Inherited Polyagglutination Syndrome

Suchanowska

Kaczmarek

Duk

et al. 2012

Journal of Biological Chemistry

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Background: Inheritable NOR polyagglutination is a rare phenomenon caused by the unusual Gal(␣1-4)GalNAc glycolipid epitope. Results: A point mutation, 631 CϾG, in the gene encoding Gb3/CD77 synthase causes the enzyme to synthesize both Gal(␣1-4)Gal-and Gal(␣1-4) GalNAc-moieties. Conclusion:The results pinpoint the cause of the NOR phenotype. Significance: This is the first report of an altered acceptor specificity of a glycosyltransferase caused by a point mutation.

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“…Recently, we found that rare erythrocytes with an inherited NOR characteristic [4] contain two unusual glycosphingolipids which are recognized by antibodies present in most human sera [5]. These glycolipids are globoside extension products terminating with the unique sequence Galα1-4GalNAcβ1-3Gal [6,7].…”

Section: Introductionmentioning

confidence: 99%

Presence of natural anti-Galα1-4GalNAcβ1-3Gal (anti-NOR) antibodies in animal sera

Duk

Lisowska

2006

Glycoconj J

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Rare polyagglutinable NOR erythrocytes contain unusual globoside extention products terminating with a Galalpha1-4GalNAcbeta1-3Gal- unit. This trisaccharide epitope is recognized by recently characterized antibodies naturally occurring in most human sera (Duk et al., Glycobiology, 15, 109, 2005). These antibodies represent two major types of fine specificity. All these antibodies are most strongly inhibited by Galalpha1-4GalNAcbeta1-3Gal (NOR-tri), and weakly by Galalpha1-4Gal. However, the type 1 antibodies are strongly inhibited by Galalpha1-4Galbeta1-3Gal-R and weakly by Galalpha1-4GalNAc, while the type 2 antibodies show the opposite reactivities with these two oligosaccharides. Similar antibodies have now been found in horse, rabbit and pig sera. The antibodies were purified from animal sera by affinity chromatography on Galalpha1-4GalNAcbeta1-3Gal-human serum albumin(HSA)-Sepharose 4B conjugate. The specificity of the antibodies was determined by binding to ELISA plates coated with several alpha-galactosylated oligosaccharide-polyacrylamide (PAA) or -HSA conjugates and by inhibition with synthetic oligosaccharides. The purified antibodies bound specifically to conjugates containing NOR-tri. The inhibition of binding showed that the animal sera also contain two types of anti-NOR antibodies: type 2 was found in the horse serum, and a mixture of both types was present in rabbit and pig serum. These results indicate that anti-NOR, a new and distinct kind of anti-alphaGal antibody, are present in animal sera and show similar specificties and diversity as their counterparts found in human sera.

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An Inherited RBC Characteristic, NOR, Resulting in Erythrocyte Polyagglutination

Cited by 36 publications

References 16 publications

Structure of a Neutral Glycosphingolipid Recognized by Human Antibodies in Polyagglutinable Erythrocytes from the Rare NOR Phenotype

Structure of a Neutral Glycosphingolipid Recognized by Human Antibodies in Polyagglutinable Erythrocytes from the Rare NOR Phenotype

A Single Point Mutation in the Gene Encoding Gb3/CD77 Synthase Causes a Rare Inherited Polyagglutination Syndrome

Presence of natural anti-Galα1-4GalNAcβ1-3Gal (anti-NOR) antibodies in animal sera

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