DR2 and DQw1 Molecules in Narcolepsy

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Immunobiology of HLA

Abstract

Narcolepsy is a clinical disease entity characterized by excessive daytime sleepiness and cataplexy. The cause and genetic basis for the disease has been unknown until 1984, when an extraordinary association between HLA-DR2 and narcolepsy was reported (1,2). This association was confirmed to be complete by the HTC ty** (Dw2) (3,4) and by molecular genetics (DQR2.6) (4,5) and was confined to a specific DR2-carrying haplotype, HLA-DR2-Dw2-DQw1 (6). We have isolated DR2 and DQw1 molecules from nine DR2-positive Japanese patients with narcolepsy using anti-DR and anti-DQw1 related monoclonal antibodies and analyzed them by one- and two-dimensional IEF gel electrophoresis. The results indicated that both DQw1 α and β chains focused identically among patients and DR2-Dw2-positive normals but clearly distinct from those of DR2-Dw12 normal Japanese. DR2 α and β chains were identical among patients and DR2-Dw12 as well ad DR2-Dw2 normals. A second DR2 β chain isolated from one DR2 homozygous patient was identical with those of normal DR2-Dw2 individuals but distinct from DR2-Dw12 normals. The data strongly suggest that both DR2 and DQw 1 molecules are identical among patients and normals.

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References

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© 1989 Springer Science+Business Media New York

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Maeda, H., Hirata, R., Matsuki, K., Juji, T., Honda, Y. (1989). DR2 and DQw1 Molecules in Narcolepsy. In: Dupont, B. (eds) Immunobiology of HLA. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-39946-0_179

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  • DOI: https://doi.org/10.1007/978-3-662-39946-0_179

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-38980-5

  • Online ISBN: 978-3-662-39946-0

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