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
Juji T, Satake M, Honda Y, Doi Y. HLA antigens in Japanese patients with narcolepsy. Tissue Antigens 1984; 24: 316.
Langdon N, Welsh KI, Dam MV, Voughan RW, Parkes D. Genetic markers in narcolepsy. 1984;ii:1178.
Honda Y, Juji T, Matsuki K, Naohara T, Satake M, Inoko H, et al. HLA-DR2 and Dw2 in narcolepsy and in other disorders of excessive somnolence without cataplexy. Sleep 1986; 9: 133.
Marcadet A, Gebuhrer L, Betuel H, Seignalet J, Freidel AC, Confavreux C, et al. DNA polymorphism related to HLA-DR2 Dw2 in patients with narcolepsy. Immunogenetics 1985; 22: 679.
Inoko H, Ando A, Tsuji K, Matsuki K, Juji T, Honda H. HLA-DQ chain DNA restriction fragments can differentiate between healthy and narcoleptic individuals with HLADR2. Immunogenetics 1986; 23: 126.
Matsuki K, Juji T, Tokunaga K, Naohara T, Satake M, Honda Y. HLA haplotype frequencies estimated from the data on HLA class I, II, III antigens in 111 Japanese narcoleptics. J Clin Inv 1985; 76: 2078.
Billiard M, Seignalet J. Extraordinary association between HLA-DR2 and narcolepsy. Lancet 1985;i:226.
Guilleminault C, Grumet C. HLA-DR2 and narcolepsy: Not all narcoleptic-cataplectic patients are DR2. Human Immunol 1986; 17: 1.
Andreas-Zietz A, Keller E, Scholz S, Albert ED, et al. DR2-negative narcolepsy. Lancet 1986;ii:684.
Maeda H, Hirata R, Okuyama M, Thompson A, Tohyama H. Two-dimensional gel analysis of a second family of class II molecules by polymorphic HLA-DR4, 5, and w9 monoclonal antibodies. J Immunol 1984; 132: 2478.
Maeda H, Hirata R, Kambayashi H, Koning F, Schreuder GMT. Multiple epitopes on a single DQ molecule from the DQw3-carrying haplotypes. Tissue Antigens 1986; 28: 136.
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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
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