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Original Article
Brief Report
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Volume 332:86-90 January 12, 1995 Number 2
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Twin Boys with Major Histocompatibility Complex Class II Deficiency but Inducible Immune Responses
Hermann M. Wolf, M.D., Ilona Hauber, Ph.D., Heinz Gulle, Ph.D., Vojtech Thon, M.D., Heinz Eggenbauer, M.D., Michael B. Fischer, M.D., Sabine Fiala, M.D., and Martha M. Eibl, M.D.

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The major histocompatibility complex (MHC) class II molecules are composed of two polypeptide chains ({alpha} and {beta}). In humans the three distinct isotypes are designated HLA-DR, DP, and DQ. The different isotypes serve similar functions, and their expression on cells is regulated in coordinated fashion.1 MHC class II molecules are expressed on the surface of certain cells of the immune system, such as monocytes and macrophages, B cells, and dendritic cells, where they play an important part in the immune response.2

MHC class II deficiency is an autosomal recessive disease characterized by cellular and humoral immunodeficiency.3 Family studies show that . . . [Full Text of this Article]

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From the Institute of Immunology, University of Vienna (H.M.W., I.H., H.G., V.T., H.E., M.B.F., M.M.E.), and the Pediatric Department, Wilhelminenspital of the City of Vienna (S.F.) — both in Vienna, Austria.

Address reprint requests to Dr. Eibl at the Institute of Immunology, University of Vienna, Borschkegasse 8A, A-1090 Vienna, Austria.

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