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Science 6 August 1971:
Vol. 173. no. 3996, pp. 553 - 554
DOI: 10.1126/science.173.3996.553
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Articles

C1 Inhibitor: Evidence for Decreased Hepatic Synthesis in Hereditary Angioneurotic Edema

A. Myron Johnson 1, Chester A. Alper 2, Fred S. Rosen 2, and John M. Craig 3

1 Department of Pediatrics, University of North Carolina School of Medicine, Chapel Hill 27514
2 Department of Pediatrics, Harvard Medical School, and Blood Grouping Laboratory and Laboratory of Immunology, Children's Hospital Medical Center, Boston, Massachusetts 02115
3 Department of Pathology, Harvard Medical School, and Department of Pathology, Boston Hospital for Women, Boston, Massachusetts 02115

Although the Cl inhibitor was detected in 5 to 10 percent of normal hepatic parenchymal cells by means of the immunofluorescent technique, none was seen in liver biopsies from two individuals with hereditary angioneurotic edema having low concentrations of Cl inhibitor in the serum. In contrast, the percentages of cells which reacted with fluorescent antiserums to C4 and transferrin were normal. These data suggest that in most subjects with hereditary angioneurotic edema, there is decreased synthesis of the C1 inhibitor but normal synthesis of C4, and that the disease results from this biosynthetic error.


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Danazol.
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Ann Intern Med 96, 625-630
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Long-Term Therapy of Hereditary Angioedema with Danazol.
S. W. HOSEA, M. L. SANTAELLA, E. J. BROWN, M. BERGER, K. KATUSHA, and M. M. FRANK (1980)
Ann Intern Med 93, 809-812
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Methyltestosterone Therapy in Hereditary Angioedema.
A. L. SHEFFER, D. T. FEARON, and K. F. AUSTEN (1977)
Ann Intern Med 86, 306-308
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Hereditary Angioedema: the Clinical Syndrome and Its Management.
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Ann Intern Med 84, 580-593
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