Monocyte C1-inhibitor synthesis in patients with C1-inhibitor deficiency

Lappin, D.F., McPhaden, A.R., Yap, P.L., Carter, P.E., Birnie, G.D., Fothergill, J.E. and Whaley, K. (1989) Monocyte C1-inhibitor synthesis in patients with C1-inhibitor deficiency. European Journal of Clinical Investigation, 19(1), pp. 45-52. (doi: 10.1111/j.1365-2362.1989.tb00194.x)

Abstract

Monocytes of seven out of eight patients with type 1 C1-inhibitor (C1-inh) deficiency (HAE) produced 40% as much C1-inh as monocytes from normal donors (controls). In contrast, monocytes from three patients with type 2 and three patients with acquired C1-inh deficiency produced similar amounts of C1-inh as controls. Recombinant gamma-interferon (gamma-interferon 10 ng/ml) stimulated C1-inh production of C1-inh (eight-10-fold) by control and patients' monocytes. Monocytes from patients with type 1 HAE contained 40% the level of C1-inh messenger ribonucleic acid (mRNA) found in control monocytes. Gamma-interferon increased the abundance of C1-inh mRNA by the same extent in both control and patients' monocytes. C1-inh protein and mRNA were undetectable in the monocytes of one patient, unless stimulated by gamma-interferon. Under these conditions, his monocytes produced comparable amounts of C1-inh (protein and mRNA) as gamma-interferon-stimulated monocytes of the other type 1 HAE patients. The data suggest that in most type 2 HAE patients there is a lesion in the C1-inh gene such that mRNA is transcribed by a single allele.

• Item Type: Articles
• Status: Published
• Refereed: Yes
• Glasgow Author(s) Enlighten ID: Lappin, Dr David and McPhaden, Dr Allan
• Authors: Lappin, D.F., McPhaden, A.R., Yap, P.L., Carter, P.E., Birnie, G.D., Fothergill, J.E., and Whaley, K.
• Subjects: Q Science > QR Microbiology > QR180 Immunology
• College/School: College of Medical Veterinary and Life Sciences > School of Medicine, Dentistry & Nursing > Dental School
• Journal Name: European Journal of Clinical Investigation
• Publisher: Wiley-Blackwell Publishing Ltd.
• ISSN: 0014-2972
• ISSN (Online): 1365-2362