摘要:

Angioedema may be due to hereditary forms of CI-lnh deficiency, but recently an autoimmune form of angioedema has been described in which the mechanism is novel. While the peripheral blood monocytes of patients with autoimmune angioedema produce a normal, functionally active, 105 KD CI-Inh in normal quantities, the CI-Inh isolated from the patient's plasma exists in a dysfunctional lower molecular weight (96 KD) performance. Rather than bind and biologically inactivate the enzyme, a relatively common phenomenon in autoimmune disease, the autoimmune angioedema cleave the CI-Inh molecule. The following sequence of events is proposed: structural and functionally normal CI-lnh is synthesised and secreted, this secreted inhibitor is complexed by autoantibody and following enzyme interaction. denatured 96 KD CI-lnh is proposed. This process depletes the pool of normal, functional CI-lnh to crticial levels and predisposes patients to episodes of oedema.

ISSN:0891-6934    

DOI:10.3109/08916939008993390

出版商:Taylor&Francis    

年代:1990

数据来源: Taylor

摘要:

Immune activation events regulated by the human MHC are triggered by interactions between HLA class II molecules, antigenic peptides, and reactive T cells. In most autoimmune diseases. little is known about the latter two elements of this trimolecular complex, while the HLA class II contribution is being deciphered in increasingly sophisticated detail. In two cases in particular, type I diabetes and rheumatoid arthritis, susceptibility is tied to structural elements within specific HLA class II genes. Site-directed mutagenesis and gene expression studies provide a means to directly test the contribution of specific residues within individual candidate disease susceptibility genes to peptide and T cell interactions.

ISSN:0891-6934    

DOI:10.3109/08916939008993391

出版商:Taylor&Francis    

年代:1990

数据来源: Taylor

ISSN:0891-6934    

DOI:10.3109/08916939008993392

出版商:Taylor&Francis    

年代:1990

数据来源: Taylor

ISSN:0891-6934    

DOI:10.3109/08916939008993393

出版商:Taylor&Francis    

年代:1990

数据来源: Taylor

ISSN:0891-6934    

DOI:10.3109/08916939008993394

出版商:Taylor&Francis    

年代:1990

数据来源: Taylor