[Anomaly of the polypeptide and oligosaccharide chains of alpha-2-neuraminoglycoprotein in various types of hereditary angioneurotic edema].
Using two-dimensional immuno-electrophoresis to study a morphological abnormality of the alpha 2-neuramino-glycoprotein (alpha 2-NGP) we have been able to observe allelic exclusion in patients afflicted by hereditary angioneurotic oedema [1]. With the aid of two-dimensional immuno-affino-electrophoresis, we have been able to show that in some cases, there exists a double abnormality of alpha 2-NGP associating a deficiency in post translating glycosylation with a loss of the inhibitory function of the C1 esterase.
D000799:Angioedema / Q000097:blood* / Q000235:genetics; D055598:Chemical Phenomena; D002621:Chemistry; D003174:Complement C1 Inactivator Proteins / Q000097:blood*; D003208:Concanavalin A; D006801:Humans; D005779:Immunodiffusion; D007123:Immunoelectrophoresis, Two-Dimensional; D037102:Lectins; D009844:Oligosaccharides / Q000032:analysis; D010455:Peptides / Q000032:analysis; D012795:Sialoglycoproteins / Q000097:blood*; D014909:Wheat Germ Agglutinins
D003174:Complement C1 Inactivator Proteins; D037102:Lectins; D009844:Oligosaccharides; D010455:Peptides; D012795:Sialoglycoproteins; D014909:Wheat Germ Agglutinins; D003208:Concanavalin A
Anomalie des chaînes polypeptidique et oligosaccharidique de l'alpha-2-neuraminoglycoprotéine au cours de certaines formes d'oedème angioneurotique héréditaire.