[Deficiency of mannan-binding protein--a recently discovered complement defect syndrome].
Mannan-binding protein (MBP) is a newly discovered serum protein which activates the complement system by the classical pathway by binding to mannose-rich surfaces on microorganisms. MBP deficiency predisposes to infection in infancy. MBP is a C-type lectin with collagen structure, a so-called collection. The paper discusses the structure and physiological functions of MBP. MBP may be an important parameter in the investigation of immunodeficiencies in children.
Garred
P
P
Vaevstypelaboratoriet, Rigshospitalet, København.
Thiel
S
S
Madsen
H O
HO
Ryder
L P
LP
Jensenius
J C
JC
Svejgaard
A
A
D002352:Carrier Proteins / Q000737:chemistry* / Q000276:immunology; D037222:Collectins; D003171:Complement Pathway, Classical / Q000276:immunology*; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000209:etiology* / Q000276:immunology; D007223:Infant; D011951:Receptors, Complement / Q000276:immunology / Q000378:metabolism; D013577:Syndrome
D002352:Carrier Proteins; D037222:Collectins; D011951:Receptors, Complement