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Rabbit Anti-Properdin/FITC Conjugated antibody
background:
A positive regulator of the alternate pathway of complement. It binds to and stabilizes the C3- and C5-convertase enzyme complexes.
Involvement in disease:Defects in CFP are the cause of properdin deficiency (PFD). PFD results in higher susceptibility to bacterial infections; especially to meningococcal infections. Three phenotypes have been reported: complete deficiency (type I), incomplete deficiency (type II), and dysfunction of properdin (type III).
Function:
A positive regulator of the alternate pathway of complement. It binds to and stabilizes the C3- and C5-convertase enzyme complexes.
Subcellular Location:
Secreted
DISEASE:
Defects in CFP are the cause of properdin deficiency (PFD) [MIM:312060]. PFD results in higher susceptibility to bacterial infections; especially to meningococcal infections. Three phenotypes have been reported: complete deficiency (type I), incomplete deficiency (type II), and dysfunction of properdin (type III).
Similarity:
Contains 6 TSP type-1 domains.
Database links:
Entrez Gene: 5199 Human
Entrez Gene: 18636 Mouse
Entrez Gene: 299314 Rat
Omim: 300383 Human
SwissProt: P27918 Human
SwissProt: P1336 Mouse
Unigene: 53155 Human
Unigene: 3064 Mouse
Unigene: 204119 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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