PLASMA REGULATORY PROTEINS FOR COMPLEMENT Flashcards
includes DEFICIENCY OF COMPLEMENT REGULATORS
dissociates C1r and C1s from C1q
C1 INHIBITOR (C1 INH)
cleaves C3b and C4b
FACTOR I
cofactor with I to INACTIVATE C3b, prevents binding of B to C3b
FACTOR H
acts as a cofactor with I to inactivate C4b
C4-BINDING PROTEIN
the principal soluble regulator of the ALTERNATIVE PATHWAY
FACTOR H
prevents attachment of the C5b67 complex to cell membrane
S PROTEIN (VITRONECTIN)
dissociates C2b or Bb from binding sites, thus preventing formation of c3 convertase
DAF (DECAY ACCELERATING FACTOR)
DAF is associated with what CD number?
CD 55
DAF, daghan F…FIFTY-FIVE
PREVENTS INSERTION OF C9 into cell membrane
MIRL
MIRL is associated with what CD number?
CD 59
cofactor with I; mediates transport of immune complexes; it is found MAINLY ON PERIPHERAL BLOOD CELLS
CR1
it binds C3b and C4b but has the GREATEST AFFINITY FOR C3b
CR1
found on virtually all epithelial and endothelial cells EXCEPT ERYTHROCYTES
MCP
it is THE MOST EFFICIENT COFACTOR FOR FACTOR I-MEDIATED CLEAVAGE OF C3b
MCP
MOST COMMON DEFICIENCY
C2
dagha’g C2 na drink sa grocery so common siya 🤩
MOST SEVERE DEFICIENCY
C3
related MAINLY with Neisseria infections
C5-C8
NO KNOWN DISEASE ASSOCIATION
C9
associated with intravascular or extravascular hemolysis because of COMPLEMENT ACTIVATION; assoc with PNH
DAF and MIRL
PNH is caused by a mutation of what gene?
PIG-A GENE
PHOSPHATIDYL INOSITOL GLYCAN A
associated with HEREDITARY ANGIOEDEMA
C1 INH DEFICIENCY
associated with RECURRENT PYOGENIC INFECTIONS; C3 will be constantly turn over to be activated until it becomes depleted
FACTOR H and FACTOR I DEFICIENCY
