Table 2.

Complement system-based therapeutics. The clinical situation, principle of anticomplement therapy, and complement-based treatment are detailed.

Complement Deficiencies
Complement pathways involved			Protein	Associated gene	Complications
Alternative	Lectin	Classical
			Activation
			Recognition protein
	X		MBL	MBL2	Infection in immunocompromised patient
	X		H-ficolin	FCN3	Immune deficiency, necrotizing enterocolitis
		X	C1q	C1QA, C1QB, C1QC	SLE-like syndrome, recurrent bacterial infections
X			MASP-2	MASP-2	Immune deficiency
		X	C1r/s	C1R, C1S	SLE-like syndrome, recurrent bacterial infections
	X	X	C2	C2	Autoimmune disease
X			Factor D	CFD	Meningococcal and encapsulated bacterial infections
X			Factor I	CFI	Encapsulated bacterial infections
			Common Pathways
			Structural protein
X	X	X	C3	C3	Bacterial infections, SLE-like syndrome
X	X	X	C4	C4A, C4B	SLE- like syndrome, encapsulated bacterial infections
X	X	X	C5	C5	Meningococcal infection
X	X	X	C6	C6	Meningococcal infection
X	X	X	C7	C7	Meningococcal infection
X	X	X	C8	C8A, C8B, C8G	Meningococcal infection
X	X	X	C9	C9	Meningococcal infection
			Regulatory proteins
			Control protein as
X			Properdin	CFP	Meningococcal infection
			Factor H	CFH	Hemolytic uremic syndrome (HUS), dense deposit disease
			CD11a (LFA-1), CD11b (CR3), CD11c (CR4)/CD18'	ITGAL, ITGAM, ITGAX, ITGB2	Leucocyte adhesion deficiency type I ( LAD I)
			CD46 (MCP)	CD46	Atypical hemolytic uremic syndrome (aHUS)