Table 1 Levels of complement proteins... | American Society of Hematology

Table 1

Levels of complement proteins and found mutations for the 2 discussed families

	Family 1			Family 2			Normal ranges
	P1		P2	I.1	I.2	P3
	I.1	II.1	II.2	I.1	I.2	II.2
C3, mg/L	256	985	287	833	725	172	660-1250
C4, mg/L	372	205	305	174	111	207	93-380
FB, mg/L	110	132	109	128	99	55	90-320
H, mg/L	555	545	474	566	520	499	338-682
I, mg/L	68	63	63	67	61	69	42-78
CD46	nd	850	904	909	917	818	660-1400
Factor B *(protein)	p.Asp279Gly	p.Asp279	p.Asp279Gly	p.Lys350	p.Lys350	p.Lys350Asn
Factor B† (structure)	Asp²⁵⁴Gly (D²⁵⁴G)	Asp²⁵⁴	Asp²⁵⁴Gly (D²⁵⁴G)	Lys³²⁵	Lys³²⁵	Lys³²⁵Asn (K³²⁵G)

	Family 1			Family 2			Normal ranges
	P1		P2	I.1	I.2	P3
	I.1	II.1	II.2	I.1	I.2	II.2
C3, mg/L	256	985	287	833	725	172	660-1250
C4, mg/L	372	205	305	174	111	207	93-380
FB, mg/L	110	132	109	128	99	55	90-320
H, mg/L	555	545	474	566	520	499	338-682
I, mg/L	68	63	63	67	61	69	42-78
CD46	nd	850	904	909	917	818	660-1400
Factor B *(protein)	p.Asp279Gly	p.Asp279	p.Asp279Gly	p.Lys350	p.Lys350	p.Lys350Asn
Factor B† (structure)	Asp²⁵⁴Gly (D²⁵⁴G)	Asp²⁵⁴	Asp²⁵⁴Gly (D²⁵⁴G)	Lys³²⁵	Lys³²⁵	Lys³²⁵Asn (K³²⁵G)

Bold indicates values below the normal range.

Numbering with the peptide signal.

Numbering without the peptide signal.