Measurement of Complement Deficiencies
The complement system is a complex series of proteins that act as a cascade. They react in the sequence C1q, C1r, C1s, C4, C2, C3, C5, C6, C7, C8, C9; many requiring proteolytic cleavage to become active. The cascade is activated either via the Classical Pathway, the Alternative Pathway or the Lectin Pathway.
Complement is involved in initiating an inflammatory response and destroying certain bacteria and viruses. Complement proteins are assayed to measure complement consumption and to diagnose inherited deficiencies of cascade components.
The CH50 method is used as a screening test (for the measurement of total classical complement activity) when assessing the ability of an individual to fight infection.
The measurement of complement CH50 activity is now recommended as part of the diagnostic protocol for Primary Immunodeficiency 1,2 and it can also provide important information for many other disease states such as Systemic Lupus Erythematosus and bacterial infections.3
CH50 measures the lysis of antibody-sensitised sheep erythrocytes after addition of fresh serum. The result is expressed as the reciprocal of the dilution that yields 50% erythrocyte (red blood cell) lysis. In comparison the CH100, or total haemolytic complement, is a measurement of 100% lysis of the red blood cells.
The AH50 (or AH100) is used to detect a deficiency of the alternative complement pathway.
This assay has been developed to measure total complement activity (CH50), by directly testing the function of the resulting end product of the complement cascade, the Membrane Attack Complex (MAC). Measurement of total complement activity (CH50) is recommended in Primary Immunodeficiency guidelines and provides important additional information for many other disease states and infections.
| Description | Pack | Code |
| CH50 SPAplus reagent Range 12-95 U/mL Sensitivity 12 U/mL |
200 test | NK095.S |
| CH50 controls for SPAplus 5 x high control, 5 x low control |
1 set | NQ095.S |
| CH50 calibrators for SPAplus | 1 set | NC095.S |
-
Terminal Complement Complexes which occur naturally during sample handling are recognised by the CH50 Eq ELISA
-
No requirement for red blood cells and therefore not subject to seasonal variation and shelf-life limitations
-
Good correlation with gold standard methods4
-
Results are reported directly in CH50 Eq units by plotting a simple linear graph
-
CH50 Eq ELISA is provided as a complete kit which can be automated for ease of use
-
Once activated, samples can be stored frozen at -20°C to allow batch testing
| Description | Pack | Code |
|
Classical Complement Pathway CH50 Eq ELISA kit |
96 test | MK095 |
Total Haemolytic Complement (THC) and Alternative Pathway Haemolytic Complement (APHC) kits are effective screening assays to aid in the identification of complement deficiency.
The Total Haemolytic Complement kit is primarily designed to detect deficiencies of the Classical Complement Pathway and the terminal sequence (C3-C9) components, whereas the Alternative Pathway Haemolytic Complement kit is designed to measure the activity of the Alternative Complement Pathway.
Simple methodology, no sample dilutions are necessary- A 2 step incubation allows the formation of large, easy to read zones of haemolysis
- Results are interpreted easily without the need for expensive equipment
- Assays are supplied in kit format for ease of use
- 1, 2 or 3 plate kits are available to suit individual laboratory requirements
|
Description
|
Pack
|
Code
|
|
Total Haemolytic
Complement kit
|
3 plate kit
2 plate kit
1 plate kit |
RC001.3
RC001.2
RC001.1 |
|
Alternative Pathway
Haemolytic Complement kit*
|
3 plate kit
1 plate kit
|
RC003.3
RC003.1
|
|
C1 Inactivator
Functional activity kit
C1 Inactivator
Functional activity COMBI kit
Combi kit contains 2 functional activity plates and 1 antigenic quantitation plate.
|
3 plate kit
3 plate kit
|
RC002.3
RK019
|
If a deficiency in one of the complement pathways is detected after screening, the level of individual complement components may be measured separately.
Binding Site supplies a wide range of assays (automated, semi-automated and RID) for quantitative assessment of complement components.
These assays have been developed to quantify the levels of complement proteins C3c and C4 in human serum. Measurement of C3c and C4 aid in the diagnosis of immunologic disorders, especially those associated with deficiencies of complement components.
| Description | Pack | Code |
| C3c SPAplus kit Range 250 - 3000 mg/L (1/10) Sensitivity 250 mg/L |
100 test | NK023.S |
| C4 SPAplus kit Range 64 - 900 mg/L (1/10) Sensitivity 64 mg/L |
100 test | NK025.S |
|
Description
|
Pack
|
Code
|
|
C1 Inactivator RID kit – NL
Range: 45 – 450 mg/L
|
3 plate kit
|
RN019.3
|
|
C1q RID kit – NL
Range: 23 - 230# mg/L
|
3 plate kit
|
RN020.3
|
|
C2 RID kit – NL
Range: 3.6 - 36 mg/L
|
3 plate kit
|
RN022.3
|
|
C3 RID kit – NL
Range: 155 - 1550 mg/L
|
3 plate kit
|
RN023.3
|
|
C4 RID kit – NL
Range: 58 - 580 mg/L
|
3 plate kit
|
RN025.3
|
|
C4 - Binding Protein RID kit – NL*
Range: 50 - 500 mg/L
|
3 plate kit
|
RN026.3
|
|
C5 RID kit – NL
Range: 20 - 200 mg/L
|
3 plate kit
|
RN027.3
|
|
C6 RID kit - NL*
Range: 12 - 120 mg/L
|
3 plate kit
1 plate kit
|
RN102.3
RN102.1
|
|
C7 RID kit - NL*
Range: 22 - 110 mg/L
|
3 plate kit
1 plate kit
|
RN103.3
RN103.1
|
|
C8 RID kit - NL*
Range: 20 - 200 mg/L
|
3 plate kit
1 plate kit
|
RN089.3
RN089.1
|
|
C9 RID kit – NL
Range: 50 - 500 mg/L
|
3 plate kit
1 plate kit
|
RN028.3
RN028.1
|
|
Factor B RID kit – NL
Range: 45 - 450 mg/L
|
3 plate kit
|
RN029.3
|
|
Factor H (β 1H) RID kit - NL*
Range: 70 - 700 mg/L
|
3 plate kit
1 plate kit
|
RN030.3
RN030.1
|
|
Factor I RID kit - NL*
Range: 7 - 70 mg/L
|
1 plate kit
|
RN031.1
|
# Diluted sample applied - assay range may be extended using undiluted sample.
* Research use only
|
Description
|
Pack
|
Code
|
|
MININEPH C1 Inactivator
Range: 0.075 – 0.90 g/L
|
25 tests
|
ZK019.R
|
|
MININEPH C3
Range: 0.28 - 4.44 g/L
|
50 tests
|
ZK023.R
|
|
MININEPH C4
Range: 0.08 - 1.23 g/L
|
50 tests
|
ZK025.R
|
More assays are available on the MININEPH and the MININEPHplus™.
- De Vries E. Patient-centred screening for primary immunodeficiency: a multi-stage diagnostic protocol designed for non-immunologists. Clin Exp Immunol 2006;145:204-214
- Bonilla FA, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. Ann Allergy Asthma Immunol 2005; 94:S1-63
- Botto M, et al. Complement in human diseases: Lessons from complement deficiencies. Mol Immunol 2009; 46:2774-2783
- Kabat EA, et al. Complement and Complement Fixation. Experimental Immunochemistry 1967







Screening for complement deficiencies
Screening for complement deficiencies