C1q deficiency is linked to autoimmune disorders

12 March 2010
Last modified: 12/03/2010 14:20

C1q deficiency is linked to autoimmune disorders

Human C1q ELISA: a reliable measurement

C1q: first step in classical complement activation

  • C1q is an integral part of the first component of complement (C1)
  • Formation of an antibody-antigen complex (immunocomplex) activates the classical pathway of the complement system. 
  • C1q triggers the activation process by docking onto antibodies within these immunocomplexes
  • C1q bridges the innate and adaptive immune systems
  • Necrosis and apoptosis results in exposure of endogenous danger patterns, which can be directly recognized by C1q, thus activating the classical pathway
  • Activated C1 cleaves complement proteins C2 and C4, resulting in the formation of C4bC2a, also called C3 convertase

C1q and diseases

  • Deficieny of C1q is directly linked to Systemic Lupus Erythematosus (SLE) and glomerulonephritis due to homozygous congenital deletions
  • Deficiency of C1q is associated with a higher risk of pyogenic infections
  • Decrease in C1q serum levels results in reduced uptake of apoptotic cells
  • The decrease of phagocytosis results in increased autoimmunity resulting in more circulating auto-antibodies

Matrix influences in human C1q ELISA

  • Different matrices were evaluated in the human C1q ELISA
  • Serum as well as plasma has to be diluted 200x to obtain reliable measurement (Fig 1)
  • C1q in bronchoalveolar lavages (BAL) can be detected accurately when BAL is diluted at least 2x (Fig 2)
  • No influence of anticoagulant was detected

Validation of human C1q ELISA

  • C1q-depleted serum was spiked with purified C1q
  • Recovery of spiked C1q was within the limits of 80-120% 
  • No C1q was detected in C1q-depleted serum

Reference standard

  • Purified C1q derived from Complement Technology Inc was used to validate our standard calculation (Fig 3)
  • Perfect match between external C1q source and Hycult Biotech C1q (R2= 0.9997)

Specifications of human C1q ELISA (Cat.# HK356)

  • Working time of 3.5 hours
  • 100 µl sample/well
  • Detection limit of 15.6 ng/ml
  • Concentration range 15.6 – 1000 ng/ml
  • Useful for serum,  plasma and BAL
  • Detection of unbound C1q as well as C1q-immunocomplexes (CIC)
  • Cross-reactive with mouse and rat C1q

Available related products:

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 HK323  MBL, Human, ELISA kit
 HK326  MASP2, Human, ELISA kit
 HK327  MBL/MASP2, Human, ELISA kit
 HK328  TCC, Human, ELISA kit
 HK336  L-Ficolin, Human, ELISA kit
 HK339  MASP3, Human, ELISA kit
 HK340  H-Ficolin, Human, ELISA kit
 HK342  Factor H, Human, ELISA kit

More information on complement and collectins