catalog number :
MBS627016
products full name :
Complement, C1q, Human
products short name :
Complement, C1q
products name syn :
Anti -Complement, C1q, Human
other names :
complement C1q subcomponent subunit C; Complement C1q subcomponent subunit C; complement C1q subcomponent subunit C; complement component 1, q subcomponent, gamma polypeptide; complement component 1, q subcomponent, C chain
other gene names :
C1QC; C1QC; C1QG; C1Q-C; C1QG
uniprot entry name :
C1QC_HUMAN
specificity :
Recognizes human C1Q: 100%.
form :
Supplied as a liquid.
storage stability :
May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
tested application :
ELISA (EL/EIA), Radioimmunoassay (RIA)
app notes :
Suitable for ELISA or RIA. Dilution: RIA: 20,000 tests/ml
other info1 :
Immunogen: Human C1Q
products categories :
Antibodies; Abs to Complement
products description :
C1q is serum glycoprotein of 18-polypeptides chains consisting of three non-identical subunits, A (29kD), B (26kD) and C (246aa, 19kD) in molar ratio of 1:2:2. C1Q in the plasma is complexed with two proenzymes C1r and two C1s molecules to form the first component of complement (C1). Activation of complement via classical pathway is triggered by binding of globular head of C1q to immune complexes containing IgG (Fc-region) or IgM or to a variety of other activating substances, including C-reactive protein, retrovirus and mitochondria. Subsequent to C1q binding, c1r and C1s are converted to proteolytic enzymes that are responsible to continuation of activation via the classical pathway. Normal plasma concentration of C1q is 100-180mg/L.
ncbi acc num :
NP_001107573.1
ncbi gb acc num :
NM_001114101.1
ncbi mol weight :
25,774 Da
ncbi pathways :
Chagas Disease (American Trypanosomiasis) Pathway (147809); Chagas Disease (American Trypanosomiasis) Pathway (147795); Classical Antibody-mediated Complement Activation Pathway (106409); Complement Activation, Classical Pathway (198823); Complement And Coagulation Cascades Pathway (198880); Complement And Coagulation Cascades Pathway (83073); Complement And Coagulation Cascades Pathway (484); Complement Cascade Pathway (106405); Creation Of C4 And C2 Activators Pathway (106407); Immune System Pathway (106386)
ncbi summary :
This gene encodes a major constituent of the human complement subcomponent C1q. C1q associates with C1r and C1s in order to yield the first component of the serum complement system. A deficiency in C1q has been associated with lupus erythematosus and glomerulonephritis. C1q is composed of 18 polypeptide chains: six A-chains, six B-chains, and six C-chains. Each chain contains a collagen-like region located near the N-terminus, and a C-terminal globular region. The A-, B-, and C-chains are arranged in the order A-C-B on chromosome 1. This gene encodes the C-chain polypeptide of human complement subcomponent C1q. Alternatively spliced transcript variants that encode the same protein have been found for this gene. [provided by RefSeq, Jul 2008]
uniprot summary :
C1QC: C1q associates with the proenzymes C1r and C1s to yield C1, the first component of the serum complement system. The collagen-like regions of C1q interact with the Ca(2+)-dependent C1r(2)C1s(2) proenzyme complex, and efficient activation of C1 takes place on interaction of the globular heads of C1q with the Fc regions of IgG or IgM antibody present in immune complexes. Defects in C1QC are a cause of complement component C1q deficiency (C1QD). A rare defect resulting in C1 deficiency and impaired activation of the complement classical pathway. C1 deficiency generally leads to severe immune complex disease with features of systemic lupus erythematosus and glomerulonephritis. Protein type: Secreted; Secreted, signal peptide. Chromosomal Location of Human Ortholog: 1p36.11. Cellular Component: extracellular space; collagen; extracellular region. Biological Process: negative regulation of macrophage differentiation; innate immune response; immune response; complement activation, classical pathway; complement activation; negative regulation of granulocyte differentiation. Disease: C1q Deficiency