catalog number :
MBS537423
products type :
Native Protein
products full name :
Complement C1q protein
products short name :
Complement C1q
products name syn :
C1q protein
other names :
complement C1q subcomponent subunit B; Complement C1q subcomponent subunit B; complement C1q subcomponent subunit B; complement component 1, q subcomponent, B chain
other gene names :
C1QB; C1QB
uniprot entry name :
C1QB_HUMAN
form :
10mM HEPES, 300mM NaCl, pH 7.2.
concentration :
1.1 mg/ml. Activity: 555,000 U/mg
storage stability :
Aliquot and store at -70 degree C or lower. Avoid repeated freeze/thaw cycles.
other info1 :
Source Note: Human serum/plasma. Protein Type: Native. Biological Significance: C1q is part of the C1 complex and this complex is the first complement component in the cascade referred to as the classical pathway of complement. C1 is actually a non-covalent assembly of three different proteins (C1q, C1r, and C1s) bound together in a calcium-dependent complex. C1q has six extended arms with domains at the end of each arm that bind to the Fc domains of immunoglobulins. Bioactivity: >1,000,000 C1H50 units/mg
other info2 :
Biohazard Information: Donor samples were tested and found to be negative for HBsAg, HTLV-I/II, STS, and for antibodies to HCV, HIV-1 and HIV-II. Nonetheless caution should be used when handling this material as there is a margin of error in all tests. Dry Ice Shipment: Extra charge fee may add to your shipping cost as dry ice is required to ship this product.
products categories :
Immunology; Native Protein; Complements
products description :
Purified Complement C1q protein isolated from human plasma
ncbi acc num :
NP_000482.3
ncbi gb acc num :
NM_000491.3
ncbi mol weight :
410 kDa
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. Deficiency of 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 B-chain polypeptide of human complement subcomponent C1q [provided by RefSeq, Jul 2008]
uniprot summary :
C1QB: 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 C1QB 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, signal peptide; Secreted. Chromosomal Location of Human Ortholog: 1p36.12. Cellular Component: collagen; extracellular region; complement component C1 complex. Molecular Function: protein binding; protein homodimerization activity. Biological Process: innate immune response; complement activation, classical pathway; inner ear development; complement activation. Disease: C1q Deficiency