The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
1/5000. Detects a band of approximately 105 kDa (predicted molecular weight: 105 kDa).
Constituent of the membrane attack complex (MAC) that plays a key role in the innate and adaptive immune response by forming pores in the plasma membrane of target cells.
Involvement in disease
Defects in C6 are the cause of complement component 6 deficiency (C6D) [MIM:612446]. A rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections, predominantly by Neisseria gonorrhoeae or Neisseria meningitidis.
Belongs to the complement C6/C7/C8/C9 family. Contains 1 EGF-like domain. Contains 2 Kazal-like domains. Contains 1 LDL-receptor class A domain. Contains 1 MACPF domain. Contains 2 Sushi (CCP/SCR) domains. Contains 3 TSP type-1 domains.
All cysteine residues are assumed to be cross-linked to one another. Individual modules containing an even number of conserved cysteine residues are supposed to have disulfide linkages only within the same module.