For Research Use Only. Not for use in diagnostic procedures.

Monoclonal

IgG1

056B-373

Mouse

This item recognises complement component 8 (C8), a 151 kDa member of the complement C6/C7/C8/C9 family, present in blood serum. C8 is a terminal component of the complement system, part of both the complement membrane attack complex (MAC), and important to MAC assembly. C8 binds to the C5b-7 complex, anchored to the membrane, creating C5b-8. C5b-8 binds C9 and catalyses the polymerization of C9 molecules to form C5-b9 (MAC). C8 is thought to contain lipid s, facilitating the insertion of MAC into the membrane.

Defects in the alpha chain of C8 can result in complement C8 deficiency type I. Furthermore, C8 deficiencies can cause recurring bacterial infections, in particular from Neisseria meningitides.

Purified
Purified IgG - liquid

IgG concentration 1.0mg/ml (lot specific)

Store at 4 degree C or at -20 degree C if preferred. Storage in frost-free freezers is not recommended. This product should be stored undiluted. Avoid repeated freezing and thawing as this may denature the antibody. Should this product contain a precipitate we recommend microcentrifugation before use.
Shelf Life: 18 months from date of despatch.

Small volumes of anti-C8 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS212936 recognises complement component 8 (C8), a 151 kDa member of the complement C6/C7/C8/C9 family, present in blood serum. C8 is a terminal component of the complement system, part of both the complement membrane attack complex (MAC), and important to MAC assembly. C8 binds to the C5b-7 complex, anchored to the membrane, creating C5b-8. C5b-8 binds C9 and catalyses the polymerization of C9 molecules to form C5-b9 (MAC). C8 is thought to contain lipid s, facilitating the insertion of MAC into the membrane. Defects in the alpha chain of C8 can result in complement C8 deficiency type I. Furthermore, C8 deficiencies can cause recurring bacterial infections, in particular from Neisseria meningitides.

Immunohistology Frozen, ELISA (EIA), Flow cytometry (FC/FACS), Western Blot (WB)

67,047 Da

complement component 8, beta polypeptide

complement component C8 beta chain; complement component 8 subunit beta

Complement component C8 beta chain

CO8B_HUMAN

This gene encodes one of the three subunits of the complement component 8 (C8) protein. C8 is composed of equimolar amounts of alpha, beta and gamma subunits, which are encoded by three separate genes. C8 is one component of the membrane attack complex, which mediates cell lysis, and it initiates membrane penetration of the complex. This protein mediates the interaction of C8 with the C5b-7 membrane attack complex precursor. In humans deficiency of this protein is associated with increased risk of meningococcal infections. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jun 2013]

C8B: 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. Defects in C8B are a cause of complement component 8 deficiency type 2 (C8D2). 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.

Protein type: Secreted, signal peptide; Secreted

Chromosomal Location of Human Ortholog: 1p32.2

Cellular Component: membrane attack complex; extracellular space; membrane; extracellular region; vesicle

Molecular Function: protein complex binding

Biological Process: cytolysis; complement activation, alternative pathway; regulation of complement activation; innate immune response; immune response; complement activation, classical pathway; complement activation

Disease: Complement Component 8 Deficiency, Type Ii

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