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    For Research Use Only. Not For Clinical Use.


    Background

    This gene encodes a plasma glycoprotein that positively regulates the alternative complement pathway of the innate immune system. This protein binds to many microbial surfaces and apoptotic cells and stabilizes the C3- and C5-convertase enzyme complexes in a feedback loop that ultimately leads to formation of the membrane attack complex and lysis of the target cell. Mutations in this gene result in two forms of properdin deficiency, which results in high susceptibility to meningococcal infections. Multiple alternatively spliced variants, encoding the same protein, have been identified.[provided by RefSeq, Feb 2009]
    Protein class

    Disease related genes, Human disease related genes, Plasma proteins

    Predicted location

    Intracellular, Secreted (different isoforms)

    Single cell type specificity

    Cell type enhanced (monocytes, Kupffer cells, Macrophages, Langerhans cells, Hepatic stellate cells)

    Immune cell specificity

    Group enriched (intermediate monocyte, classical monocyte, myeloid DC, non-classical monocyte, neutrophil)

    Cell line specificity

    Cell line enhanced (EFO-21, HEL, HMC-1, NB-4, U-937)

    Interaction

    In plasma, properdin exists as dimers, trimers or tetramers in the relative proportions of 26:54:20 (PubMed:20382442, PubMed:15491616, PubMed:28264884, PubMed:31507604). Interacts with the pro-C3-convertase enzyme complex (C3b-Bb) comprised of Complement C3 beta chain (C3b) and the Complement factor B Bb fragment (Bb), where it binds (via its TSP type-1 5 domain) with C3b and Bb (PubMed:28264884, PubMed:31507604). This interaction stabilizes the complex and allows it to become the active C3-convertase enzyme complex (C3b-Bb-FP) (PubMed:28264884, PubMed:31507604). Interacts with C3b (PubMed:28264884, PubMed:31507604). Interacts with CFB (PubMed:31507604).

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