Neisseria meningitidis inside neutrophils, revealing properdin deficiency

Gillet, Benjamin; Joram, Nicolas; Bacchi, Veronique Frémeaux; Thomas, Caroline; Béné, Marie Christine; Wuillème, Soraya

doi:10.1016/j.ijid.2020.07.040

Cited by 3 publications

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“…A young childbearing carrying this mutation had less than 10% AP activity and died due to Neisseria menigitidis infection. 60 As alluded to already, we also described a type II deficiency caused by mutation of glutamate 244 to lysine in TSR3, which results in absence of AP activity. Recombinant FP E244K is secreted in cell culture almost exclusively as a monomer with a compact structure 19 that is likely to be shared with the wildtype but rare FP1 single-chain monomer (Figure 2).…”

Section: A S Truc Ture-ba S Ed Under S Tanding Of Properdin Deficien ...mentioning

confidence: 58%

“…Accordingly, only very low levels of recombinant FP C32Y could be expressed in mammalian cell culture compared with the wildtype FP. A young childbearing carrying this mutation had less than 10% AP activity and died due to Neisseria menigitidis infection 60 …”

Section: A Structure‐based Understanding Of Properdin Deficienciesmentioning

confidence: 99%

“…A young childbearing carrying this mutation had less than 10% AP activity and died due to Neisseria menigitidis infection. 60 …”

Section: A Structure‐based Understanding Of Properdin Deficienciesmentioning

confidence: 99%

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Structural studies offer a framework for understanding the role of properdin in the alternative pathway and beyond

Pedersen

Lorentzen

Andersen

2022

Immunological Reviews

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The complement cascade is activated when pattern recognition molecules recognize pathogens, dying host cells or immune complexes. The proteolytic cascades in complement can initiate through the classical pathway (CP) or the lectin pathway (LP) which leads to assembly of the CP/LP C3 convertase C4b2a. This proteolytic enzyme turns over complement C3 into the anaphylatoxin C3a and the opsonin C3b that may become covalently linked to the surface of the complement activator (Figure 1A). The downstream alternative pathway (AP) provides an amplification loop for the two other pathways. 1 The C3b initially deposited by the CP C3 convertase can associate with Factor B (FB) and form the AP proconvertase C3bB that upon cleavage by Factor D (FD) becomes the active AP C3 convertase C3bBb. 1 This initiates a positive feedback loop, which markedly amplifies the complement activation through the CP and LP. 2,3 FP is central in the AP amplification loop and binds directly to C3b, the C3bB proconvertase, and the C3bBb convertase (Figure 1A). The alternative pathway may also initiate in the

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Section: A S Truc Ture-ba S Ed Under S Tanding Of Properdin Deficien ...mentioning

confidence: 58%

Section: A Structure‐based Understanding Of Properdin Deficienciesmentioning

confidence: 99%