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001-es BibID:	BIBFORM094509
035-os BibID:	(Cikkazonosító)31877 (WOS)000381563400001 (Scopus)84983354675
Első szerző:	Dobó József
Cím:	MASP-3 is the exclusive pro-factor D activator in resting blood : the lectin and the alternative complement pathways are fundamentally linked / József Dobó, Dávid Szakács, Gábor Oroszlán, Elod Kortvely, Bence Kiss, Eszter Boros, Róbert Szász, Péter Závodszky, Péter Gál, Gábor Pál
Dátum:	2016
ISSN:	2045-2322
Megjegyzések:	MASP-3 was discovered 15 years ago as the third mannan-binding lectin (MBL)-associated serine protease of the complement lectin pathway. Lacking any verified substrate its role remained ambiguous. MASP-3 was shown to compete with a key lectin pathway enzyme MASP-2 for MBL binding, and was therefore considered to be a negative complement regulator. Later, knock-out mice experiments suggested that MASP-1 and/or MASP-3 play important roles in complement pro-factor D (pro-FD) maturation. However, studies on a MASP-1/MASP-3-deficient human patient produced contradicting results. In normal resting blood unperturbed by ongoing coagulation or complement activation, factor D is present predominantly in its active form, suggesting that resting blood contains at least one pro-FD activating proteinase that is not a direct initiator of coagulation or complement activation. We have recently showed that all three MASPs can activate pro-FD in vitro. In resting blood, however, using our previously evolved MASP-1 and MASP-2 inhibitors we proved that neither MASP-1 nor MASP-2 activates pro-FD. Other plasma proteinases, particularly MASP-3, remained candidates for that function. For this study we evolved a specific MASP-3 inhibitor and unambiguously proved that activated MASP-3 is the exclusive pro-FD activator in resting blood, which demonstrates a fundamental link between the lectin and alternative pathways.
Tárgyszavak:	Orvostudományok Elméleti orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban folyóiratcikk MASP-3 factor D
Megjelenés:	Scientific Reports. - 6 (2016), p. 1-12. -
További szerzők:	Szakács Dávid Oroszlán Gábor Kortvely, Elod Kiss Bence Boros Eszter Szász Róbert (1972-) (belgyógyász, haematológus) Závodszky Péter Gál Péter Pál Gábor
Internet cím:	Szerző által megadott URL DOI Intézményi repozitóriumban (DEA) tárolt változat
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