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Deficiency of CD59 can result in an overactivity of complement, uncontrolled host cell lysis and development of paroxysmal nocturnal haemoglobinuria (Walport, 2001).
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One potential explanation for the uncontrolled complement activities is that complement regulatory proteins in the serum may be reduced in the patients.
Uncontrolled complement activation can induce many inflammatory and life threatening conditions.
Atypical hemolytic uremic syndrome (aHUS) is a rare genetic life-threatening disease of chronic uncontrolled complement activation leading to thrombotic microangiopathy (TMA) and severe end-organ damage.
Physically induced (cold, pressure, vibration, ultraviolet, etc).: histamine ↑ (direct) V. Deficiency or inactivation of C1-INH : uncontrolled complement activation → bradykinin ↑ 1.
HAE types 1 and 2 and AAE (deficiency or inactivation of C1-INH) are characterised by uncontrolled complement activation and resultant increased bradykinin production [2, 3, 5, 10].
Selective inhibition of the AP by properdin inhibitors such as IxACs may therefore be used to treat pathological effects of uncontrolled complement activation by this pathway, without compromising the classical and lectin pathways.
Atypical haemolytic-uraemic syndrome (aHUS) is a disease of uncontrolled complement alternative pathway activation.
These results further emphasize the role of uncontrolled complement activation in the pathophysiology of PNH-related mortality.
The patient received 1000 U 1 h before surgery and 1000 U 1 h before extubation (7 h after operation), and the uncontrolled complement activation was avoided.
Dense deposit disease (formerly membranoproliferative glomerulonephritis type II) and C3 glomerulonephritis are also associated with uncontrolled complement activation and mutations in CFH or CFI have been described [ 8].
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