The Hemolysis of Red Cells from Patients with Paroxysmal Nocturnal Hemoglobinuria by Partially Purified Subcomponents of the Third Complement Component*

Yachnin, Stanley

doi:10.1172/jci105260

Cited by 39 publications

(18 citation statements)

References 16 publications

(20 reference statements)

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“…Because of the small number of patients in each group, abnormalities of metabolism have not been excluded in all patients with these conditions but our results indicate that such abnormalities are not the rule. The failure to find increased catabolism in patients with paroxysmal nocturnal hemoglobinuria is in accord with the concept that the abnormality of the erythrocytes in patients with this disorder makes them unusually susceptible to lysis by whatever complement activation that normally occurs (25).…”

Section: Resultssupporting

confidence: 65%

“…I-labeled C'3 I was prepared from the serum of a normal individual, added to a second lot of the same serum, and reisolated. The protein content of the final preparation was determined by optical density at 280 mA with Yachnin's estimate of the extinction coefficient for C'3 (25). From the number of counts per minute introduced into the second serum lot, the volume of this lot, and the specific activity of the final preparation, we calculated that this serum contained 150 mg/100 ml of C'3.…”

Section: Introductionmentioning

confidence: 99%

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Studies of the In Vivo Behavior of Human C′3 in Normal Subjects and Patients*

Alper¹,

Rosen²

1967

J. Clin. Invest.

203

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Section: Resultssupporting

confidence: 65%

Section: Introductionmentioning

confidence: 99%

Studies of the In Vivo Behavior of Human C′3 in Normal Subjects and Patients*

Alper¹,

Rosen²

1967

J. Clin. Invest.

203

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“…It would however seem quite likely that the lysis of paroxysmal nocturnal hemoglobinuria (PNH) cells is due to reactive hemolysis. PNH cells have been found in our hands to be extremely sensitive to reactive hemolysis and there is already evidence (15) that fluid phase complement activation is associated with PNH cell lysis.…”

Section: The Properties Conferring Reactor Activity On a Serum--the mentioning

confidence: 53%

Reactive Lysis: The Complement-Mediated Lysis of Unsensitized Cells

Lachmann

Thompson

1970

The Journal of Experimental Medicine

228

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R e a c t i v e lysls is the consequence of the interaction of two reagents. One, previously called indicator, is a normal s e r u m protein and has been identified as C7 (1); the other is generated b y c o m p l e m e n t activation from certain sera (referred to as " r e a c t o r " sera) and has been called a c t i v a t e d reactor. I n this paper the n a t u r e of the activated reactor is explored and the course of the reaction analyzed. Materials and MethodsThese are described in the preceding paper (1). Diisopropyl fluorophosphate (DFP) was obtained from Boots Pure Drug Co., Nottingham, England.1:10 phenanthroline hydrate was obtained from Hopkins & Williams, Ltd., Chadwell Heath, Essex, England. A 0.5 ~ solution was made in dimethylformamide and this was diluted 1 : 50 in the E/EDTA/agarose mixture. 1Quantitation of Activated Reactor.--An estimate of activated reactor was obtained by filling a standard size well in an E/EDTA/agarose plate with activated reactor and allowing diffusion to occur for 18 hr at room temperature. The plate was then immersed in 1: 50 guinea pig EDTA complement and incubated at 37°C for 6 hr. The diameter of the zone of lysis was measured. RESULTS Fractionation of Activated ReactorAbsorption with Zymosan (4 mg/ml) and Precipitation as Euglobulin.--These were performed in the usual way. Reactor activity in the euglobulin fraction, when redissolved in 0.3 N NaCI, pH 7.2, was stable on storage both at 4°C and at -20°C. Addition of glycerol to a final concentration of 25% allows the activated reactor euglobulin to be stored, certainly for some months, at --20°C without loss of activity.

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“…?ached very iimal levels Recent advances in the understanding of the compleonal normal ment system have offered new insights to several iected, CH50 physiological and pathological processes (22)(23)(24)(25)(26)(27)(28)36).…”

Section: Discussionmentioning

confidence: 99%