Hemolytically inactive C5b67 complex: an agonist of polymorphonuclear leukocytes
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Hemolytically inactive C5b67 complex: an agonist of polymorphonuclear leukocytes
From www.bloodjournal.org by guest on November 18, 2014. For personal use only. 1995 85: 2570-2578 Hemolytically inactive C5b67 complex: an agonist of polymorphonuclear leukocytes C Wang, S Barbashov, RM Jack, T Barrett, PF Weller and A Nicholson-Weller Updated information and services can be found at: http://www.bloodjournal.org/content/85/9/2570.full.html Articles on similar topics can be found in the following Blood collections Information about reproducing this article in parts or in its entirety may be found online at: http://www.bloodjournal.org/site/misc/rights.xhtml#repub_requests Information about ordering reprints may be found online at: http://www.bloodjournal.org/site/misc/rights.xhtml#reprints Information about subscriptions and ASH membership may be found online at: http://www.bloodjournal.org/site/subscriptions/index.xhtml Blood (print ISSN 0006-4971, online ISSN 1528-0020), is published weekly by the American Society of Hematology, 2021 L St, NW, Suite 900, Washington DC 20036. Copyright 2011 by The American Society of Hematology; all rights reserved. From www.bloodjournal.org by guest on November 18, 2014. For personal use only. Hemolytically Inactive C5b67 Complex: An Agonist of Polymorphonuclear Leukocytes By Ce Wang, Sergei Barbashov, Richard M. Jack, Tonya Barrett, Peter F. Weller, and A n n e Nicholson-Weller The activity of hemolytically inactive C5b67, designated iC5b67, was evaluated as an agonist for functional responses of human polymorphonuclear leukocytes (PMN). C5b67 was formed from purified human complement components and decayed in phosphate-buffered saline (PBS) until it had no lytic activity for sheep erythrocytes in a standard assay. iC5b67, at nanomolar concentrations, stimulated PMN chemotaxis and Ca2+ fluxes, but inhibited superoxide production and failed to upregulate CR1 and CR3. There was no significant contamination of the iC5b67 with C5a to explain these results. Neither isolated C5b6 nor C7 alone exhibited the activities of iC5b67, while insolubilized anti-C7 could remove the PMNagonist activity from theiC5b67 preparation. Binding studies to define a specific receptor for iC5b67 on PMN were hampered by the very hydrophobic nature of the ligand. '251-iC5b67. by contrast to hemolytically active '251C5b67, was unable to insert in erythrocytes, suggesting that iC5b67 need not insert in the PMN membrane to induce signaling. Two lines ofevidencesuggest that iC5b67 and C5a and FMLP sharecommon steps in intracellular signaling (1) pretreatment of PMN with iC5b67 deactivates PMN for C5a- and FMLP-inducedchemotaxis; and (2) pretreatment of PMN with pertussis toxin inhibits iC5b67-induced chemotaxis. Thus, iC5b67 has important effects on the activity of PMN and G-proteins and Ca2+are involved in thesignaling. 0 1995 by The American Societyof Hematology. T defect. The second reason that studies of the chemotactic activity of iC5b67 were not pursued related to the discovery of C5a as a potent chemotaxin.' With the chromatographic and immunologic reagents available at the time, it would have been difficult to rule out the possibility of C5a contamination of iCSb67.Withnewer chromatographic methods, specific antisera, and better assays, it is now possible to define the composition of this ligand. Therefore, we reexamined the signaling potential of iC5b67 for human polymorphonuclear leukocytes (PMN). Our findings indicate that iC5b67 is potent in nanomolar concentrations for inhibiting superoxide production, while stimulating chemotaxis and Ca2* fluxes. The ability of iC5b67 to deactivate the chemotactic activity of C5a and FMLP, as well as the demonstration of pertussis toxin (PTX) inhibition of iC5b67 activity, are consistent with iC5b67 sharing elements of the signaling pathway used by C5a and FMLP. When complement is activated in the fluid phase, such as would happen in the presence of soluble lipopolysaccharide (LPS) or immune cornplexes, a large fraction of the C5b67 formed may decay to iC5b67, and thus iC5b67 has the potential to be an important modulator of PMN activation. However, a ligand with the potency of iC5b67 might be expected to have a very short half life in vivo, and iC5b67 binding proteins, such as vitronectin and clusterin, may exert regulatory roles by inactivating iC5b67. HE FORMATION OF C5b67 is initiated by the limited proteolysis of complement C5 to form C5b, which binds C6 to form a stable C5b6 complex. The addition of C7 to C5b6 forms the C5b67 complex, which transiently rearranges to expose the hydrophobic domains necessary for membrane insertion.'.' There are three possible fates for the nascent C5b67: (1) insert into membrane lipids and act as a signaling ligand3.'; (2) insert into membrane lipids and, by providing binding sites for C8 and C9, become part of a transmembrane channel (hemolytically active); or (3) decay to a hemolytically inactive complex in thefluidphase (iC5b67).5 The iC5b67 complex can react with components C8 and C9 andwith vitronectin (S-protein) and clusterin, but no biologic functions have been described for anyof these nonhemolytic terminal complement complexes. In retrospect, over 2 decades ago a fluid phase complex comprised of C5, C6, and C7, likely what we refer to as iC5b67, was reported to have chemotactic a~tivity.~.' There were at least two reasons why this work was not pursued: first, critics at that time held that fluid phase C5b67 could not be important as a chemotactic factor if C6 deficient rabbits had normal chemotactic function.' We now have a growing appreciation of the redundancy of crucial mediators, even a deficiency of C5, which precludes the generation of both C5a and iC5b67, does not lead to a global immune From theCharles A. Dana Research Institute and Harvard-Thorndike Laboratory of Beth Israel Hospital; Department of Medicine, Harvard Medical School and Beth Israel Hospital, Boston; and the Department of Rheumatology and Immunology, Brighum and Women's Hospital andthe Department of Medicine, Harvurti Medical School, Boston, MA. Submitted September 8, 1994; accepted December 12, 1994. Supported by National Institutes of Health Grant MO. HL.33768 to A.M.W., Grant No. AI20241 to P.F. W., and Grant No. AI26292 to R.M.J. Address reprint requests to Anne Nicholson-Weller, MD, Division of Infectious Diseases, BethIsrael Hospital, 330 Brookline Ave, Boston, MA 022 15. The publication costs of this article were defrayed in part by page charge payment. This article must therefore be hereby marked "advertisement" in accorclance with 18 U.S.C. section 1734 solely to indicate this fact. 0 1995 by The American Society of Hematology. 0ao6-497l/95/8509-0008$3.00/0 2570 MATERIALS AND METHODS Complement Complexes and Assays CSb6 was made frompurified CS, C6, cobra venom factor (CVF), factor B (all from Quidel, San Diego, CA), and recombinant mouse factor D (adipsin; gift of Dr Ty White, Scios, Mountain View, CA) C5b6 was fractionwith modifications"'of the original method.'' The ated by highperformanceliquidchromatography(HPLC)ona DEAE column (AP-1 Protein Pak 8HR; Waters Associates, Milford, MA) asdescribed."The peak CSb6containingfractionswere pooled, and the pool titered using human erythrocytes. One unit of C5b6 was defined as the amount of C5b6 required to produce SO% lysis of 2.5 x IO' human E when incubated in a total volume of 300 pL with C7 (0.1 pg), C8 (0.5 pg), and C9 (0.5 pg) (all from Quidel)."' To f o m CSb67,30 pg of CSb6andatwofoldmolar excess of C7(20pg)weremixedandincubatedat 37°C for IS minutes, then 4°C overnight. Although iC5b67 may aggregate," we have used the monomeric molecular weight (mw) of 400 kD in the calculation of molarity. Blood, Vol 85,No 9 (May l ) , '1995:pp 2570-2578 From www.bloodjournal.org by guest on November 18, 2014. For personal use only. AGONIST COMPLEMENT iC5b67: AN FOR PMN Antibody Methods For the iC5b67 enzyme-linked immunosorbent assay (ELISA), wells (Immulon 2; Dynatech, Chantilly, VA) were coated with polyclonal goat antihuman C7 (U200 dilution; Quidel) overnight at 4°C. All subsequent steps were performed at m m temperature. Phosphatebuffered saline (PBS) 0.1% Tween was used for all washes and dilutions, except as noted; blocking was performed withSuperblockbuffer Dilutions of sample, as well as dilutions of an (Pierce, Rockford, L). iC5b67 standard (16 to 130 ng), were added to wells for 30 minutes. Indicator monoclonal antibody (MoAb) anti-C6 (Quidel; 111,OOO dilutionin0.5%TweenPBS)wasadded for 30 minutes followed by the sequential additions of the following reagents: biotinylated goatantimouse IgG(H+L) (1/1,ooO,Kirkegaard & Perry, Gaithersburg, MD) for 30 minutes; streptavidin-horseradish peroxide (111,OOO; Pierce) for 30 minutes, and finally 100 pL of the substrate solution (TMB Microwell Peroxidase Substrate System; Kirkegaard & Perry). After blue color developed, the reaction was terminated by addition of 1 0 0 pL of 250 mmoVL phosphoric acid. The absorbance unit (AU) at 450 nm of the wells were read within 1 hour using a Thermomax Microplate Reader (Molecular Devices, Menlo Park, CA). The assay was linear over a range of 16 to 130 ng. IgG fractions of goat antihuman C7 (Quidel) and of goat antihuman factor H (Quidel) were prepared by adsorption to protein G Superose (HR 10/2; Pharmacia, Piscataway, NJ) and elution with 0.1 molL glycine-HC1 buffer, pH 2.7. The IgG peaks were neutralized, and reabsorbed to protein G agarose beads (P-7700; Sigma, St Louis, MO) for use in the iC5b67 absorption experiments. Protein Methods Protein was assayed using a modified Fohn assay (BCA, enhanced, Pierce), using BSA as a standard. Sodium dodecyl sulfatepolyacrylamide gel electrophoresis (SDS-PAGE) was performed using a 7% to 10% gradient slab gel with discontinuous buffers." To radiolabel C5b6, approximately 40 pg of C5b6 was mixed with 40 pCi carrier free sodium IZ5I (New England Nuclear, Boston, MA) in Iodogen (Pierce)-coated tubes according to the manufacturer's specifications. A PD-10 sizing column (Pharmacia) wasusedto remove free Iz5I.Ninety-six percent of the cpm of the "'I-C5b6 pool were precipitable with 10% trichloroacetic acid (TCA), yielding a specific activity of 5 X lo3 c p d p g . The IZ5I-C5b6 wasfully functional in terms of forming C5b67 and iC5b67. Preparation of PMN PMN were isolated at room temperature from 20 mL of venous blood from normal volunteers. Blood was anticoagulated with 5 mL acidified citrate, mixed with 5 mL dextran (6% Dextran 70 in 0.9% NaC1; Kendall McGaw Laboratories, Inc, Irvine, CA) and the E allowed to sediment for 60 minutes at room temperature. The leukocyte-rich supernatant was under-layered with LO mL Ficoll-Paque (Phannacia) and centrifuged for 20 minutes at 500g. The PMN fractions were recovered, and contaminating E lysed with hypotonic saline. PMN were washed 2X, quantified by hemocytometer, and resuspended in Hanks' Balanced Salt Solution (HBSS), without Caz+ or Mg2+but with 0.1% bovine serum albumin (HBSA). The isolated cells were greater than 95% to 98% neutrophils (2% to 5% eosinophils) and greater than 98% viable as assessed by Wright-Giemsa and trypan blue staining, respectively. PMN were used within 2 hours of venipuncture. 2571 taxis chamber (Neuro Probe, Cabin John, MD). The apparatus was placed in a 37"C, humidified, 5% CO, incubator for a specified time to allow migration. The first assay to assess PMN chemotaxis utilized 5 pm pore polyvinylpyrrolidone-free polycarbonate filters (Nucleopore, Neuro Probe #NFB5).I4 Each dilution of chemoattractant was set up in quadruplicate. After a 60-minute incubation, the filters were stained with Wright-Giemsa (Diff Quik; Baxter Scientific, McGaw Park, IL) and the cells on the bottom surface of the filter were enumerated by an individual blinded to the protocol. For each well, four to five fields on the filter were analyzed using a 63X objective. The second chemotactic assay utilized 5 pm pore nitrocellulose mesh filters (Toyo, Neuro Probe #TCB5). Each dilution of chemoattractant was added to triplicate wells. After a 60-minute incubation, the filters were fixedand stained with Congo red.''.'6 An Optomax V image analyzing system (Analytical Instruments, Shaffron Walden, Essex, England) enumerated the cells in four 25X fields at 20-pm intervals, beginning 20 pm from the upper surface. The third chemotactic assay measured the ability of PMN that were loaded with BCECF-AM (Molecular Probes, Eugene, OR) (2 pmol/ L for 20 minutes) to migrate through a 3-pm pore polyvinylpymolidone-free polycarbonate filter (Neuro Probe #PFD3). An incubation time of 45 minutes was sufficient time to allow cells responding to chemoattractants to migrate through the filter, and minimized the chance the PMN would fall off the filter, as detected by the fluorescence in the lower chamber. Fluorescence was quantified with a fluorescence plate reader (Cytofluor, Millipore, Bedford, MA) using excitation at 485 nm and emission at 530 nm. The ability of PTX (Sigma) (2 pg/mL for an hour at 37"C), to inhibit the chemotactic response was assessed using the first chemotactic assay. Superoxide Release Assay Superoxide release was measured as the superoxide dismutase (SOD)-inhibitable reduction of fenicytochrome c. In the routine assay, PMN suspensions (8 X lo5 cells/mL) were incubated with fenicytochrome c (1 mg/mL; Sigma) and either buffer or buffer plus agonist. Replicate tubes contained SOD (21 pglmL; Sigma) in addition. In the experiment depicted (see Fig 3), the reaction tubes were preincubated with a 0.5% gelatin solution, which eliminated the release of superoxide by resting PMN. After 15 minutes at 37"C, the reaction was stopped by chilling to 4°C and centrifugation. The optical density (OD) 550 nm of the supernatants was determined in a spectrophotometer (DU series 60; Beckman Instruments, Fullerton, CA). In the microassay, each component was reduced to 114 volume of the regular assay, and 210 pL of the supernatant was transferred to a microtiter plate welland the OD 550 nm quantified with a ThermoMax microtiter plate reader (Molecular Devices, Menlo Park, CA). All reactions were performed in duplicate or triplicate for the regular assay and in triplicate for the microassay. Results are expressed asthemean nanomoles superoxide/number of cells/l5 minutes.17 To test if iC5b67 might itself inhibit the detection of superoxide anion, superoxide was generated in a cell free system." Purine (10 mmoVL, Sigma), cytochrome c (1 mg/mL, Sigma) and either iC5b67 mom), or buffer, or SOD were mixed, followed by the (3 X addition of xanthine oxidase 15 pL (0.05 U; Worthington Biochemical Corp, Freehold, NJ). The reaction mixtures were incubated for 15 minutes at 37°C then immediately put on ice. Cold PBS (750 pL) was added to each tube and the OD 550 nm was determined. Ca2+Flux Assay Chemotaxis Three different chemotactic assay procedures were used. Freshly isolated PMN (2.5 x IO') were placed in the upper wells and dilutions of chemoattractants (28 @L),or control buffer (HBSS" with 0.1% ovalbumin) were added to the bottom wells of a microchemo- PMN (1 X 107/mLin HBSA++)were incubated with Indo-l AM (5 pmoVL; Molecular Probes, Eugene, OR) at 37°C for 7 minutes. The cell suspension was diluted 5X with HBSA2+,and incubated a further 10 minutes at 37°C. The Indo-loaded cells were pelleted at 500g for 10 minutes, and resuspended in HBSA at 107/mLat room From www.bloodjournal.org by guest on November 18, 2014. For personal use only. WANG ET AL 2572 0 l I 2 6 4 I I I 8 10 I 12 16 I I 14 Preincubation time (min) Fig 1. C5b67 decays in the absence of serum proteins. Purified C5b6 ( l 0 0 pL, 9 ngl was aliquoted, and purified C7 (l00 pL, 6 ng) was added to the tubes sequentially at the indicated timesincubafor tion at 37°C. At zero time, 100 p L human E (2.5 x 10') were then added t o each tube and the reaction continued for the hemolytic assay for C5b67, as described in Materials and Methods. The results were expressed as 2, the average hemolytic sites per cell." A timedependent loss of hemolytic activity was observed. The initial halflife was approximately 30 seconds.This experiment is representative of t w o performed. fication and characterization of the components of the complex and the method of forming the complex at that time differed from our procedures. Because the characterization of iCSb67 iscentral to the thesis of this work, we reevaluated the time course of the decay of C5b67 to iCSb67. One hundred microliters of diluted C5b6 (9 ng)wasaliquotedto tubes and a twofold molar excess of C7 (6 ng) was added to different tubes at -30, - 15, -5, -2, - 1, and -0.5 minUtes for incubation at 37°C. At zero time, 1 0 0 yL human E was added to each tube with incubation at 37°C for 20 minutes followed by addition of 0.5 yg C8 and 0.5 yg C9 and a further incubation at37°C for 60 minutes. The maximal hemolytic potential of the C5b6 was assessed by adding C5b6 and C7 to a tube already containing E, and continuation of the reaction as noted above. All reactions were stopped by the addition of 2 mL 0.15 molL saline-EDTA to each tube, centrifugation of the samples, and determination of the hemoglobin in the supernatant (OD 541 nm). The initial halflife of this second order decay reaction was approximately 30 seconds (Fig l ) . Although this value is longer than the < 0.1 second half-life previously reported when different experimental conditions were used,' our results do confirm 1 temperature. One hundred microliters o f the cell suspensionwas diluted with 400 pL prewarmed HBSA'+. and 50 pL dilutions o f iC5b67 were added to the cells. The cell fluorescence was analyzed by flow cytometry (FACStar Plus; BecktonDickinson,Mountain View. CA). The ratio o f bound/free Ca" (fluorescence intensity at 405 nmlfluorescence intensity at 485 nm) was determined (Chronys Software; Becton Dickinson). Ca" ionophore (A23187, 3 pmol/L; Sigma) and FMLP (10"' mol/L) were used as positive controls; HBSS buffer was used as a negative control. Assa.yfor CRI and CR3 Expression PMN (200 pL at 2 X 10'/mL HBSA) were incubated at 37°C for 30 minutes with iC5b67 (10"' mol/L), buffer alone (negative control), and either C5a (IO-' mol/L) or FMLP ( IO" mol/L), as positive controls. Fifty microliters o f the above reaction suspensionswas aliquoted into four tubes, and further manipulations weredoneat 4°C. Each aliquot o f cells was reacted with saturating doses o f one o f the following MoAb: anti-HLA class I (W6/32).'" anti-CRI(YZl),"' anti-CR3 (OKM-l)?' or an equivalent amount o f an irrelevant M o A b (TIB157. antihuman I g A chain). After addition o f F I T C goat-antimouse IgG (Jackson Labs, West Grove, PA), the fluorescence intensity o f washed cells was measuredby FACS(BD FacSort; Becton Dickinson). Statistical Analysis Data are presented as mean 5 SEM. The Student's t-test was used to compare two samples. RESULTS Confirmation That C5h67 Decays to a Hemolytically Inactive Form Although the decay of C5b67 to a hemolytically inactive form was previously experimentally documented,' the puri- Incubationtime (min) '% Fig 2. Differential trypsin susceptibility of lnl-iC5b67-E and C5b67-E. Erythrocyte-complement complexes were formed by the addition of 1 mL of human E (2.5 x 10'lmL) t o 2 mL buffer (GVB") containing '"I-C5b6, or preformed '251-iC5b67, or lnl-C5b6 plus C7 t o generate nascent '''l-C5b67. Incubation (37°C x 20 minutes) was terminated by washing the cells and resuspending them in 3 mL buffer. Three hundred microliters of each reaction was aliquoted into t w o sets of tubes, one set containing 6 pLltube trypsin(6Yo wtlvol) (-1 and the other set of tubes containinga comparable volume of buffer ( - 4 . Each reaction was run in duplicate. After incubation(37°C) for 0.5,20, 40, and 60 minutes, the tubes were put intoan ice bath and 10 p L of 9% soybean trypsin inhibitor (wtlvoll was added t o stop thedigestion. The pellets of E were washed and counted (Cobra Auto-Gamma; Packard Instrument CO, Meriden, CT). The percentage of cpm remaining on E was calculated as (mean cpm of samplehean cpm of buffer treatedcells at zero time) x 100. Cpm at zero time for '251-C5b6,preformed '%C5b67, or nascent '251-C5b67were 137,322, and 521 respectively. Inset: Net percent of cpm removed after a 20minute incubation with trypsin. This experiment was performed six times with similarresults. From www.bloodjournal.org by guest on November 18, 2014. For personal use only. 2573 COMPLEMENTiC5b67: AN AGONIST FOR PMN .-c E m 0.6 T- buffer C5a C5a+ FMLP FMLPt iC5b67 iC5b67 t 0.5 addition of trypsin caused the time-dependent loss of cell associated cpm that differed for CSb67 and iCSb67. Consistent with its known insertion in membranes after 20 minutes of trypsin treatment, only 34% of the nonbuffer elutable c m associated with EC5b67 were susceptible totrypsin cleavage; whereas for EC5b6, which is known not to insert v) 2 X Q! 0.4 v) a, 3 0.3 2 a, 2 0.2 v) v) a, z 0.1 c Fig 3. iC5b67 inhibits C5a- and FMLP-induced superoxide production by PMN. Additions of buffer, or iC5b67 (lo-' mol/L) were made t o PMN (2 x lo5),which weresuspended in ferricytochrome c( l m g l mL), and the reactants were incubated at 37°C. After 15 minutes, C5a (lo-'mol/L) or FMLP mol/L)were added for an additional incubation of 15 minutes at37°C. Reactions were set up in sextuplicate with half thesamples containing SOD 121 pg/mLI. The reaction tubes had been precoated with gelatin 10.50101 for 1 hour at room temperature before the additionof PMN t o decrease contact activation of the cells. A microassay was performedas described in Materials and Methods. The nanomoles of superoxide produced per 2 x lo5 PMN per 15 minutes werecalculated from thedifferences in the OD5= of the supernatants from samples with SOD and without SOD. The results are the mean 2 SEM of triplicate values. "Compared with C5a or FMLP alone,P < .05. This experiment isrepresentative of two performed. I 0.2 l I l 0.3 0.5 0.4 l B Resting ** that there is a relativelyrapid decay of CSb67 hemolytic function. Accessihiliry of Erythrocyte-Bound '251-iC5h67to Trypsin Although iC5b67 was not active in lysing erythrocytes, it was possible that it inserted into the plasma membrane. Recently published data indicate that hemolytically competent terminal complement complexes, including CSb67 and CSb8, as well as C5b-9, can directly interact with G proteins and thereby mediate signaling by a receptor independent mechanism: In the early studies of the biology of the terminal complement complexes, evidence that hemolytically competent CSb67 was physically inserted into the plasma membrane of target E came from measuring the accessibility of radioiodinated components of the complex to trypsin digestion.' When EC5b6 was formed using radiolabeled C6, virtually all the cpm were solubilized when the target cells were treated with trypsin. In contrast, whenECSb67 was formed using either radiolabeled C6 or radiolabeled C7, approximately 50% of the original cpm remained cell associated after trypsin treatment. We used a similar experimental design to test the relative trypsin susceptibility of bound EiCSb67 and hemolytically competent ECSb67, which were formed from Iz5I-C5b6and C7 (Fig 2). Only about 10% of the cpm associated with either erythrocyte-complement complex eluted in buffer during the l-hour incubation. The I 0.6 iC5b67 * iC5b67+a C7 m I 0.2 0.30.5 ** I 0.4 iC5b67+a H I I 0.6 nmoles 0, / 15 min / 10 PMN Fig 4. The specificity of the iC5b67 ligand. (A) iC5b67 activity is not reproduced by isolatedC5b6 or C7. C5b6 and C7 used for making iC5b67 were assayed for superoxide production byPMN as described in Materials and Methods. The reaction tubes were not precoated with gelatin, which explains the high constitutiverelease of resting cells. C5b6 (0.18 pg) and C7 (0.8 pgl did not affect the superoxide production of PMN. The results represent the mean f SEM of triplicate values. (B) lnsolubilized antLC7 IgG removes iC5b67 activity. Polyclonal anti-C7 IgG and anti-H IgG, as control IgG, were each purified from respective antiserum by adsorption and elution from Protein G. The IgG peaks were separately reabsorbed t o protein Gagarose, and iC5b67 (15pg) was mixed with each type ofbead overnight at 4°C. Aliquots from supernatants of the reaction mixtures were tested in the superoxide assay. The results represent the mean 2 SEM of triplicatevalues. Compared with resting, " P > .05; ""P < .05. The supernatant from anti C7-beads had no reactivity by ELSAand failed t o inhibit superoxide production by PMN. From www.bloodjournal.org by guest on November 18, 2014. For personal use only. WANG ET AL 2574 in the membrane, 75% of the cpm were trypsin susceptible (Fig 2 inset). Eighty-one percent of the EiC5b67 associated cpmweretrypsin sensitive. indicating thatiCSb67, like CSb6, was extracellular. iCSh67 Inhibits CSa- and FMLP-Induced Superoxide Production Superoxide production is a well-characterized response of PMN to agonists such as C5a and FMLP. We useda standard assay based on the reduction of cytochrome c to test whether iC5b67 might havean effect on the generation of superoxide. Unexpectedly, iC5b67 not only failed to stimulate superoxide production (data not shown). it inhibited superoxide production elicited by CSa (IO-’ mol/L) and FMLP (IO-” mol/ L) (Fig 3). To rule out the possibility that iC5b67 might be inhibiting the assay for superoxide. superoxide was generated in a cell free system using purine as the substrate for xanthine oxidase and cytochrome c as the electronic acceptor and indicator of color change.’’ When additions of PUS, iC5b67 (3 X IO“) mol/L), or SOD (21 pg/mL) were made to the reaction, the mean OD 550 was 0.012, 0.01 1, and 0.0.respectively (n = 5). Thus, the iC5b67 must be inhibiting the ability of PMN to generate superoxide, because the iCSb67 has no significant effect on the detection of chemically generated superoxide by this assay. Additional experiments were performed to confirm that the biologic activity we were noting was really caused by the iC5b67 and was not caused by one of its constituents, or a contaminant. In these experiments the reaction tubes were not precoated with gelatin, and there is substantial superoxide produced by the resting cells, presumably because of contact activation. First, C5b6 (0.18 pg) alone and C7 (0.8 pg) alone were tested and neither affected the ability of PMN to produce superoxide (Fig 4A). Second, the ability of insolubilized anti-C7 to remove the biologic activity of the iC5b67 was tested. iC5b67 complex was mixed with anti-C7 IgG- (or, anticomplement factorH IgG-) protein G-beads for an indicated time. After the beads were pelleted, the supernatants were assayed for iC5b67 by ELISA. The supernatant from anti-C7 protein G-beads were ELISA-negative and failed to inhibit PMN superoxide production, whereas the supernatantsfrom antiH protein G-beads retained inhibitory biologic activity (Fig 4B). Chemotaxis and Chemokinesis To screen for an effect of iC5b67 on PMN mobility, dilutions of iC5b67 (IO”’ to IO” mol/L) were tested for chemotactic activity using a Boyden chamber with a polyvinylpyrrolidone-free polycarbonate filter. There was distinct chemotactic activity, maximal activity at 10“) mol/L,with apparent high dose inhibition at IO-’ and IO” mol/L (Fig 6). Effects of iC5b67 on random migration were evaluated. To perform this analysis. three chambers with nitrocellulose filters were used:one chamber contained dilutions of iCSb67 in the lower chamber to test chemotaxis; the second contained dilutions of iC5b67 in the upper chamber to assess chemokinesis; and the third contained dilutions of iCSb67 in both the upper and lower, which is also a test of chemokinesis. Each chamber also contained positive controls for the migratory capacity of the PMN: PMN in the upper wells, with FMLP (IO-’ mol/L) in the lower wells. The results (Table I ) again show a peak of iC5b67 chemotactic activity at IO-’ mol& and no dose-related effect on chemokinesis. In fact, when iC5b67 was in the upper wells with the cells, there was frequently less migration into the filter than when the cells were in medium alone, which is indicated by negative numbers in Table 1. In this assay, cells were counted at multiple depths within the filter, which specifically would allow the identification of distinct subpopulations of migrating cells. The distribution of cells migrating to iC5b67 was 1400 t 1000 Y2 800 600 400 200 0 iCSb67 Complex Does Not Upregulate the Expression of CRI and CR3 iC5b67 was compared with FMLP and CSa for its ability to affect the expression of class I MHC, CRI (CD3S), and CR3 (CD1 1b, CD 18). None of the agonists significantly affected the expression of class I MHC. FMLP and CSa both upregulated CRI and CR3, as previously noted.”.” By contrast, iC5b67 (1 0”’ mol/L) did not affect the expression of these complement receptors (Fig 5). In another experiment, seven twofold dilutions of iC5b67 from 6.4 X IO-’ mol/L to IO-’ mol/L had no effect on class I, CRI, or CR3 expression (data not shown). Control W6/32 I!@= YZ-l OKM-1 Fig 5. Comparison of the effects of iC5b67and C5a onthe expression of CR1 and CR3 by PMN. PMN (200 pL at 2 x 10’lmLI were incubated at 37°C for 30 minutes with buffer alone, FMLP (10” moll L), iC5b67 mollLI, or C5a (lo-’ mollL), respectively. Aliquots (50 pL) were reacted with the following MoAb control MoAb, antiHLA class I (W6/32), anti-CR1 (YZ-l) or anti-CR3 (OKM-l), followed by FITC-conjugated second antibody, as described in Materials and Methods. The cells were analyzed by flow cytometry and the mean fluorescentchannel (MFC) determined. Nanomolar iC5b67did not affect the expression of either CR1 or CR3, which were shown to be upregulated by C5a and FMLP. This experiment was representative of t w o performed. From www.bloodjournal.org by guest on November 18, 2014. For personal use only. COMPLEMENTiC5b67: AN AGONIST FOR PMN 2575 100 T I Medium C5a FM LP I 0 Log Concentration(M) of iC5b67 Fig 6. iC5b67 stimulates chemotaxis of PMN. Different doses of iC5b67 (lo”* -10” mol/L, 28 pL) were testedin chemotaxis assays using polycarbonatefilters, as described in Materials and Methods. The filter was fixed and stained by Quick. Diff The mean PMN number per field was calculated. iC5b67 significantly stimulated chemotaxis of PMN and theeffects were dose-dependent. The error bars indicate standards errors of quadruplicate samples. This experiment isrepresentative of fourperformed. like that of FMLP and provided no evidence that iC5b67 stimulated only a subpopulation of PMN. I I I I I I 500 o l o 0 15002000250030003500 I 4oo0 4 x) Relative Fluorescence Unit Fig 7. iC5b67 deactivates C5a and FMLP-induced chemotaxis. PMN 12.5 x lo5)were preincubatedwith iC5b67 (lo-’ mol/L) or buffer alone at 37°C for 60 minutes and then added to the upper wells of Boyden chambers for the assessment of chemotaxis toward either rC5a (10” mol/L) or FMLP [lo-’ mol/L), which were in the lower used, and the cells were enumerated wells. A nitrocellulose filter was and the migration index calculated as described in Materials and Methods. Results are expressed as the mean 2 SEM, n = 9. Three fields were counted and reactions were setupin triplicate. Preincubation of PMN with iC5b67 significantly diminished their Chemotactic response t o C5a and FMLP. *Compared with C5a or FMLP alone, P c .05. Deactivation of CSa- or FMLP-Induced Chemotaxis by iCSb67 Both C5a and FMLP are well characterized chemotactic factors for PMN. Although each has a distinct G protein Table 1. Effect of iC5b67 on PMN Mobility: Chemotactic and Chemokinetic Analysis Net Cell No. at 40 pm Depth. Chamber No. Presence of iC5b67 10”’ mol/L 10”’ mol/L 1OP mol/L mol/L 1 Top-/Bottom+ 42.2 2 12.5 57.7 ? 8.4 72.3 2 10.3 15.9 52.3 -C -34.0 2 Top+/Bonom+ Top+/Bottom- 0.6 -c 7.7 -14.9 -t 10.4 -17.6 -C 9.0 -t 20.9 6.7 -41.8 -C 6.5 2.3 2 10.7 ND 2 6.7 3 Each chemotactic chamber had its own controls to test for PMN responsiveness. Medium alone was a negative control and FMLP at 10.’ mol/L in the bottom well only was the positive control. The net cell number migrating toward FMLP for chambers 1, 2 and 3 were 72.3,58.6, and 65.1, respectively. + with iC5b67added: - without iC5b67. Abbreviation: ND, no data. Net cell number = total cells migrating into the filter at 40 pg depth - cells migrating to the same depth in response to medium alone. Results are expressed as the mean, n = 9 fields (three fields counted from triplicate wells). Negative numbers indicate that there was more cell mobilityin the medium control well than in the sample well. coupled receptor, exposure to one factor diminishes the response to the second, by a process known as cross-deactivation. Experimental evidence indicates that cross-deactivation results from the fact that boththe C5a receptor and the FMLP receptor share the same signaling pathway. To test if iC5b67 might also share the same pathway, BCECF-loaded PMN that had been pretreated with iC5b67 (IO-’ mol/L at 37°C X 1 hour), were compared with control cells incubated with buffer alone, for their migratory response to C5a (IO-’ mol/ L) or FMLP (IO-’ mol/L) in the bottom wells. After a 45minute incubation, the fluorescence of the cells that reached the bottom of the filter was quantified. The results (Fig 7) indicate that previous exposure to iC5b67 inhibited the chemotactic response of PMN to both C5a and FMLP. P7X Inhibition of iCSb67 Stimulated Chemotaxis The receptors for both C5a and FMLP signal through PTX inhibitable Gi proteins. We tested the PTX susceptibility of iC5b67 signaling by preincubating the PMN with the toxin ( 2 ,ug/mL for 1 hour at 37°C). Subsequently, the PMN were assayed for their chemotactic response to iC5b67 ( 1 O-’ mol/ L) and FMLP (IO-’ mol&). Compared with PMN that had been preincubated in buffer, PMN that were preincubated with PTX had a modest inhibition of random motility (Fig 8). FTX completely inhibited the PMN chemotactic response From www.bloodjournal.org by guest on November 18, 2014. For personal use only. WANG ET AL 2576 70 60 50 40 30 20 10 0 Fig 8. Effect of PTX on the PMN response t o iC5b67. PMN were exposed t o PTX (2 pg/mL for1 hour at 37°C). The cells were assayed for their chemotactic response t o iC5b67 (lo-* mol/L) andFMLP (lo-* mol/L) using a polyvinylpyrrolidone-free polycarbonate filter. The filter was stained and cells the that had migrated to the bottom surface were enumerated as described in Materials andMethods. Each reaction mixture wasassayed in triplicate. The results are expressed as the mean f SEM.PTX completely inhibited the PMN chemotactic response t o iC5b67. Comparing the responses of control cells with PTX treated cells: *P > .05 and * * P < .05. In two other experiments PTX treatment inhibited the PMN chemotactic response t o iC5b67 by 50% and 100%. We performed ligand binding assays using "%iCSb67 to establish if there were saturable binding, which would provide evidence for a specific receptor on the PMN. Replicate samples did not yield reproducible binding (data not shown), a fact we attribute to aggregation of this hydrophobic ligand. Aggregation of iCSb67 has been noted previously." To determine if iCSb67 were acting on the surface of the cell, the accessibility of erythrocyte bound "'I-iCSb67 to trypsin was assessed. Weused erythrocytes for these studies because they had been used to compare the trypsin accessibility of ECSb6 and EC5b67.' Additionally, complement-induced release of intracellular proteases is less of a consideration with erythrocytes thanwith PMN. In thepublished study, 90% of the cpm associated with bound '"I-CSb6 were released by trypsin digestion, while only about SO% the cpm associated with bound '251-CSb67 were releasedby trypsin.' These data were interpreted as evidence that the CSb6 was on the surface of the cell, while theCSb67wasinserted in the plasma membrane and partially shielded from proteolysis by trypsin. In terms of its susceptibility to trypsin digestion, the iCSb67 behaved like CSb6 (Fig 2), which is outside the cell. Thus, our results s*.ronglysuggest that iCSb67 binds to the cell surface, perhaps to a specific cell receptor. The degree of proteolysis of CSb67 was comparable with that previously reported. In retrospect, the reported cpm released by trypsin from E-'2sI-CSb67,as well as our current result, may overestimate the accessibility of the complex. We now know that when the CSb67 is formed, some may insert, and the iCSb67 1.6 to iCSb67, and significantly inhibited the PMN response to FMLP. Thus, iCSb67, like CSa and FMLP mustuse a G protein for signaling. iC5b67 Induces the CO'+ Flux of PMN Ca'+ fluxes are an integral part of many signaling pathways used by the PMN and other cells. Indo-I -loaded PMN were tested for their ability to generate a Ca'+ flux when challenged with iCSb67. The Ca" flux was determined by the ratio of fluorescent intensity (405/48S)as measured by flow cytometry (Fig 9). Over the 1.4 X 10"' to 2.8 X IO"' mol/L range tested, iCSb67induced a dose-dependent increase in the Ca'+ flux of PMN. h m CO 2 Ln 0 d v 2 1.4 v) C Q) c. c c C Q) 0 v) g 1.2 3 G .c 0 DISCUSSION .-0 c. (d We have confirmed that newly formed C5b67 decays rapidly to a hemolytically inactive form, designated iC5b67 (Fig l ) . Using a different experimental method, the half-life previously reported was calculated to be less than 0.1 second.' Because the formation of CS67 is rate-limiting, experimental conditions that would favor the formation of CSb67 would shorten the apparent half-life of nascent CSb67.I' The important consideration for the interpretation of our results is the confirmation that the CSb67 complex we formed using a twofold molar excess of C7 is labile with respect to its hemolytic activity. Although the decay rate we measured was at 37°C and we routinely stored our C5b67 at 4"C, we confirmed that ligand, which was active for PMN,hadno hemolytic activity. U 1.o 10-1 1o4 l og Doses of iC5b67 (M) Fig 9. iC5b67 induces a Ca" flux in PMN. PMN (1 x lO'/rnL HBSA2*) were incubated with Indo-l AM (5 pmol/Ll at 37°C for 7 minutes. The cells were treatedas described in Materials and Methods. PMN suspension (100 pL) wasadded t o prewarmed HBSS" and 50 p L various doses of iC5b67 were added t o PMN. The change of fluorescent intensity within thePMN of the fluorochrome was monitored for 5 minutes by FACS. The fluorescent intensity ratio ofCa2+ bound/Caz' free (405/485), which reflects Ca2' flux, was calculated from the curves. iC5b67 induced a Ca" flux, and the magnitude of the positive flux was dose-dependent. The experiment is representative of three performed. From www.bloodjournal.org by guest on November 18, 2014. For personal use only. 2577 COMPLEMENT iC5b67: A N AGONIST FOR PMN which is also formed may associate with the cell surface and be accessible to trypsin degradation. The iC5b67 ligand exhibited divergent biologic activities. iC5b67 did not stimulate PMN superoxide production, rather it inhibited it. This was surprising because C5b-9 has been reported to stimulate superoxide prod~ction.’~ By contrast, we found that a preparation of C5b-9 made from purified C5b6 and terminal components also inhibited superoxide formation, but the active moiety was iC5b67 (C. Wang and A. Nicholson-Weller, unpublished results, July 1994). We propose that the differences in activity may be due to our use of purified components, compared with the use of terminal complexes made from deficient sera. Many ligands can lead to the stimulation of superoxide production, including the chemotactic factors C5a and FMLP, which act through specific receptors, as iC5b67 was able to inhibit C5a- and FMLP-induced superoxide production (Fig 3). Neither C5b6, nor C7 could reproduce the activity of iC5b67 (Fig 4A), while anti-C7 could remove the activity (Fig 4B). All these data support the conclusion that the active ligand is iC5b67. Receptor pathways, which inhibit superoxide are not known, although the intracellular accumulation of CAMP is known to inhibit PMN superoxide prod~ction.~’ iC5b67, unlike C5a and FMLP22.23 did not cause the upregulation of CR1 and CR3 (Fig 5), which is a second example of this complement complex evoking a different response than C5a and FMLP. iC5b67 did stimulate chemotaxis (Fig 6) with maximal activity at m o a , and displayed inhibition at higher doses. An assay to test for directed versus random migration confirmed that iC5b67 primarily stimulated directed migration (Table l). These results are important for two reasons: first, they confirm the original reports of iC5b67 chemotactic activity6,’; second, they indicate that the ability of iC5b67 to inhibit superoxide production and its failure to stimulate complement receptor upregulation are not the result of a general suppression of all cell functions. The ability of iC5b67 to inhibit C5a and FMLP directed chemotaxis (Fig 7) may be via activation of the same pathway that inhibited C5a and FMLP-induced superoxide production, or it may reflect cross-deactivation. Cross-deactivation occurs when the same signaling machinery is used by two receptors, and the first receptor activated preempts the ability of the second ligand to signal, as reviewed.26The FTX susceptibility of iC5b67 induced chemotaxis (Fig 8) indicates that this ligand uses G, proteins for signaling. However, the finding of iC5b67 chemotactic activity in vitro may not mean that this is the principal function of this ligand in vivo. For example, c-kit is chemotactic for mast cells, but its primary function is apparently as a growtwdifferentiation factor.28 Indo- 1-loaded PMN showed a iC5b67 dose-dependent Ca2+flux (Fig 9). Ca2+fluxes are common to many signaling pathway^,'^ and further studies will be necessary to define if the Ca2+is coming from intracellular or extracellular stores. We have shown in these studies that iC5b67 is active in signaling PMN. Further studies will be necessary to define if iC5b67 occurs in vivo, andif the binding of vitronectin (Sprotein) or the later complement components affects iC5b67 signaling. The fact that iC5b67 is potent at nanomolar concentrations suggests that it, like other potent complement ligands including C3a and C5a, may have a short half-life. To date, active terminal complement complexes have included membrane inserted C5b67, C5b-8, and C5b-9 complexes. Now we have also reconfirmed that iCSb67 has biologic activity, and that unlike the other terminal complement complexes, it apparently signals from outside the cell, rather than by insertion through the bilayer. We do not know if it is acting through a specific receptor. It is interesting that the changes in light polarization could be inhibited by PTX, making iC5b67 similar to signaling by C5a and FMLP, yet in the assays of superoxide and CRlKR3 upregulation, iC5b67 evoked different responses. Preliminary data indicate that multiple signaling pathways are triggered by this ligand (C. Wang and A. Nicholson-Weller, manuscript in preparation). Finally, the fact that iC5b67 is large, hydrophobic, and has some proinflammatory andsome antiinflammatory activities, while C5a is small, hydrophilic, and is only proinflammatory, suggests that these two ligands have differing roles in the initiation and regulation of inflammation. REFERENCES 1. Gotze 0, Muller-Eberhard HJ: Lysis of erythrocytes by complement in the absence of antibody. J Exp Med 132:898, 1970 2. 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