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Báo cáo khoa học: A steady-state competition model describes the modulating effects of thrombomodulin on thrombin inhibition by plasminogen activator inhibitor-1 in the absence and presence of vitronectin ppt

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Results TM is an effective inhibitor of the thrombin interaction with PAI-1 in the absence and presence of VN Earlier studies both from our group and others have shown that the rate ofth

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A steady-state competition model describes the modulating effects

of thrombomodulin on thrombin inhibition by plasminogen activator inhibitor-1 in the absence and presence of vitronectin

Rob J Dekker, Hans Pannekoek and Anton J G Horrevoets

Department of Biochemistry, Academic Medical Center, University of Amsterdam, the Netherlands

Thrombomodulin (TM) slows down the interaction rate

between thrombin and plasminogen activator inhibitor 1

(PAI-1) We now show that the 12-fold reduced inhibition

rate in the presence ofTM does not result from an altered

distribution between PAI-1 cleavage and irreversible

com-plex formation Surface plasmon resonance (SPR) revealed

an over 200-fold reduced affinity of TM for

thrombin-VR1tPA as compared to thrombin, demonstrating the

importance ofthe VR1 loop in the interaction ofthrombin

with both TM and PAI-1 Furthermore, in contrast to ATIII,

PAI-1 was not able to bind the thrombin/TM complex

demonstrating complete competitive binding between PAI-1

and TM Kinetic modeling on the inhibitory effect ofTM

confirms a mechanism that involves complete steric blocking

ofthe thrombin/PAI-1 interaction Also, it accurately

decribes the biphasic inhibition profile resulting from the

substantial reduction ofthe extremely fast rate ofreversible

Michaelis complex formation, which is essential for efficient inhibition ofthrombin by PAI-1 Vitronectin (VN) is shown

to partially relieve TM inhibitory action only by vastly increasing the initial rate ofinteraction between free thrombin and PAI-1 In addition, SPR established that solution-phase PAI-1/VN complexes and non-native VN (extracellular matrix form) bind TM directly via the chon-droitin sulphate moiety ofTM Collectively, these results show that VR1 is a subsite ofexosite 1 on thrombin’s surface, which regulates exclusive binding ofeither PAI-1 or TM This competition will be physiologically significant in con-trolling the mitogenic activity ofthrombin during vascular disease

Keywords: serine protease; serpin; suicide substrate mecha-nism; competitive inhibitor; kinetic modeling

Classically, the serine protease thrombin is known for its

dual role in hemostasis, exhibiting coagulant as well as

anticoagulant properties Reversible binding ofthrombin to

the endothelial cell surface cofactor thrombomodulin (TM)

endows thrombin with potent anticoagulant properties [1,2]

The thrombin/TM complex is no longer able to bind and

cleave fibrinogen and various other substrates and inhibitors

but becomes a potent activator ofprotein C The catalytic

activity ofthrombin can be inhibited by a number ofserine

protease inhibitors (serpins), including antithrombin III (ATIII), heparin cofactor II, and PAI-1 Inactivation of thrombin by PAI-1, however, is a very inefficient process with a second-order rate constant (ki) of 103M )1Æs)1, which can be increased up to 250-fold by the cofactors vitronectin (VN) and heparin [3,4]

The VR1- or 37-loop ofthrombin has been implicated in

a number ofintriguing interactions First, substitution ofthe VR1-loop ofthrombin by that oft-PA, yielding thrombin-VR1tPA, increases the bimolecular rate constant ofinhibi-tion by PAI-1 at least 1000-fold to 106M )1Æs)1[5] Recently,

we reported that this alteration results from an increased rate ofa unimolecular catalytic step [6] It has been unambiguously evidenced that VR1 is essential for the interaction ofboth t-PA [7] and thrombin [5,6] with PAI-1 Second, binding kinetics and structural studies have esta-blished that the epidermal growth factor domains 4–6 (EGF4–6) ofTM bind thrombin electrostatically at exosite

1 [8], but are also involved in hydrophobic contacts with VR1-loop residues [9,10] As a result, a marked influence of

TM binding on the interaction ofPAI-1 and thrombin can

be envisioned The hirudin-derived decapeptide hirugen, however, also interacts with thrombin by utilizing exosite I [1,2,11], although it did not prevent binding ofPAI-1 but altered a catalytic step ofthe reaction between thrombin and PAI-1 [6]

TM acts as a positive effector on other thrombin interactions, e.g with ATIII, protein C inhibitor, thrombin-activatable fibrinolysis inhibitor (TAFI) and protein C

Correspondence to A J G Horrevoets, Academic Medical Center,

Department ofBiochemistry, Room K1-161, Meibergdreef15,

1105 AZ Amsterdam, the Netherlands.

Fax: + 31 20 6915519, Tel.: + 31 20 5665153,

E-mail: a.j.horrevoets@amc.uva.nl

Abbreviations: VR1, variable region-1 (also 37-loop); t-PA, tissue-type

plasminogen activator; u-PA, urokinase-type plasminogen activator;

VR1 tPA , VR1 loop oft-PA; serpin, serine protease inhibitor; PAI-1,

plasminogen activator inhibitor type 1; RCL, reactive center loop;

PPACK, Phe-Pro-Arg-chloromethylketone; VN, vitronectin;

TM, thrombomodulin; rl-TM, rabbit-lung thrombomodulin;

solulin, soluble human recombinant thrombomodulin; SPR, surface

plasmon resonance; ATIII, antithrombin III; k i , second-order rate

constant ofinhibition; r, partition ratio; k on , association rate constant;

k off , dissociation rate constant; K d , thermodynamic equilibrium

dissociation constant.

(Received 15 October 2002, revised 24 December 2002,

accepted 3 March 2003)

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[11–13] However, previous work from our laboratory has

demonstrated that thrombin inhibition by PAI-1/VN

com-plexes is impaired in the presence ofTM [4] These findings

have later been studied in more detail, though the

mecha-nism ofTM interference in this interaction has not yet been

elucidated, nor is any evidence available on the possible

physiologic role [14,15] Interestingly, using

immunohisto-chemistry, TM antigen was demonstrated on vascular

smooth muscle cells (SMC), monocytes, and macrophages

in atherosclerotic lesions ofthe human and rabbit aorta [14]

Also, due to the colocalization ofthrombin, PAI-1 and VN

in the vessel wall, increasing attention is being paid to the

mitogenic effect of thrombin, and its control by PAI-1/VN

in the pathogenesis ofvascular disease [16,17] Together, the

presence ofPAI-1, VN and TM in the vessel wall, including

the unique property ofthrombin to inactivate PAI-1,

suggests a novel role ofTM in controlling the behavior of

vascular cells

Here, we report that binding ofTM and PAI-1 to

thrombin is mutually exclusive, both in the presence and

absence ofVN Furthermore, the data presented here is in

agreement with a mechanism in which the rate ofthrombin

inhibition by PAI-1 is dependent on the rate ofdissociation

ofthrombin from TM, explaining the observed biphasic

inhibition profiles These findings are in marked contrast to

the binding ofall other thrombin-binding components, and

comprise yet another level ofspecificity switching of

thrombin that is controlled by TM

Materials and methods

Materials

The chromogenic substrate H-D-Phe-Pip-Arg-p-nitroaniline

(where Pip is l-pipecolic acid; S2238) was obtained from

Chromogenix (Mo¨lndal, Sweden) All additional chemicals

were obtained from Sigma (St Louis, MO, USA)

Poly-sorbate-20 (Surfactant P20), and all additional BIAcore

materials were obtained from BIAcore AB (Uppsala,

Sweden)

Proteins

Ovalbumin (grade V) was obtained from Sigma

Rabbit-lung TM (rl-TM) was purchased from American

Diagnos-tica Inc (Lot #970117A, Veenendaal, the Netherlands)

Recombinant soluble human TM (solulin) was a gift of

J Morser (Berlex Biosciences, Richmond, CA, USA)

Active PAI-1 was generously provided by T M Reilly

(Dupont de Nemours, Wilmington, DE, USA) Human

a-thrombin purified from plasma was a gift of G Tans

(University ofMaastricht, the Netherlands) Construction,

expression, and activation ofrecombinant prothrombin

variants were described [6] VN was a kind gift of K T

Preissner (Justus Liebig University, Giessen, Germany)

Antithrombin III was obtained from the Sanquin

Founda-tion (CLB, Amsterdam, the Netherlands)

Determination of the PAI-1 inhibition rates

To prevent protein adsorption, all experiments were

performed in Eppendorftubes or in wells ofa microtiter

plate (Nunc Maxisorp; Gibco-BRL, Gaithersburg, MD, USA) that had been pretreated for 1 h at 37C with 1% (w/v) polyethylene glycol 20 000 and subsequently washed with distilled water Prior to all experiments, PAI-1 dilutions were titrated on a calibrated t-PA standard The decrease of thrombin amidolytic activity during the inhibition by PAI-1 was determined after incubating 15 nM thrombin with 1.5 lM PAI-1 at 37C in HBSO buffer (20 mM Hepes,

pH 7.4, 150 mM NaCl, and 0.5 mgÆmL)1ovalbumin) At specific time intervals, aliquots of5 lL were withdrawn and the reaction was quenched by diluting 45-fold in HBSO buffer containing 0.65 mMofS2238 chromogenic substrate Residual thrombin amidolytic activity in these aliquots was measured at 37C by continuously recording the absorb-ance at 405 nm in a Titertek Twinreader (Flow Laborat-ories, Irvine, UK) Plots ofresidual activity (relative to thrombin activity in the absence ofPAI-1) vs time were constructed and analyzed as described [6] The effect of increasing concentrations ofsolulin on the inhibition of thrombin and thrombin-VR1tPAby PAI-1 was determined

To that end, a solution of15 nM human a-thrombin was prewarmed in NaCl/Pi/Tween buffer [NaCl/Piwith 0.01% (v/v) Tween 80] for 5 min at 37C, in the presence of increasing concentrations ofsolulin (0–800 nMin NaCl/Pi/ Tween buffer) or rl-TM (0–100 nM in 20 mMTris buffer,

pH 7.4, with 100 mM NaCl) Subsequently, the inhibition reaction was started by the addition ofPAI-1 to a final concentration of1.5 lM At various time intervals, the residual thrombin amidolytic activity was determined as described above Alternatively, the inhibition of2 nM thrombin-VR1tPAby 10 nMPAI-1 was determined in the absence or presence of800 nM solulin Therefore, 13 lL aliquots were quenched by diluting ninefold in HBSO buffer, containing 0.9 mMofS2238 chromogenic substrate Surface plasmon resonance (SPR) binding studies Reversible binding ofvarious components was studied using SPR in a BIAcore 2000 system (BIAcore AB, Uppsala, Sweden) Binding experiments were performed using CM5 Sensor Chips (BIAcore AB) at 25.0C All recorded sensorgrams were corrected for refractive index variations, using an empty flow cell Thrombin-S195A and thrombin-S195A-VR1tPA were immobilized on a sensor chip as described for thrombin [18] Thrombin was immobilized at

40 ngÆlL)1in 10 mMsodium acetate buffer (pH 6.0), rl-TM was immobilized at 15 ngÆlL)1in 10 mMsodium formate buffer (pH 3.6), and vitronectin was immobilized at

120 ngÆlL)1 in 10 mM sodium acetate buffer (pH 4.8), resulting in approximately 4000, 2000, and 17 000 immobi-lized resonance units, respectively In all SPR experiments, HBS buffer [20 mMHepes, pH 7.4, 150 mM NaCl, 2 mM CaCl2, 0.005% (v/v) P20] was used at a 20-lLÆmin)1flow rate Binding ofthrombin and thrombin-VR1tPAto immobi-lized rl-TM was monitored by applying either thrombin (0.5–20 nM), or thrombin-VR1tPA (10–100 nM) in HBS buffer, at 20 lLÆmin)1 Association and dissociation rate constants were determined from the SPR sensor grams by global nonlinear regression using theBIAEVALUATION soft-ware (BIAcore AB) Binding ofvarious analytes to rl-TM/ thrombin complexes was studied as follows: 10 lL of

200 n human recombinant a-thrombin in HBS buffer was

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injected on a sensorchip with immobilized rl-TM, directly

followed by a 40-lL injection (using the coinject option) of

the respective proteins or HBS buffer alone Hereafter,

dissociation ofthrombin and bound analyte from TM was

continuously monitored in HBS buffer

Direct binding to rl-TM of200 nMPAI-1, 200 nMlatent

PAI-1, 300 nM VN, or 200 nM active or latent PAI-1

preincubated with 75–300 nMVN was studied by injecting

60 lL ofthe respective proteins in HBS buffer Latent PAI-1

was obtained by incubating 200 nMPAI-1 at 37C f or at

least 20 h

Direct binding ofrl-TM and solulin to VN was studied by

injecting 40 lL 200 nM rl-TM, or 1 lM solulin, in the

absence or presence ofheparin (0–1000 UÆmL)1)

Alternat-ively, previous to the TM injections, 40 lL 500 nMPAI-1

solution was injected to form PAI-1/VN complexes on the

chip surface Hereafter, during the slow dissociation of

PAI-1/VN, rl-TM or solulin was injected as described above

Kinetic modeling

The procedure ofnumerical integration ofthe rate

equa-tions derived from the mechanism shown below has been

described elsewhere, including the rate constants ofthe suicide-substrate mechanism (k1 to k3) that were used [6] Briefly, at various combinations of kon and koff for the thrombin/TM interaction, the total thrombin amidolytic activity was calculated at various time intervals, i.e the sum

of actual free thrombin, thrombin/TM complex, and free thrombin resulting from completion of all thrombin/PAI-1 intermediates after quenching of the reaction The throm-bin/TM complex has a similar amidolytic activity towards S2238 as thrombin (data not shown) For all combinations

of konand koff, the calculated total thrombin activity was compared to the experimental activity decrease shown in Fig 1A

Results

TM is an effective inhibitor of the thrombin interaction with PAI-1 in the absence and presence of VN

Earlier studies both from our group and others have shown that the rate ofthrombin inhibition by PAI-1 is significantly reduced in the presence ofTM [4,15] In this study, we performed quantitative measurements of this inhibition

Fig 1 Effect of TM and VN on the rates of thrombin inhibition by PAI-1 Residual thrombin amidolytic activity was measured at various time intervals and used to calculate the halftimes (t 1/2 ) ofPAI-1 inhibition (A) Residual activity was monitored during the inhibition of15 n M human thrombin by 1.5 l M PAI-1, in the absence (d) or presence of 30 n M (s), 50 n M (j), 100 n M (h), 400 n M (m) or 800 n M (n) TM (solulin) (B) Residual thrombin activity was monitored as in (A), but in the presence of0 (d), 10 (s), 20 (j), 50 (h) or 100 (m) nM rl-TM (C) Residual activity decrease was monitored during the inhibition of2 n M thrombin-VR1tPAby 15 n M PAI-1 in the absence (d) or presence of100 n M rl-TM (s) (D) Thrombin activity decrease that was observed during the inhibition of15 n M thrombin by 100 n M PAI-1/VN complexes, in the absence (d)

or presence of100 n M rl-TM (s).

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Furthermore, we attempted to elucidate the mechanism of

interference by TM on thrombin inhibition by PAI-1 The

rate ofthrombin inhibition by 1.5 lMPAI-1 was measured

in the presence ofincreasing concentrations (0–800 nM) of

solulin Solulin lacks the transmembrane domain and does

not contain chondroitin sulphate, which might have an

additional heparin-like effect on the thrombin/PAI-1

inter-action [19] In this way, only protein–protein interinter-actions

between thrombin and TM are considered At the highest

concentration ofTM (800 nMsolulin), thrombin is inhibited

by PAI-1 with a half-time of the reaction (t1/2) that is

12.5-fold longer than in the absence of TM, i.e 6810 and 545 s,

respectively (Fig 1A) Because ofthe lower affinity of

solulin for thrombin (due to the lack of the chondroitin

sulphates) [20], high concentrations ofthe TM preparation

were necessary to obtain the observed effect Hence,

rabbit-lung TM (rl-TM), which has a higher affinity for thrombin

due to its chondroitin sulphate moiety, was also used to

study the effect on the thrombin/PAI-1 interaction The

inhibitory effect of only 100 nM rl-TM on the rate of

inhibition ofhuman plasma thrombin by PAI-1 was more

substantial than that ofthe highest concentration ofsolulin

(800 nM) (Fig 1B) Next, the effect of TM on the inhibition

ofthe substitution variant thrombin-VR1tPAby PAI-1 was

studied In marked contrast to thrombin, only a 1.7-fold

inhibitory effect of 100 nM rl-TM is observed on the

inhibition of2 nM thrombin-VR1tPA by 15 nM PAI-1,

measured as difference of the half-time (t1/2) of the reaction

(Fig 1C) Opposed to the accelerating effect of TM on

thrombin inhibition by ATIII and protein C inhibitor, these

findings indicate that TM considerably reduces the rate of

thrombin inhibition by PAI-1

The poor rate ofinhibition ofthrombin by PAI-1 alone

(ki  103

M )1Æs)1) can be substantially increased by the

cofactor VN [3] Complexed to VN, PAI-1 inhibits

throm-bin at a rate that is at least two orders ofmagnitude higher

compared to PAI-1 alone (ki 105

M )1Æs)1) Therefore, the inhibitory effect ofTM on the inhibition of15 nMthrombin

by preincubated PAI-1/VN complexes (100 nM PAI and

150 nM VN) was determined (Fig 1D) The presence of

100 nMrl-TM in this reaction decreased the inhibition rate

by 14-fold (t1/2¼ 940 and 67 s, respectively) Still, even in

the presence ofrl-TM, VN accelerates the rate ofthrombin

inhibition by PAI-1 36-fold [Fig 1B (m) vs 1D (s)]

TM binding to thrombin-VR1tPAis substantially

reduced

The minor effect of TM on the rate of thrombin-VR1tPA

inhibition by PAI-1 suggests that the binding between

thrombin and TM, involving the VR1 loop ofthrombin, is

affected in the substitution variant Indeed, the rate of

protein C activation by thrombin-VR1tPA, which is

com-parable to that of thrombin, was not affected by TM,

whereas TM substantially increased the rate ofprotein C

activation by thrombin, as expected (data not shown) The

affinity of TM for thrombin-VR1tPAwas determined using

Surface Plasmon Resonance (SPR, data not shown)

Binding ofthrombin-VR1tPA to immobilized rl-TM was

significantly reduced (Kd¼ 121 ± 23 nM) compared to

thrombin (Kd 0.5 nM) [2,11] Thus, the minor effect of

TM on the thrombin-VR1tPA–PAI-1 interaction appears to

be the result ofthe decreased ability ofTM to bind thrombin-VR1tPA Moreover, these results demonstrate that, as for PAI-1, the VR1 loop of thrombin is an essential interaction site for TM

The stoichiometry of the suicide-substrate mechanism is not influenced by TM The kinetics ofthe inhibition ofthrombin by PAI-1 can be described by the so-called suicide-substrate mechanism as previously elaborated by our group [6,21] In this mecha-nism, each productive encounter ofserpin and protease can either lead to formation of the enzyme/inhibitor complex or can result in cleavage ofthe inhibitor and release ofactive enzyme A decreased overall inhibition rate can thus be the result ofa shift ofthe rate constants ofthe branched part of mechanism, i.e increased cleavage at the expense of complex formation Therefore, the products of the reaction were analyzed by SDS/PAGE (Fig 2) We found no evidence ofincreased cleavage indicating that TM does not alter the product distribution ofthe suicide-substrate reaction between thrombin and PAI-1 These findings leave

a role for TM open in altering the initial binding step between thrombin and PAI-1 or in changing the ability of thrombin to catalyze subsequent steps that are common

to both branches in the mechanism, i.e steric hindrance

vs allosteric modulation, respectively

PAI-1 and TM compete for an overlapping binding site on thrombin

At this point, the mechanism of the inhibitory effect of TM

on the interaction between PAI-1 and thrombin remains to

be elucidated To that end, binding ofPAI-1 to immobilized thrombin/TM complexes was studied in real-time using SPR The high rate constant ofinitial thrombin/PAI-1 complex formation (k1 106m)1Æs)1) [6] would result in a significant increase in surface-bound mass if formation of

Fig 2 TM does not alter the distribution of the cleavage and substrate pathway Analysis by SDS/PAGE ofthe products ofthe reaction of

600 n M thrombin with 3.5 l M PAI-1 in the presence of0 (lanes 2–3),

430 (lanes 1, 4–5), 630 (lanes 6–7), and 885 (lanes 8–9) nM TM (sol-ulin) After 0 min (lane 1), 3 min (lanes 2, 4, 6 and 8) or 16 h (lanes 3, 5,

7 and 9) the samples were immediately quenched by adding sample buffer, subjected to 10% (w/v) SDS/PAGE and stained with Coo-massie Brilliant Blue Indicated are free thrombin (T), intact PAI-1 (P), cleaved PAI-1 (P*), SDS-stable thrombin/PAI-1 complex (T-P), and thrombomodulin (TM), as described in the legend ofFig 4 Note that after 16 h incubation, the thrombin–PAI-1 complexes formed in the presence ofTM (lanes 5, 7 and 9) have mostly been degraded to lower molecular mass species by the remaining free, active thrombin as noted before [21].

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ternary thrombin/PAI-1/TM complexes occurs First,

thrombin/TM complexes were formed by applying a

solution of200 nMthrombin on rl-TM that was

immobi-lized on a sensor chip A high affinity interaction between

thrombin and TM was observed, consistent with a

disso-ciation constant ofthe thrombin/TM complex in the

subnanomolar range [2,11] Binding was mass

transport-limited, precluding exact determination ofthe rate constants

for this interaction under these conditions Alternatively, an

estimate of koffcan be given using the halftime

ofthrom-bin/TM dissociation, i.e t1/2¼ 470 s and koff 10)3Æs)1

Second, immediately following the thrombin injection,

either a solution of800 nM PAI-1, 800 nM latent PAI-1

or buffer was applied to study the formation of ternary

TM/thrombin/PAI-1 complexes on the chip surface

How-ever, thrombin slowly and continuously dissociated from

the immobilized TM at the same rate as in the absence of

PAI-1 and no increase in surface-bound mass was observed

as a result ofPAI-1 binding to TM/thrombin complexes

(Fig 3A) Also, no significant difference in binding between

the active and latent form of PAI-1 was observed, the latter rendered unable to bind thrombin The absence ofternary complex formation implies that TM has a steric effect on the interaction between thrombin and PAI-1 An allosteric effect of TM is not consistent with these results as the formation of ternary complexes, that would be more slowly converted to stable protease/serpin complexes due to TM-induced allosteric changes in thrombin, is not observed, even at the high concentrations ofPAI-1 used

As a positive control, identical experiments were per-formed with ATIII that inhibits thrombin at a rate comparable to PAI-1, and in contrast to PAI-1 is known

to inhibit the thrombin/TM complex even slightly more efficient than thrombin alone [11] Injection of ATIII after formation of thrombin/TM complexes on the chip surface, resulted in a considerably increased dissociation ofthrom-bin from TM, depending on the ATIII concentration that was used (Fig 3A) These findings are in agreement with fast binding of ATIII to thrombin/TM followed by rapid dissociation ofthe thrombin/ATIII complex from TM,

Fig 3 TM and PAI-1 competitively bind thrombin Binding ofvarious proteins to immobilized rl-TM was studied using SPR (A–C) Plots show the increase in surface-associated mass (D Resonance Units) measured in real-time, resulting from binding to rl-TM that was immobilized on the sensor chip surface (A) At 0 s 10 lL 200 n M recombinant thrombin was injected directly followed (at 30 s) by 40 lL 800 n M active PAI-1 (––), 800 n M

latent PAI-1 (- - -), buffer (ÆÆÆ), 600 n M (-Æ-) or 6 l M (-ÆÆ) ATIII Hereafter, dissociation was continuously monitored by injecting buffer alone (beyond

150 s) (B) Again, 10 lL 200 n M rII A was injected directly followed by 40 lL 800 n M active PAI-1 (ÆÆÆ), 300 n M VN (—), 200 n M latent PAI-1 preincubated with 300 n M VN (- - -), or 200 n M active PAI-1 preincubated with 75–300 n M VN (–– labeled 75–300) (C) 60 lL ofthe following solutions was directly applied (at 0 s) to the immobilized rl-TM in the absence ofthrombin, and dissociation was monitored under continuous buffer flow (180 s and beyond): 800 n M active PAI-1 (ÆÆÆ), 300 n M VN (- - -), 200 n M latent PAI-1 preincubated with 300 n M VN (––), or 200 n M

active PAI-1 preincubated with 300 n M VN (-Æ-) (D) Alternatively, direct binding ofrl-TM to VN was studied by immobilizing VN on a sensor chip Next, at 0 s 40 lL 200 n M rl-TM was directly injected in the absence (––) or presence of200 (- - -), 500 (-Æ-) or 1000 (ÆÆÆ) UÆmL)1heparin Subsequently, dissociation was monitored under continuous buffer flow (beyond 120 s).

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resulting in a net decrease in TM-associated mass on the

chip surface Moreover, these data are consistent with

the previously described strong reduction ofthe affinity of

the thrombin/ATIII complex for TM [11,22]

The PAI-1/VN complex directly binds to TM

Recently, we have shown that the PAI-1/vitronectin

com-plex can be treated as an entity with different kinetic

properties than PAI-1 alone [21] The finding that the PAI-1/

VN complex still inhibits the thrombin/TM complex at a

36-fold higher rate than thrombin alone, suggests that a

possibly different binding mode of PAI-1/VN to thrombin

might allow the formation of quaternary TM/thrombin/

PAI-1/VN complexes Therefore, binding of PAI-1/VN to

TM/thrombin complexes was tested using SPR Increasing

concentrations ofVN (0–300 nM) were preincubated with

200 nMPAI-1 and binding to preformed thrombin/rl-TM

complexes was monitored (Fig 3B) In contrast to PAI-1

and ATIII, binding ofPAI-1/VN to the chip surface was

observed without an apparent increased dissociation of

TM-bound thrombin Interestingly, neither active PAI-1 or

VN alone, nor latent PAI-1 preincubated with VN, had a

significant effect on the thrombin–TM dissociation rate

However, direct binding ofactive PAI-1/VN complexes to

rl-TM was observed making a possible interaction between

PAI-1/VN and TM-bound thrombin unlikely (Fig 3C)

Again, the observed binding was specific for active PAI-1

when preincubated with VN, as no binding was observed

for latent PAI-1 in the presence of VN Additional binding

studies demonstrate that rl-TM (glycosylated), but not

solulin (not glycosylated) binds directly to immobilized VN,

even in the absence ofPAI-1 (data not shown) Therefore,

binding ofVN to rl-TM occurs either directly to

immobi-lized VN or to VN in solution exclusively when bound to

active PAI-1 The latter is in agreement with the inability of

latent PAI-1 to bind VN [23]

Finally, the lack ofthe chondroitin sulphate moiety on solulin, in conjunction with its inability to bind PAI-1/VN complexes, suggests the involvement ofthe chondroitin sulphate ofrl-TM in the binding ofPAI-1/VN Indeed, both in the absence and presence ofPAI-1, the inter-action ofrl-TM with immobilized VN could be com-peted by including increasing concentrations ofheparin (200–1000 UÆmL)1) in the SPR experiments (Fig 3D)

In conclusion, these results suggest a mechanism in which TM sterically blocks both PAI-1 and PAI-1/VN complexes in the association with thrombin In addition, upon binding PAI-1, VN is able to bind the chondroitin sulphate moiety ofrl-TM independent ofTM-bound thrombin

Kinetic modeling using TM as a competitive inhibitor correctly predicts the inhibitory effect of TM

We decided to model the kinetics ofthe modulating effect of

TM on the thrombin/PAI-1 reaction to supply a mecha-nistic basis for the multicomponent reactions Solulin kinetic and binding data were used throughout the initial modeling, as the equilibrium binding ofrl-TM to thrombin displayed a sigmoidal character due to the cooperative effects of both protein/protein and protein/glycosamino-glycan interactions Complete sterical blocking ofthe thrombin/PAI-1 interaction would suggest the ability of

TM to completely prevent the inhibition ofthrombin by PAI-1 at high TM concentrations However, this is not observed experimentally at TM concentrations that are several fold higher than Kd(rl-TM 200-fold; solulin > 10-fold) An indication that could explain this apparent discrepancy is our previous description ofthe high reversible association rate ofthrombin and PAI-1 [6] The reaction scheme in Fig 4 implies that at infinite TM concentration all thrombin is in complex with TM, and the inhibition of thrombin activity by PAI-1 is thus dependent on the

Fig 4 Competitive mechanism of TM inhibition of the thrombin/PAI )1 interaction The inhibitor PAI-1 (P) forms a reversible Michaelis-type complex (TP) with thrombin (T), characterized by the bimolecular association rate constant k 1 and the dissociation rate constant k)1 Subsequently,

an intermediate irreversible complex (TP¢) is formed with rate constant k 2 , that can convert with a rate constant k 3 into the SDS-stable complex (T-P), or it can react according to a substrate mechanism, resulting in the release offree enzyme together with cleaved, inactive inhibitor (P*) with the rate constant rÆk 3 The partition ratio (r) represents the number ofcatalytic turnovers per inactivation event, where 1 + r is the apparent stoichiometry The rate ofPAI-1 conversion to its latent form (PL) is described by the rate constant k L Alternatively, thrombin binds to TM forming a reversible complex (T-TM) described by the association and dissociation rate constants k and k , respectively.

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dissociation rate ofthrombin/TM complexes Upon

disso-ciation ofthe thrombin/TM complex, there will be

compe-tition between PAI-1 and TM for (re)associating with

thrombin Competition is dependent on the second-order

rate constants for the thrombin/PAI-1 and thrombin/TM

interaction, including the actual concentration ofTM and

PAI-1 throughout the course ofthe reaction Therefore, an

infinite concentration ofTM would completely compete

PAI-1 binding to thrombin To test this concept, numerical

integration ofthe rate equations that describe the

mechan-ism in Fig 4 was perf ormed to obtain theoretical insight

into the effect of the second-order rate constants and

concentrations ofTM and PAI-1 on the rate ofthrombin/

PAI-1 complex formation For various combinations of kon

and kofffor the thrombin/TM interaction, the concentration

ofall reactants and intermediates was calculated

through-out the course ofthe reaction The model fits the experi-mental data best when kon¼ 3 · 104

M )1Æs)1 and

koff¼ 1 · 10)3Æs)1 (data not shown) The Kd for solulin ( 30 nM) that was derived from these values is in good agreement with literature [24] The total thrombin amido-lytic activity that is thus predicted was compared to the experimentally observed decrease in thrombin activity (Fig 5A) The modeling adequately predicts the effect of

TM on the thrombin/PAI-1 inhibition kinetics The extremely fast formation of the initial thrombin/PAI-1 Michaelis-type complex, which is predicted during the presteady state phase ofthe reaction, is dependent on the starting concentration offree thrombin molecules (which itselfis dependent on the total TM concentration) (Fig 5B) Consequently, the maximal concentration ofthis reversible intermediate determines the rate offormation ofthe first irreversible intermediate TP¢ and thus establishes the overall rate ofthrombin inhibition, i.e the rate at which TP disappears in time at steady state after the rapid initial increase The pronounced biphasic character ofthe inhibi-tion profiles in Fig 1A–C is explained by the presteady state and steady state phases ofthe reaction that are predicted by the modeling At presteady state free thrombin is quickly captured in the reversible TP complex, which accounts for the rapid decrease ofthrombin activity that is observed during the first minutes The second phase in the inhibition profiles describes the steady state phase ofthe reaction where thrombin is slowly released by TM and inhibited by PAI-1 The reduced affinity of TM for thrombin-VR1tPA results in a higher free thrombin concentration at the start of the reaction and thus a more prominent biphasic inhibition profile with a longer initial phase (Fig 1C) According to Fig 5B the difference in the maximal concentration of TP, which is reached in the absence or presence of800 nMTM,

is approximately 12-fold This value is in agreement with the inhibitory effect of TM that is observed experimentally Finally, a similar model describing an allosteric inhibitory

Fig 5 A computer-simulated competitive model correctly predicts the effect of TM on the thrombin/PAI-1 inhibition kinetics Computer-aided numerical integration was performed, using the method of Runge-Kutta, to predict the concentration ofall reactants and intermediates described in Fig 4 during the full time course of the inhibition reac-tion The rate constants k on and k off were fitted to the experimental data from Fig 1A All other rate constants have been described in a previous study [6] (A) The time-dependent decrease ofresidual thrombin activity predicted by the model fits closely to the experi-mental data Lines represent the residual thrombin activity that was calculated using the same set ofrate constants for all TM concentra-tions The lower panel shows the residuals ofthe fit expressed as the difference between the experimental and calculated values (B) Shows the calculated change in concentration ofthe thrombin–PAI-1 reversible Michaelis complex ([TP]), as predicted throughout the course ofthe reaction modeled in panel A The maximal amount ofTP complexes that is formed during the initial phase of the reaction (< 10 s) is reduced in a TM concentration-dependent fashion This decrease is related to the free thrombin concentration at the start of the inhibition reaction that is determined by the TM concentration and the

K d ofthe thrombin–TM complex Symbols are identical to Fig 1A Lines represent the TM concentration that was used, i.e 0 (––), 30 (– – –),

50 (- - -), 100 (-Æ-), 400 (-ÆÆ) and 800 (ÆÆÆ) n

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effect of TM, by allowing thrombin/PAI-1/TM complex

formation, did not fit the experimentally observed

TM-dependent decrease ofthe thrombin/PAI-1 interaction rate

(results not shown)

Discussion

TM functions as a powerful procoagulant to anticoagulant

specificity switch upon binding to thrombin with high

affinity [1,2] The binding of Na+to thrombin constitutes a

second switch that potently modulates the coagulant vs

anticoagulant functions of thrombin [25] The allosteric

effect that results from the binding of Na+makes thrombin

a significantly more efficient procoagulant [26] However, in

both its Na+-bound and Na+-free form thrombin has a

high specificity for protein C in the presence of TM The

data presented in this study demonstrates that the VR1 loop

ofthrombin, being in close vicinity ofthe compact

functional epitope for TM [27], is the subsite of exosite-1

that regulates exclusive binding ofeither PAI-1 or TM

Previous studies have unambiguously demonstrated that the

VR1 loop is responsible for the specific interaction of PAI-1

with t-PA [7] and thrombin [5,6,21] The dominant

contri-bution ofthe t-PA VR1 loop residues to inhibition by PAI-1

strongly suggests binding ofPAI-1 to VR1 residues in t-PA

and thrombin-VR1tPA In contrast, the interaction of

thrombin with ATIII is primarily determined by subsites

located in the Western-exit ofthrombin (hydrophobic

binding pocket/N-terminal subsites), which is located

distant from the VR1 loop on the opposite side of the

active-center [9,28,29] Interestingly, PAI-1 appears to be the

only serpin that utilizes the VR1 loop, in contrast to ATIII

and protein C inhibitor (PCI) In agreement with this

concept, the thrombin/TM complex can be efficiently

inhibited by ATIII and PCI [11,12] Moreover, TM acts

as a stimulator ofthrombin inhibition by these serpins, in

line with the cofactor effect of TM on protein C activation

The exclusive function of the VR1 loop is now further

supported by the results obtained with the exosite-binding

proteins TM and hirugen (this study and [6]) The possibility

ofternary complex formation between thrombin (–VR1tPA),

PAI-1 and hirugen demonstrates that PAI-1 does not bind

the part ofthe anion binding exosite-1 ofthrombin that is

utilized by hirugen as well as by TM [22,29,30] However, as

demonstrated in this study, binding ofTM does sterically

hinder PAI-1 binding to thrombin (–VR1tPA) Therefore,

the binding ofPAI-1 to thrombin either involves a small

part ofthe VR1 loop that is physically blocked by TM, or

the bulkiness ofTM bound to exosite-1 decreases the

accessibility ofthe VR1 loop for PAI-1 [9,10,27] Previous

Ala scanning mutagenesis studies have demonstrated that

the VR1 residues Phe34, Lys36, Pro37 and Gln38 are

involved in the binding ofTM to thrombin [27,31] These

residues therefore comprise the most likely overlapping

binding site for TM and PAI-1 on thrombin, as they are also

ofsubstantial importance to the inhibition ofthrombin by

PAI-1 [5,6,21] In addition, the reduced affinity of TM for

thrombin-VR1tPA is in agreement with the significant

contribution ofthis part ofthe VR1 loop to the binding

ofTM by thrombin Binding ofthe carboxy-terminal part

ofthe reactive center loop ofPAI-1 in the small cleft formed

by the 60-loop and VR1 loop can be envisioned The

kinetics ofthe interaction ofthrombin and TM were shown

to be governed by electrostatic interactions, explaining the fast association rates [8] This does not explain, however, the high affinity binding of TM to thrombin as shown by the slow dissociation rates observed in this study (koff

 10)3Æs)1; Fig 3) Structural arguments were put for-ward that a major hydrophobic interaction in this strong hydrophilic environment governs the specificity and tight-ness ofTM binding to thrombin [10] Hydrophobic residues ofTM are buried in a surface hydrophobic pocket that is partly formed by VR1 and exosite-1 This would explain the significantly reduced binding ofTM to thrombin-VR1tPAas compared to thrombin, despite the fact that according to the structure the lower, highly charged rim ofexosite-1 is unchanged [6] This hydrophobic interaction, involving Phe34 in the VR1 loop, would then exclude the interaction ofPAI-1 with the VR1 ifthrombin is bound to TM The kinetic model ofTM/PAI-1 competition for throm-bin described here has the following features Previous studies from our laboratory have shown that initial Michaelis complex formation is not the rate-limiting step

in the thrombin/PAI-1 reaction, but rather a unimolecular step in the mechanism (k2in Fig 4) These findings imply that with the high PAI-1 concentrations that were used to rapidly inhibit thrombin, a major fraction of the thrombin molecules is quickly forming a reversible Michaelis complex with PAI-1 during the initial phase ofthe reaction Subsequently, the formation of stable thrombin/PAI-1 complexes is dependent on the (rate-limiting) efficiency of successive catalytic events in the inhibition pathway (i.e k2 and k3in Fig 4) When the amidolytic activity ofthrombin

is assayed during the course ofthe reaction, quenching of the reaction mixture leads to dissociation ofthe majority of initial Michaelis complexes, and thus releases active throm-bin In the presence ofa theoretical infinite concentration of

TM, all thrombin would be in complex with TM at the start ofthe reaction When PAI-1 is added, competition between

TM and PAI-1 for thrombin will occur after dissociation of each thrombin/TM complex, which was thus far at equi-librium Consequently, the concentrations ofPAI-1 and

TM, including their rate constants ofassociation with thrombin, will determine the maximum rate at which the thrombin/TM complex will be irreversibly inhibited by PAI-1 (Fig 5) Under the experimental conditions used in this study, relatively low TM concentrations (< 1 lM) are sufficient to have most thrombin in complex with TM At the high PAI-1 concentration (> 1 lM) that was used, PAI-1 will compete efficiently with TM as k1> kon and [PAI-1] > [TM] Therefore, even though TM significantly slows down the thrombin/PAI)1 interaction, eventually thrombin will be completely inhibited by PAI-1 The inhibitory effect of TM on the thrombin/PAI-1 interaction

is partly masked in the presence ofthe cofactor VN when assayed kinetically ([15] and this study) The data presented

in this study demonstrate that only PAI-1/VN complexes and immobilized VN directly bind to TM, most likely via its glycosaminoglycan sugar moiety In contrast, free VN or PAI-1 does not bind to TM In agreement with this finding,

VN is known to expose a high-affinity heparin binding-site only after it is converted to its non-native (active) confor-mation, i.e when bound to PAI-1 or when immobilized on a surface [32] Therefore, the interaction between VN and TM

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has probably no effect on the rate of thrombin inhibition by

PAI-1 in the presence ofTM A slightly larger inhibitory

effect ofTM on the rate ofthrombin inhibition by PAI-1/

VN was observed compared to inhibition by PAI-1 alone,

i.e 14 vs 12-fold, respectively In the presence of TM,

however, thrombin is still inhibited 36-fold faster by the

PAI-1/VN complex compared to PAI-1 alone The vastly

increased association rate between thrombin and PAI-1/VN

would result in an immediate capturing ofany free

thrombin that is at equilibrium with the thrombin/TM

species by competing more efficiently for reassociating

with TM

The physiologic relevance ofTM interference in the

thrombin/PAI-1 interaction can possibly be found in the

(atherosclerotic) vessel wall, where all these proteins and

cofactors are present [17], including TM on vascular SMC

[14] Both thrombin and PAI-1 can substantially influence

migration and proliferation of vascular SMC, the latter

process via the protease-activated receptors, ofwhich

PAR1 was found to be expressed by SMC in vivo [33] In

this respect, the interplay between thrombin and PAI-1 in

the vessel wall has two f aces First, PAI-1 is able to inhibit

the mitogenic potential ofthrombin On the other hand,

cleavage and inactivation ofPAI-1 by thrombin controls

the urokinase-type plasminogen activator (u-PA)-mediated

migratory effect of PAI-1 on SMC The suicide-substrate

mechanism stoichiometry is rather unfavorable for the

thrombin/PAI-1 protease/serpin pair, especially in the

presence ofVN, being six inactivated (cleaved) PAI-1

molecules for each thrombin molecule that is inhibited

(r¼ 5) [21] Probably the main physiologic consequence of

this interaction is an inactivation ofthe PAI-1 pool in the

vascular wall by thrombin, making it no longer available

for interaction with u-PA and VN, which can explain part

of the effect of thrombin on the proliferation and

migration ofvascular SMC [4,34] In the context ofthe

vessel wall, TM might therefore function as a regulator of

PAI-1 inactivation by thrombin in the presence ofthe

abundant matrix protein VN The presence ofTM on the

surface of SMC might be important in focusing its

modulatory potential to the cell surface In this respect,

physiologic significance can be attributed to the binding of

VN in its unfolded conformation (i.e as adhered matrix

protein or in solution complexed to PAI-1) to the

chondroitin sulphate moiety ofTM as was observed in

this study Neointimal vascular SMC can thus focus TM

to sites where VN is present, e.g at the leading edge of

migration, and prevent local inactivation ofPAI-1 by

thrombin The ability ofPAI-1 to compete with the SMC

surface-exposed integrin avb3 and u-PA receptor for

binding VN therefore suggests a possible migratory role

ofTM in neointimal hyperplasia This concept is in

agreement with the expression ofTM by neointimal

vascular SMC that was found in vivo [14,34]

In conclusion, this study provides a mechanistic concept,

elucidating a multicomponent system ofproteases, serpins

and cofactors Again, TM acts as a molecular switch by

excluding an interaction between thrombin and PAI-1

thereby protecting the serpin from inactivation

Further-more, these findings propose a possible novel role for TM

expressed by vascular SMC in the pathogenesis ofvascular

disease

Acknowledgements

This work was supported by the Netherlands Heart Foundation, the Hague, by grant NHS 96.094 and the Molecular Cardiology Program grant M 93.007.

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