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Interaction of plasminogen activator inhibitor type-1PAI-1 with vitronectin Characterization of different PAI-1 mutants Nuria Arroyo De Prada1,*, Florian Schroeck1,*, Eva-Kathrin Sinner2

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Interaction of plasminogen activator inhibitor type-1

(PAI-1) with vitronectin

Characterization of different PAI-1 mutants

Nuria Arroyo De Prada1,*, Florian Schroeck1,*, Eva-Kathrin Sinner2, Bernd Muehlenweg1,3,

Jens Twellmeyer1, Stefan Sperl3, Olaf G Wilhelm3, Manfred Schmitt1and Viktor Magdolen1

1 Klinische Forschergruppe der Frauenklinik der Technischen UniversitaÈt MuÈnchen, Klinikum rechts der Isar, Germany;

2 Max-Planck-Institut fuÈr Biochemie, Martinsried, Germany; 3 Wilex AG, MuÈnchen, Germany

The serpin plasminogen activator inhibitor type 1 (PAI-1)

plays an important role in physiological processes such as

thrombolysis and ®brinolysis, as well as pathophysiological

processes such as thrombosis, tumor invasion and

metas-tasis In addition to inhibiting serine proteases, mainly

tissue-type (tPA) and urokinase-type (uPA) plasminogen

activators, PAI-1 interacts with di€erent components of

the extracellular matrix, i.e ®brin, heparin (Hep) and

vitronectin (Vn) PAI-1 binding to Vn facilitates migration

and invasion of tumor cells The most important

deter-minants of the Vn-binding site of PAI-1 appear to reside

between amino acids 110±147, which includes a helix E

(hE, amino acids 109±118) Ten di€erent PAI-1 variants

(mostly harboring modi®cations in hE) as well as wild-type

PAI-1, the previously described PAI-1 mutant Q123K, and

another serpin, PAI-2, were recombinantly produced in

Escherichia coli containing a His6 tag and puri®ed by

anity chromatography As shown in microtiter

plate-based binding assays, surface plasmon resonance and thrombin inhibition experiments, all of the newly generated mutants which retained inhibitory activity against uPA still bound to Vn Mutant A114±118, in which all amino-acids

at positions 114±118 of PAI-1 were exchanged for alanine, displayed a reduced anity to Vn as compared to wild-type PAI-1 Mutants lacking inhibitory activity towards uPA did not bind to Vn Q123K, which inhibits uPA but does not bind to Vn, served as a control In contrast to other active PAI-1 mutants, the inhibitory properties of A114±118 towards thrombin as well as uPA were signi®-cantly reduced in the presence of Hep Our results dem-onstrate that the wild-type sequence of the region around

hE in PAI-1 is not a prerequisite for binding to Vn Keywords: plasminogen activator inhibitor type-1; vitronectin; heparin; mutational analysis; surface plasmon resonance

The urokinase-type plasminogen activation system plays

an important role in tumor growth, invasion, and

metastasis The serine protease urokinase-type

plasmino-gen activator (uPA) activates plasminoplasmino-gen, the zymoplasmino-gen

of plasmin, thus generating a protease with broad substrate speci®city and leading to degradation of extra-cellular matrix (ECM) proteins [1±3] The activity of uPA

is focussed to the cell surface by interaction with its speci®c receptor uPAR (CD87) Tissue-type plasminogen activator (tPA), the second type of human plasminogen activator, in contrast to uPA does not bind to a high af®nity receptor on tumor cell surfaces and therefore does not promote tumor cell-associated pericellular pro-teolysis

There are two main inhibitors of uPA and tPA, the serine protease inhibitors (serpin) plasminogen activator inhibitor type-1 (PAI-1) and type-2 (PAI-2) [4] For inhibition, the surface-exposed reactive center loop (RCL) of PAI-1 or PAI-2 interacts with the reactive site

of the target protease Initially, the P1±P1¢ bond of the inhibitor is cleaved and an intermediate enzyme±inhibitor complex is formed This is followed by the insertion of part of the RCL as additional b strand 4A, which leads to the translocation of the protease across the plane of b sheet

A of PAI-1 and formation of an SDS-stable enzyme± inhibitor complex [5] This complex dissociates very slowly and is cleared from circulation before disassembly can occur In vitro, the inhibitor can be released from the protease in a substrate-like manner, generating the so-called RCL-cleaved form of the inhibitor [6]

Correspondence to V Magdolen, Klinische Forschergruppe der

Frauenklinik der Technischen UniversitaÈt MuÈnchen, Klinikum rechts

der Isar, Ismaninger Str 22, D-81675 MuÈnchen, Germany.

Fax: + 49 89 4140 7410, Tel.: + 49 89 4140 2493,

E-mail: viktor@magdolen.de

Abbreviations: ECM, extracellular matrix; Hep, heparin; hE, helix E;

HMW-uPA, high molecular weight urokinase-type plasminogen

activator; PAI-1, plasminogen activator inhibitor type-1; PAI-2,

plasminogen activator inhibitor type-2; RCL, reactive center loop;

RU, resonance units; SPR, surface plasmon resonance; tPA,

tissue-type plasminogen activator; uPA, urokinase-tissue-type plasminogen

acti-vator; uPAR, uPA receptor; Vn, vitronectin; IPTG, isopropyl

thio-b-D -galactoside.

*Note: these authors contributed equally to the work.

Note: web pages are available at

http://www.frauenklinik-tu-muen-chen.de, http://www.biochem.mpg.de/oesterhelt/ and

http://www.wilex.com

(Received 04 July 2001, revised 22 October 2001, accepted 29 October

2001)

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Active PAI-1 is metastable and spontaneously converts to

a latent form by inserting a major part of its RCL into the

central b sheet A [7] Latent PAI-1, as well as PAI-1 in

complex with uPA or tPA, do not bind to ECM

compo-nents Active PAI-1, however, interacts with ®brin, heparin

(Hep), and vitronectin (Vn) [1,8,9] Binding to Vn doubles

the physiological half-life of active PAI-1 in solution

Moreover, by binding to Vn or Hep, the substrate speci®city

of PAI-1 is altered, because interaction with Vn or Hep

enables PAI-1 to inhibit another serine protease, thrombin

[10]

High levels of PAI-1 in tumor tissue indicate short

recurrence-free and overall survival of tumor patients

af¯icted with a broad variety of cancers, e.g mammary,

ovarian, cervical, colorectal, bladder, renal and lung

carcinomas [2,3] In line with this, it was demonstrated

that PAI-1 affects tumor cell adhesion and/or tumor

angiogenesis and, as a result of this, may support tumor

invasion [11±13]

As the PAI-1-binding site on Vn partially overlaps with

the binding sites of cellular adhesion proteins, e.g uPAR

and some integrins, addition of PAI-1 to Vn-bound tumor

cells leads to detachment of these cells [11,14] This effect can

be reversed by uPA, as Vn-bound PAI-1 dissociates from

Vn upon interaction with uPA [9] Interestingly, as

demon-strated by Bajou et al [13], the in¯uence of PAI-1 on tumor

vascularization is due to the inhibition of proteases and not

due to its interaction with Vn Thus, the balance of several

tumor-associated factors seems to control the modulatory

effects of PAI-1 on cell adhesion, migration, and

angiogen-esis and may play a crucial role in tumor invasion and

metastasis [13,15]

Various research groups have attributed the Vn-binding

site on PAI-1 to different epitopes However, essential

amino acids appear to be located in a region within amino

acids 110±147 [16±19], most of them being localized in the

a helix E (hE) The major aim of the present study was to

further analyze the structure-function relationship of PAI-1

mainly regarding its interactions with Vn, but also with

Hep Therefore, a number of PAI-1 variants preferentially

containing modi®cations in hE of PAI-1 were generated and

characterized biochemically

M A T E R I A L S A N D M E T H O D S

Generation of PAI-1 variants

The coding regions of wild-type PAI-1 (amino acids 1±379

according to the numbering proposed by Ny et al [20]) and

wild-type PAI-2 (amino acids 2±415; PIR protein sequence

database: A32853) have been cloned in frame with an

N-terminalHis6tagintotheE coliexpressionvectorpQE-30

(Qiagen, Hilden, Germany) as described previously [21]

Modi®cations in the wild-type cDNA sequence of PAI-1

were generated by reverse long-range PCR (ÔExpand High

Fidelity PCR System KitÕ; Roche, Mannheim, Germany)

applying mutated primers (Metabion, Martinsried,

Germa-ny) PCR products were phosphorylated (T4 polynucleotide

kinase; Roche, Mannheim, Germany), re-ligated (T4 DNA

ligase; Roche, Mannheim, Germany) and transformed into

the E coli strain XL1 blue (Stratagene, Heidelberg,

Ger-many) The mutated sequences were veri®ed by DNA

sequencing performed by TopLab, Martinsried, Germany

Expression and puri®cation of wild-type PAI-1, wild-type PAI-2, and PAI-1 variants

Expression of recombinant proteins was induced by adding isopropyl thio-b-D-galactoside (IPTG; ®nal concentration:

2 mM) to an XL1 blue (variant) PAI-1 or PAI-2 culture, pregrown in Luria±Bertani medium supplemented with

100 lgámL)1ampicillin (D600 0.6±0.7) Protein expression occurred at 37 °C overnight on an orbital shaker at

200 r.p.m The bacterial culture was harvested by centrif-ugation at 5000 g at 4 °C for 10 min Then, the bacterial pellet was frozen for 20 min at )80 °C and, subsequently suspended in 20 mMNa±acetate, 1MNaCl, 0.1% Tween-80 (v/v), pH 7.4 supplemented with the protease inhibitor mix ÔComplete EDTA-freeÕ (Roche, Mannheim, Germany) In the case of wild-type PAI-2, a slightly different buffer [20 mMNa2HPO4, 1MNaCl, 0.1% Tween-80 (v/v), pH 7.4 supplemented with Complete EDTA-free] was used Bacte-ria were disrupted mechanically by addition of glass beads (Sigma, Taufkirchen, Germany) to the bacterial cell sus-pension and 10 subsequent cycles of vortexing and incuba-tion on ice for 1 min each The lysate was centrifuged for

15 min (12 000 g, 4 °C), the supernatant recovered and subjected to Ni2+-nitrilotriacetic acid agarose af®nity column puri®cation

Ni2+-nitrilotriacetic acid af®nity chromatography The Ni2+-nitrilotriacetic acid af®nity column was prepared

as described by the manufacturer (Qiagen, Hilden, Germany) Initially, the af®nity column was equilibrated with 20 mM Na-acetate, 1MNaCl, 0.1% Tween-80 (v/v),

pH 7.4 Then, the cleared bacterial lysate was applied to the column and, subsequently, the column washed with equili-bration buffer followed by 20 mM Na-acetate, 1M NaCl, 0.1% Tween-80 (v/v), 20 mM imidazole, pH 5.6 until the absorption of the ef¯uent had returned to baseline (D280< 0.001) Finally, the adsorbed recombinant proteins were eluted with 20 mM Na-acetate, 1M NaCl, 0.1% Tween-80 (v/v), 200 mM imidazole, pH 5.6 The eluate containing the recombinant proteins was dialyzed in equilibration buffer and puri®ed by a second Ni2+ -nitrilo-triacetic acid af®nity chromatography as described above The supernatant of lysates of wild-type PAI-2 expressing bacterial cells (in a buffer containing 20 mMNa2HPO4, 1M NaCl, 0.1% Tween-80, pH 7.4) was also applied to a Ni2+ -nitrilotriacetic acid af®nity column, and washed with the same buffer (at pH 6.5) supplemented with 20 mM imidaz-ole For elution, the same buffer (at pH 6.0) containing

200 mMimidazole was used

Denaturation and refolding of the recombinant proteins The puri®ed recombinant proteins, with exception of wild-type PAI-2, were denatured for 4 h in 4Mguanidinium/HCl

at room temperature under light protection Refolding of the proteins was achieved by dialysis (2 h, 4 °C) in 20 mM Na-acetate, 1M NaCl, 0.01% Tween-80 (v/v), pH 5.6 followed by a second dialysis step (overnight, 4 °C) The proteins were subsequently concentrated in Centricon centrifugal ®lter devices (Millipore, Eschborn, Germany) Wild-type PAI-2 was dialyzed against NaCl/Pi(pH 7.4) in order to remove residual imidazole, concentrated in

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Centricon centrifugal ®lter devices, and then stored at

)80 °C until use

Characterization of the recombinant proteins

The protein concentration was determined according to

Bradford using the Bio-Rad Protein Assay Dye Reagent

Concentrate (Bio-Rad, Krefeld, Germany) PAI-1 antigen

was determined using the Imubind Tissue PAI-1 ELISA Kit

(American Diagnostica, Pfungstadt, Germany) The

iden-tity of the puri®ed proteins was demonstrated by Western

blotting employing a polyclonal antibody (pAb) from

chicken directed against human PAI-1 (a kind gift of N

Grebenschikov, Institute of Chemical Endocrinology,

Uni-versity of Nijmegen, the Netherlands), a monoclonal mouse

antibody (mAb) to human PAI-2 (#110 from American

Diagnostica, Pfungstadt, Germany), and the ECL Western

Blotting Detection Reagent (Amersham Pharmacia,

Frei-burg, Germany) for detection N-terminal amino-acid

sequence analysis performed by TopLab (Martinsried,

Germany) was used to con®rm the identity of the puri®ed

recombinant wild-type PAI-1 and wild-type PAI-2

Amidolytic assay for determination of the inhibitory

activity of the recombinant proteins against HMW-uPA

The assay was performed in 96-well microtiter plates

(Greiner, Frickenhausen, Germany) Puri®ed recombinant

proteins were diluted in a buffer containing 100 mMTris/

HCl, 0.05% Tween-20 (v/v), pH 7.5, and 100 lgámL)1BSA

(ICN, Aurora, Ohio, USA), incubated with 10 U high

molecular weight (HMW-)uPA (RheotrombÒ 500 000,

Curasan Pharma GmbH, Kleinostheim, Germany) for

15 min at room temperature and then 10 lL of

chromo-genic substrate Bz-b-Ala-Gly-Arg-pNA.AcOH

(Pefa-chromeÒ uPA, Pentapharm Ltd, Basel, Switzerland,

concentration 2 mM) were added (30 min, 37 °C) The

absorption was measured at 405 nm One unit PAI activity

was de®ned as the amount, which completely inhibited one

unit of HMW-uPA activity

Complex formation of the recombinant proteins

with HMW-uPA

For complex formation, 100 U ( 0.7 lg) of HMW-uPA

were incubated with the recombinant proteins at room

temperature for 10 min The complexes were visualized by

SDS/PAGE under nonreducing conditions and subsequent

silver staining or Western blotting with the chicken pAb

against PAI-1, mouse mAb #110 against PAI-2 (as

mentioned above), and polyclonal chicken anti-uPA Ig

(kindly provided by N Grebenschikov, Institute of

Chem-ical Endocrinology, Nijmegen, the Netherlands)

POX-labeled chicken anti-(mouse IgG) Ig and POX-POX-labeled goat

anti-(chicken IgY) Ig were purchased from Dianova,

Hamburg, Germany

Amidolytic assay for determination of the inhibitory

activity of the recombinant proteins against thrombin

The assay was performed in 96-well microtiter plates Fifty

units of active recombinant PAI-1 ( 35 nM) in the presence

or absence of 140 nM Vn (Promega GmbH, Mannheim,

Germany) or 1 UámL)1 Hep (LiqueminÒ N 25 000, Hoffmann-La Roche AG, Grenzach-Wyhlen, Germany) were incubated with 0.1 U of thrombin (from human plasma; Sigma, Taufkirchen, Germany) in a total volume of

130 lL of Tris/NaCl/Tween buffer [20 mM Tris/HCl,

100 mMNaCl, 0.1% Tween-80 (v/v), pH 8.0] at 37 °C for

1 h [10] Then, 10 lL of chromogenic substrate (Chromo-zymÒ TH, Roche, Mannheim, Germany, concentration:

2 mM) were added and the thrombin activity measured monitoring the change of D at 405 nm

Complex formation of the recombinant proteins PAI-1 with thrombin

Fifty units of PAI-1 (variant) in the presence or absence of

600 nMVn or 1 UámL)1Hep were incubated with 0.5 U of thrombin in a total volume of 30 lL Tris/NaCl/Tween buffer (1 h, 37 °C) and then subjected to nonreducing SDS/ PAGE followed by Western blotting employing chicken pAb against PAI-1 and a POX-labeled goat anti-(chicken IgY) Ig

Binding of recombinant PAI-1 to Vn-coated microtiter plates

Vn or collagen type IV (Sigma, Taufkirchen, Germany) were diluted to a concentration of 10 lgámL)1in a buffer containing 100 mMNa2CO3, pH 9.6 For coating, 50 lL of the Vn or collagen type IV dilutions were poured into wells

of a NuncMaxiSorp microtiter plate (Nunc GmbH & Co

KG, Wiesbaden, Germany) and incubated overnight at

4 °C After three washes with NaCl/Pi/Tween [NaCl/Pi containing 0.05% Tween-20 (v/v)], the wells were blocked

by addition of 200 lL per well of NaCl/Pisupplemented with 2% BSA (w/v) and incubation at room temperature for

2 h The wells were washed three times with NaCl/Pi/ Tween Afterwards 100 lL per well of (mutant) PAI-1 in the desired concentration were added at room temperature for

1 h Following three additional washing steps, 200 lL per well horseradish peroxidase labeled Ni2+-nitrilotriacetic acid (Qiagen, Hilden, Germany) at a dilution of 1 : 1000 in NaCl/Pi/Tween containing 0.2% BSA (w/v) were added (1 h, room temperature) After another four times of washing, binding of PAI-1 to the solid phase was visualized

by addition of 100 lL per well of a TMB substrate mix (KPL, Gaithersburg, Maryland, USA) The reaction was stopped after color development with 50 lL per well of 0.5MH2SO4 Optical density was measured at 450 nm Surface plasmon resonance analysis

of (mutant) PAI-1 binding to Vn Surface plasmon resonance (SPR) studies were conducted with a BIACORE 2000 (Biacore AB, Uppsala, Sweden) Approximately 2000 resonance units (RU) of collagen type

IV (10 lgámL)1in 10 mMNa-acetate, pH 4.0) (lane 1) and

Vn (10 lgámL)1in 10 mMNa-formiate, pH 4.0 [22]) (lanes 2±4) were immobilized to a CM5 sensor chip (research grade, Biacore AB, Uppsala, Sweden) using the amino coupling kit according to the manufacturer's recommenda-tion All experiments were performed in HBS-EP [10 mM Hepes,150 mMNaCl,3 mMEDTA,0.005%Tween-20(v/v),

pH 7.4] at a ¯ow rate of 20 lLámin)1 HMW-uPA was

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used in a concentration of 400 UámL)1 Regeneration of the

surface was achieved by injection of 10 mMHCl for 8 min

In order to check for reproducibility during the

measure-ment, at ®rst 80 lL of a 200 UámL)1dilution of wild-type

PAI-1 were injected for two subsequent experiments,

followed by two subsequent measurements of 80 lL of a

200 UámL)1dilution of a PAI-1 mutant Then, wild-type

PAI-1 was measured a third time in duplicate, followed by a

measurement in duplicate of the next mutant and so on

Thus, for each PAI-1 variant at least two binding pro®les

were recorded The kinetics obtained in the collagen type

IV-coated ¯ow cell were subtracted from the kinetics

derived from the Vn-coated ¯ow cell in order to obtain

binding pro®les without bulk effects

R E S U L T S

Expression and puri®cation of recombinant PAI-1,

PAI-2, and PAI-1 variants

The coding sequences for wild-type PAI-1 and wild-type

PAI-2, respectively, have previously been cloned by us in

expression vector pQE-30 [21] By reverse PCR, a series of

PAI-1 variants was generated using pQE-30-wild-type PAI-1

as the template Due to the fact that serpins have a very

compact tertiary structure (Fig 1A), large modi®cations of

the molecule may result in misfolded, and therefore inactive,

proteins Because of this, we designed various strategies such

as introduction of point mutations, substitution of few

amino acids by alanine and glycine, substitution of selected

epitopes by the homologue PAI-2 sequence, and short

deletions (Fig 1B) The generated PAI-1 variants are

summarized in Table 1 IPTG-induced recombinant protein

expression in the bacterial strain XL1 blue yielded 5±10% of the total E coli protein

The recombinant proteins contained a His6tag at their N-terminus, allowing puri®cation by Ni2+-nitrilotriacetic acid af®nity chromatography Although one cycle of af®nity chromatography substantially enriched the recombinant proteins from other bacterial proteins, a puri®cation grade

of > 95% was only achieved after a second chromato-graphic cycle as demonstrated by SDS/PAGE (Fig 2) Inhibitory activity of the recombinant proteins against HMW-uPA

PAI-1 is unique among serpins by its metastability that leads

to a short half-life of  2 h under physiological conditions Therefore, it was not surprising that after puri®cation (performed at room temperature) most of the recombinant PAI-1 wild-type protein and variants were present in the inhibitory inactive latent conformation However, denatur-ation and subsequent refolding by dialysis leads to the reactivation of latent PAI-1 [23] An up to 87% inhibitory activity of wild-type PAI-1 and PAI-1 variants against HMW-uPA (100 000 Uámg)1de®ned as the maximum [24]) was reached after denaturation with 4Mguanidinium/HCl followed by dialysis in a buffer of high ionic strength at

pH 5.6 Moreover, inhibitory activities of the protein preparations remained stable for more than one year in this buffer when stored at )80 °C The inhibitory activity of the generated PAI-1 mutants is summarized in Table 1 All inhibitory active mutants were metastable (half-life £ 2 h

at 37 °C) and the half-life was roughly doubled in the presence of Vn Furthermore, inhibitory active variants formed SDS-stable complexes with HMW-uPA; the inactive

Fig 1 Three-dimensional structure of active PAI-1 (A) Important structural elements of active PAI-1 (PDB 1B3K) The central b sheet A consisting

of strands 1A, 2A, 3A, 5A, and 6A is indicated in yellow, helix D in green, and helix E (hE) in cyan blue The P1-residue of PAI-1 (R346) is also indicated (B) Location of amino acid alterations in some of the generated PAI-1 mutants P73A, single amino acid exchange of P73 to alanine; A114±118, exchange of the sequence 114FRLFR118 to ®ve alanines; D109±112, deletion of the four amino acids 109MPHF112; Q123K, single amino acid exchange of Q123 to lysine.

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mutants did not (Fig 3) PAI-2 was stable (no loss of inhibitory activity after incubation for 24 h at 37 °C) and exerted an inhibitory activity against HMW-uPA of 90% (equivalent to 90 000 Uámg)1) after the two-step af®nity chromatography

Interaction of (mutant) PAI-1 with Vn All inhibitory active mutants but mutant Q123K [16] did interact with Vn as veri®ed by measuring (mutant) PAI-1 binding to Vn-coated microtiter plates and by surface plasmon resonance analysis (Table 1) This binding was demonstrated to be highly speci®c, as binding of (mutant) PAI-1 was completely abolished by preincubation of recombinant PAI-1 with soluble Vn (10 lgámL)1) prior to adding it to the wells or before injection in a reproducible manner (data not shown) Furthermore, latent PAI-1 (data not shown) as well as heat-denatured PAI-1 (Fig 4) did not bind to the immobilized Vn

The observed KD value for wild-type PAI-1 (KD ˆ 0.18 nM) as well as for P73A (KD ˆ 0.33 nM) is similar to that previously reported for the interaction of human PAI-1 recombinantly expressed in Chinese hamster ovary cells with vitronectin (KD ˆ 0.1 nM), also applying

Table 1 PAI-1 variants and binding to Vn PAI-1 mutants with corresponding speci®c inhibitory activity towards uPA and their ability to interact with Vn measured by use of Vn-coated microtiter plates (MP) and surface plasmon resonance (SPR) + Indicates binding to Vn; ± indicates no binding to Vn NT, not tested.

PAI-1 variant Modi®cation a Inhibitory activity

towards uPA (Uámg )1 )

Binding to Vn

Mutant 1 (D109-123) D F109-Q123 Inactive ± NT Mutant 2 (M2) F109-Q123 vs AAGAGAA Inactive ± NT Mutant 3 (M3) F109-Q123 vs homologue Inactive ± ±

PAI-2 sequence b and E128G Mutant 4 (M4) F109-Q123 vs AAAA Inactive ± ±

Mutant 6 (A114-115) F114A and R115A 57 000 + NT Mutant 7 (A114±118) F114-R118 vs AAAAA 20 800 + + Mutant 8 (M8) F114-R118 vs AAAAA and D68G 7 700 + + Mutant 9 (M9) V284-G294 vs homologue

a Numbering of PAI-1 according to Ny et al [20]; b 141YIRLCQKYYSSEPQA155, and c 318YELRSILRSMG328, PAI-2 according to PIR protein sequence database: A32853.

Fig 2 Puri®cation of recombinant wild-type PAI-1 Human

recombi-nant wild-type PAI-1 equipped with an N-terminal His 6 tag (wild-type

PAI-1) was puri®ed from an E coli cell lysate by Ni 2+ -nitrilotriacetic

acid anity chromatography and analyzed by SDS/PAGE M,

marker; lane 1, E coli lysate; lane 2, e‚uent of the ®rst Ni 2+

-nitrilo-triacetic acid anity chromatography cycle; lane 3, eluate of the ®rst

Ni 2+ -nitrilotriacetic acid anity chromatography cycle; lane 4, eluate

of the second Ni 2+ -nitrilotriacetic acid anity chromatography cycle.

Fig 3 Formation of SDS stable complexes Wild-type PAI-1 or variants thereof in the presence (+ uPA) or absence (± uPA) of 100 U ( 0.7 lg) HMW-uPA were incubated for 10 min at room temperature and then subjected to nonreducing SDS/PAGE Subsequently, the gels were silver-stained 200 U of PAI-1, 110 U of Q123K, 50 U of P73A and of A114±118 were applied; alternatively 1.4 lg of (inactive) M4 and 10 U of PAI-1 were used.

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SPR analysis [22] Mutant A114±118 displayed a slower

association to (on-rate: 5.35 ´ 105 M)1ás)1) and a faster

dissociation from Vn (off-rate: 1.0 ´ 10)3s)1) compared to

wild-type PAI-1 (on-rate: 1.0 ´ 106 M)1ás)1; off-rate:

1.9 ´ 10)4s)1) Only a low amount of mutant Q123K

associated to Vn and dissociated immediately after washing

with buffer, indicating an unspeci®c binding to Vn (Fig 4)

Moreover, mutant Q123K did not show any change in

binding to solid phase Vn in the presence or absence of

soluble Vn (data not shown) All Vn-binding mutants

dissociated immediately from Vn after uPA injection

(Fig 4) Thus, these results clearly demonstrate that

(mutant) PAI-1, but not Q123K, speci®cally binds to Vn

immobilized to the dextran matrix of the CM5 chip, and

then still was able to form complexes with uPA

Inhibition of thrombin by PAI-1 and binding to Hep

Binding to Vn provides wild-type PAI-1 with thrombin

inhibitory properties [10] Therefore, we tested whether the

PAI-1 mutants generated were also able to inhibit thrombin

in the presence of Vn In line with our data obtained by

SPR, the Vn-interacting mutants A114±118 and P73A, but

not Q123K, inhibited thrombin in the presence of Vn to

about 40% Vn alone did not reduce thrombin activity

signi®cantly (Fig 5)

We also tested the effects of Hep on the inhibitory activity

of PAI-1 towards thrombin All mutants tested but A114±

118 displayed similar properties as wild-type PAI-1 (> 90%

inhibition; Fig 5) A114±118 inhibited thrombin in the

presence of Hep clearly less effectively (about 40%; Fig 5)

These results determined by amidolytic assays measuring

(residual) thrombin activity were supported by the results

seen in Western blots visualizing the formation of SDS stable complexes between thrombin and PAI-1 with or without Vn or Hep Detection of a higher residual thrombin activity correlated with a lower amount of SDS stable complexes (data not shown)

The reduced capacity of A114±118 to inhibit thrombin with Hep as a cofactor is not related to an altered af®nity to

Fig 5 Inhibition of thrombin Fifty units of recombinant PAI-1 ( 35 n M ) in the presence or absence of 140 n M Vn or one UámL )1

Hep were incubated with 0.1 U of thrombin in a total volume of

130 lL of Tris/NaCl/Tween bu€er at 37 °C for 1 h Then, 10 lL of chromogenic substrate were added and the thrombin activity measured monitoring the change of optical density at 405 nm The activity of thrombin in the absence of inhibitor was set to 100%, the other activities were calculated accordingly Data shown are from three independent experiments, each measured in duplicate (‹ SD) As a control, the e€ect of 140 n M Vn or 1 UámL )1 Hep without inhibitor was measured Black bars, bu€er, only; hatched bars, plus Vn (140 n M ); grey bars, plus Hep (1 UámL )1 ).

Fig 4 Surface plasmon resonance: binding of (mutant) PAI-1 to VN Approximately 2000 RU of collagen type IV or Vn were immobilized to a CM5 sensor chip inserted in the BIAcore 2000 system Then, wild-type PAI-1 (200 UámL )1 ) was injected and allowed to bind to Vn, which was followed by a washing step with bu€er Finally, HMW-uPA (400 UámL )1 ) was injected Two subsequent measurements of the binding kinetics of wild-type PAI-1 were followed by two independent measurements of a PAI-1 variant (200 UámL )1 ) After that, wild-type PAI-1 was measured a third time, followed by a measurement in duplicate of the next mutant and so on Thus, for each PAI-1 variant at least two independent binding pro®les were obtained All experiments were performed at a ¯ow rate of 20 lLámin )1 ; regeneration of the surface was achieved by treatment with

10 m M HCl for 8 min The kinetics obtained in the collagen type IV-coated ¯ow cell were subtracted from the kinetics derived from the Vn-coated

¯ow cell in order to obtain binding pro®les without bulk e€ects Heat denatured controls were measured to compare the binding signal with the unspeci®c binding to the identical surface conditions.

Trang 7

Hep, as in SPR we observed that A114±118 displayed

similar binding to biotinylated Hep immobilized on a SA-5

sensor chip as wild-type PAI-1 (data not shown)

Interest-ingly, Hep did not only provide A114±118 with thrombin

inhibitory properties less ef®ciently than the other tested

mutants, it also reduced the inhibitory property of A114±

118 towards uPA in a dose dependent manner Hep had

only marginal effects on the inhibition of uPA by other

inhibitory active PAI-1 proteins (wild-type PAI-1, P73A,

Q123K, data not shown)

D I S C U S S I O N

Puri®cation and inhibitory activity of recombinant

PAI-1, PAI-2, and PAI-1 variants

Recombinant expression in a bacterial system is an easy and

quick method for the production of large amounts of

human PAI-1 and PAI-2 It has been shown previously,

that, although PAI-1 and extracellular PAI-2 are

glycosy-lated proteins, expression of both proteins in a

nonglycosy-lated form in prokaryotes does not affect production and

inhibitory activity of these proteins [25] We cloned the

cDNA sequence of both wild-type PAI-1 and wild-type

PAI-2 in an expression vector that provides an additional

histidine6-sequence at the N-terminus of the recombinant

proteins [21], thus allowing puri®cation by Ni2+

-nitrilotri-acetic acid af®nity chromatography The obtained speci®c

activities of wild-type PAI-1 and wild-type PAI-2,

respec-tively, strongly indicate that this modi®cation does not have

any signi®cant effect on the inhibitory activity of the

recombinant in comparison to the natural proteins This is

most likely due to the fact that the RCL of both serpins is

located close to the C-terminus

The recombinant proteins were puri®ed under native

conditions using a modi®ed version of a buffer that had

been described previously for the isolation of PAI-1 from

conditioned medium of human endothelial cells [26] This

buffer is characterized by a low pH (5.6) as well as a

relatively high ionic strength (1MNaCl) These conditions

resemble the inner milieu of the a-granula contained in

thrombocytes, which are the main reservoir of PAI-1 in

blood [27] Sancho et al [28] reported the isolation of

signi®cant amounts of active PAI-1 under native conditions

by using such a buffer However, we did not obtain such

high amounts of active PAI-1 under similar conditions, but

the inhibitory activity of PAI-1 was dramatically enhanced

by denaturation, refolding, and storage in a buffer at pH 5.6

containing 1MNaCl

The generated PAI-1 variants did also not display any

inhibitory activity after puri®cation under native conditions

Again, the inhibitory activity of at least some of the PAI-1

mutants could be restored by denaturation and refolding

The achieved activities among the variants were highly

related to the number of modi®cations introduced The

variants A114±115, P73A, and Q123K, in which only one or

two amino-acid were exchanged, showed the highest

inhibitory capacity against HMW-uPA among the PAI-1

variants ( 50 000 Uámg)1) Mutant A114±118 containing

®ve amino-acid substitutions displayed 24%, D109±112

(four deleted amino acids) and M8 (six amino-acid

substi-tutions) about 10% of the inhibitory activity of recombinant

wild-type PAI-1, only All of the other PAI-1 variants

(D109-123, M2, M3, M4, M9), which contained more than seven modi®ed amino-acid positions did not display any inhibitory activity towards uPA, indicating that larger modi®cations at these positions are not tolerated and lead to

a loss of activity most likely due to misfolding of the compact PAI-1 structure

The active PAI-1 variants behaved similarly to wild-type PAI-1 with respect to their metastability and the stabiliza-tion of the active form by Vn In contrast to wild-type PAI-1 and its active variants, recombinant PAI-2 did not show any signi®cant loss of activity after puri®cation under native conditions, underlining the uniqueness of the metastable PAI-1 among serpins

Interaction of (mutant) PAI-1 with Vn Using Vn-coated microtiter plates and SPR, we analyzed speci®c binding of wild-type PAI-1 and PAI-1 variants to

Vn In general, SPR measurements are considered to be very quantitative and association/dissociation constants are normally easily analyzed by Langmuir binding isotherms

to obtain the respective binding constants However, in the case of PAI-1 and variants thereof, this kind of measure-ments may not be accurate for the following reasons: PAI-1 spontaneously converts from its active to a latent form To determine the proportion of active PAI-1, we measured the speci®c inhibitory activity against uPA with a theoretical maximum de®ned as 100 000 Uámg)1[24] This method can

be used for wild-type PAI-1, but for the PAI-1 mutants a different maximum for the speci®c activity may exist Furthermore, within the time frame of determination of the amount of active PAI-1 in a given preparation to the time-point when PAI-1 is used in the SPR analysis, another as yet unknown part of active PAI-1 converts to the latent form and thus cannot bind to Vn anymore Therefore, the analysis of the interaction of PAI-1 and, especially, PAI-1 variants with Vn can only be semiquantitative It has to be emphasized, however, that in the present study the main aim was to analyze whether mutants with variations in hE are still able to bind to Vn and not to compare the binding af®nities of the various mutants in a quantitative manner

As only inhibitory active PAI-1 binds to Vn [8], conclusions about the Vn-binding site on PAI-1 can only

be drawn from the mutants with inhibitory activity All mutants with variations covering the whole area of hE (D109-123, M2, M3, M4) yielded inactive PAI-1 variants However, D109-112, in which the N-terminal amino acids of

hE were deleted, as well as A114±118, with changes in the C-terminal region of hE, still bound to Vn and inhibited uPA Thus, mutations within hE of PAI-1 are tolerated to some extent Our results from measuring binding of wild-type PAI-1 and its variants to Vn-coated microtiter plates were reproduced in SPR Furthermore, as these results are

in line with those obtained in thrombin inhibition experi-ments, it can be concluded that the mutants still interact functionally with Vn Moreover, concerning Q123K and its dramatically reduced af®nity to Vn, we reproduced the results of Lawrence et al [16], which again underlines the functionality of our test systems Lawrence et al [16] also reported about another mutation (L116P) in hE that led to Vn-binding de®ciency In two of our mutants (A114±118, M8), L116, among other amino acid changes, was substi-tuted by alanine The resulting mutant proteins, however,

Trang 8

still displayed Vn-binding activity This may indicate that

the rather conservative alteration of L116 to alanine (as

compared to proline) may not be dramatic enough to

eliminate the Vn-binding capacity of these PAI-1 variants

Padmanabhan and Sane [19] located the Vn-binding site

on PAI-1 to amino acids 115±130 employing PAI-1/PAI-2

chimera and protease-digested PAI-1 This seems to be

contradictory to our results, but one has to keep in mind

that proteolytic treatment of PAI-1 most likely leads to an

altered overall structure in the resulting fragments

Fur-thermore, all of the chimera that did not bind to Vn were

not only modi®ed around hE but additionally in the area

around Q123 of PAI-1 Van Meijer et al [17] localized the

Vn-binding region of PAI-1 to amino acids 110±145 using

epitope-mapped monoclonal antibodies which inhibited Vn/

PAI)1-interaction The region encompassing amino acids

110±145 not only comprises hE but also the region of the

strand 1 edge of b sheet A, where Q123 is located [29]

Cross-linking studies reported by Deng et al [18] localized the

Vn-binding region of PAI-1 to the same region However,

Sui and Wiman [30] did not report any changes in the

Vn-binding behavior of mutants with single amino-acid

substitutions in the region of F113 to D138, which is in line

with our results Summarizing these data, it is much likely

that the importance of hE for Vn-binding has been

overestimated previously Although hE still plays a role

for high af®nity binding of PAI-1 to Vn as demonstrated in

the altered association to and especially dissociation from

Vn in the case of A114±118 in SPR, there seems to exist

some cooperation of the region around strand 1 of b sheet A

with hE in Vn-binding

Inhibition of thrombin by PAI-1 and binding to Hep

Ehrlich et al [10] demonstrated that wild-type PAI-1

inhibits thrombin in the presence of Vn This is also true

for all tested variants in the present study that did interact

with Vn Furthermore, in line with the results from Ehrlich

et al [31], 1 UámL)1 was determined as the ideal Hep

concentration for inhibition of thrombin by wild-type

PAI-1 In addition to wild-type PAI-1, we also tested the mutants

A114±118, Q123K, and P73A at this concentration P73A

did not show any differences in interaction with Hep,

although this amino acid is located in helix D, which was

previously reported to contribute to the Hep binding site

[32] As expected, Hep-bound Q123K inhibited thrombin

exactly like wild-type PAI-1 A114±118 did not display a

signi®cantly altered af®nity to Hep in SPR, although it was

not able to inhibit thrombin as ef®ciently as wild-type PAI-1

together with Hep This contrasts with the results of Sui and

Wiman [30] who detected a change in af®nity to Hep in their

mutant R118D and proposed that mainly ionic interactions

occur between PAI-1 and Hep However, a change of R118

to alanine (as in the mutant A114±118) and not to aspartate

(as in R118D) may not change the surface charge of this

region signi®cantly enough to reduce af®nity to Hep

Surprisingly, the inhibitory activity of A114±118 towards

uPA was reduced in a dose dependent manner by addition

of Hep This may suggest that upon Hep-binding to A114±

118 conformational changes occur, affecting the inhibition

characteristics of this PAI-1 mutant towards both thrombin

and uPA

C O N C L U S I O N S

Comparison of the main protease targets of PAI-1, uPA and tPA, has previously shown that the proteolytic activity of these enzymes is not exclusively the relevant feature for cancer spread Rather, it seems that further interactions of one of the proteases, uPA, with other molecules support tumor invasion and metastasis Whereas tPA, at least in breast and ovarian cancer, does not play a major role in tumor cell invasion, uPA is an important, multifunctional component of the invasion machinery most likely due to effects excerted upon interaction with its speci®c receptor, uPAR [3] Similarly, the additional binding partners of

PAI-1 (Vn, Hep, and ®brin) strongly differentiate it from PAI-2, which extracellularly targets serine proteases only

Especial-ly, interaction of PAI-1 with Vn is strongly related to the modulation of cancer cell adhesion and, thus, may facilitate tumor cell invasion via a balanced interference/induction of tumor cell attachment/migration [11,12,33,34]

Detailedknowledgeofthestructuralregion(s)ofthePAI-1 molecule implicated in the PAI-1/Vn-interaction is the basis for the rational development of site-speci®c PAI-1 modu-lators [35] Surface-exposed loop structures, such as hE in PAI-1, represent attractive targets for the development of such modulators because of their high accessibility hE has been implicated in the binding of PAI-1 to Vn [16±19] An hE-blocking compound may not block further PAI-1 activities (inhibition of serine proteases or binding to ®brin) and, thus, would not alter functions of PAI-1 important for physiological processes such as ®brinolysis However, in the present paper, we demonstrate that the region around hE in PAI-1 is not a prerequisite for binding to Vn and, thus, may not be a target for the development of a therapeutically applicable PAI-1 modulator

A C K N O W L E D G E M E N T S

The excellent technical assistance of S Creutzburg is gratefully acknowledged We thank J StuÈrzebecher, J Krol, and S Sato for stimulating discussions Part of this work was supported by grants of the Graduiertenkolleg 333, the Sonderforschungsbereich 469 (A4), and the Sonderforschungsbereich 456 (B9) of the Deutsche Forschungs-gemeinschaft.

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