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However, soluble FN could bind to FXII, and this binding inhibited the surface-induced autoactivation of FXII and subse-quent binding of the generated FXIIa to immobilized FN.. As our pr

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to soluble and immobilized fibronectin – localization of the Hep-1/Fib-1 binding site for activated factor XII

Inger Schousboe1, Birthe T Nystrøm1and Gert H Hansen2

1 Department of Biomedical Sciences, The Panum Institute, University of Copenhagen, Denmark

2 Department of Cellular and Molecular Medicine, The Panum Institute, University of Copenhagen, Denmark

Several studies have suggested that in the

cardio-vascular system, the interaction between the vessel wall

and the contact activation system of blood

coagula-tion, including factor XII (FXII), high molecular mass

kininogen (HK) and prekallikrein, involves Zn2+ -dependent and receptor-mediated binding of FXII and

HK Thus, investigations of FXII and HK binding to endothelial cells in the vascular wall mimicked by

Keywords

association; factor XII; factor XIIa;

fibronectin

Correspondence

I Schousboe, Department of Biomedical

Sciences, Heart and Circulatory Research

Section, The Panum Institute, University of

Copenhagen, Blegdamsvej 3C, DK-2200

Copenhagen, Denmark

Fax: +45 35367980

Tel: +45 35327800

E-mail: schousboe@imbg.ku.dk

(Received 7 May 2008, revised 8 July 2008,

accepted 18 August 2008)

doi:10.1111/j.1742-4658.2008.06647.x

Fibronectins (FNs) are dimeric glycoproteins that adopt a globular con-formation when present in plasma and solution and an extended confor-mation in the extracellular matrix Factor XII (FXII) is a zymogen of the proteolytically active FXIIa that plays a role in thrombus stabiliza-tion by enhancing clot formastabiliza-tion and in inflammastabiliza-tion by enhancing bradykinin formation To investigate whether the extracellular matrix could play a role in these events, we have recently shown that FXIIa, but not FXII, binds to the extracellular matrix (ECM), and suggested that FN may be the target for the binding Immunofluorescence micros-copy has in the present investigation confirmed that FXIIa added to the ECM colocalizes with FN deposited during growth of human umbilical vein endothelial cells The aim of the present study, therefore, was to fur-ther elucidate the interaction between FXIIa and FN by the use of a solid face binding assay This showed, like the binding to the ECM, that FXIIa, but not FXII, binds in a Zn2+-independent manner to immo-bilized FN The KD for the binding was 8.5 ± 0.9 nm (n = 3) The binding was specific for the immobilized FN, as the binding could not be inhibited by soluble FN Furthermore, soluble FN did not bind to immobilized FXIIa However, soluble FN could bind to FXII, and this binding inhibited the surface-induced autoactivation of FXII and subse-quent binding of the generated FXIIa to immobilized FN The presence

of FXII in an anti-FN immunoprecipitate of plasma indicated that some FXII in plasma circulates bound to FN The binding of FXIIa to FN was inhibited by gelatine and fibrin but not by heparin, indicating that FXIIa binds to immobilized FN through the type I repeat modules Accordingly, FXIIa was found to bind to immobilized fragments of FN containing the type I repeat modules in the N-terminal domain to which fibrin and gelatine bind

Abbreviations

CTI, corn trypsin inhibitor; DS, dextran sulfate; ECM, extracellular matrix; Fib-1, the N-terminal fibrinogen binding domain on fibronectin; FN, fibronectin; FXII, factor XII; FXIIa, activated factor XII; Hep-1, the N-terminal heparin binding domain on fibronectin; Hep-2, the C-terminal heparin binding domain on fibronectin; HK, high molecular mass kininogen; HRP, horseradish peroxidase; HUVEC, human umbilical vein endothelial cell; OPD, o-phenylenediamine.

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cultures of human umbilical vein endothelial cells

(HUVECs) have shown that FXII and HK interact by

multiprotein assembly [1–3]

FXII is a precursor of the proteolytically active

acti-vated FXII (FXIIa) FXII and FXIIa bind equally well

to a confluent layer of HUVECs [4] However, a recent

investigation has shown that the binding might have

been artefactual, and that FXII in the presence but

not in the absence of a negatively charged surface

bound rather to the extracellular matrix (ECM)

gener-ated during growth of HUVECs The presence of

negatively charged surfaces appeared to serve two

purposes: (a) it induced and enhanced the

autoactiva-tion of FXII, generating FXIIa; and (b) it abrogated

nonspecific binding of FXIIa [5]

The binding of FXIIa to the ECM showed several

differences from the binding to HUVECs Thus, the

binding to the ECM was: (a) specific for FXIIa;

(b) Zn2+-independent; (c) not inhibited by HK; and

(d) nonelectrostatic As proteolytic degradation of the

ECM abrogated the binding of FXIIa, it was assumed

that a matrix protein was the target for the binding

Therefore, it was tentatively analyzed and found that

FXIIa binds to fibronectin (FN) [5]

FN is a dimeric high molecular mass glycoprotein

that is found both as a circulating soluble molecule in

the blood and as insoluble molecules forming

elon-gated multimeric structures in the ECM [6,7] The

monomer of the dimeric soluble molecule is a mosaic

protein composed of modular subunits generating

different domains [8], which harbor binding sites for

glycosaminoglycans, collagen or gelatine, fibrin, and

integrin receptors Some of these binding sites become

available only in the multimeric, elongated forms in

which internal sequences of amino acid residues

become exposed [9,10] Several factors mediate the

transition from the soluble to an elongated form,

including adsorption of FN to plastic surfaces [11–14]

As our previous studies have shown that the binding

of FXIIa to the ECM could be due to binding of

FXIIa to FN generated during growth of HUVECs

[5], we here report on studies of the interactions of

FXIIa with FN using a solid-phase binding assay in

which either FN or FXIIa is immobilized

Results

FXII/FXIIa binding to FN

The association of FXIIa with the ECM was assumed

to take place through binding to FN Therefore,

inves-tigations were first performed to determine whether it

could be shown that FXIIa associated with FN

depos-ited on the surface of the culture dish after depletion

of HUVECs by EDTA extraction Immunofluores-cence clearly showed that FXIIa bound to the depos-ited FN (Fig 1) No FN was deposdepos-ited on and no FXIIa bound to surfaces incubated with growth medium under the same conditions and for the same periods of time as the cells but in the absence of cells

To obtain more information about this association, the interaction between FXIIa and FN was subse-quently analyzed by measuring the binding of FXIIa

to FN immobilized on a plastic surface

Using a solid-phase binding assay, the binding

of FXIIa to FN was visualized by reactions with an

A

B

Fig 1 Colocalization of the ECM-bound FXII and FN HUVECs were grown to near confluence, and the generated ECM was exposed by detaching the cells with EDTA After washing, the ECM was incubated for 1 h with 20 n M FXIIa The ECM was then washed again and incubated first with a mixture of goat anti-FXII IgG (1 : 100) and rabbit anti-FN IgG (1 : 100) for 1 h, and second with a mixture of Alexa 594-conjugated donkey anti-(goat IgG) (1 : 800) and Alexa 488-conjugated goat anti-(mouse IgG) (1 : 800) (A) Red indicates the presence of FXIIa (B) Green indicates the presence of FN Bar: 20 lm.

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antibody against FXII and a horseradish peroxidase

(HRP)-labeled secondary antibody Neither the

anti-body against FXII nor the secondary antianti-body was

observed to bind to FN in the absence of FXIIa This

excludes the possibility that the response was

nonspe-cific and due to a direct interaction between the

immo-bilized FN and the immunoglobulins, as previously

noted [15] Furthermore, preincubation of FXIIa for

1 h with a two-fold molar excess of the antibody

against FXII prior to incubation with FN abolished

the binding Surprisingly, the binding could not be

inhibited if the immobilized FN had been preincubated

with a polyclonal antibody against soluble FN (data

not shown) This could be due to lack of recognition

of the binding site on the immobilized FN for FXIIa,

but it could also be due to a nonspecific interaction

between FXIIa and the plastic surface However, very

little FXIIa bound to wells devoid of FN (controls)

Moreover, nonspecific binding is nonsaturable The

binding of FXIIa to immobilized FN was saturable

even at low concentrations of FXIIa This was

demon-strated by analyzing the binding of varying

concentra-tions of FXIIa At low concentraconcentra-tions of FXIIa,

considerably more FXIIa bound to FN than to control

wells At high concentrations of FXIIa, the binding to

FN increased linearly with the concentration of FXIIa, and in parallel with the binding of FXIIa to control wells After subtraction of nonspecific binding from the total binding, saturated binding to immobilized

FN was observed at FXIIa concentrations ‡ 20 nm (Fig 2) Linear transformation of the the binding iso-therm (Fig 2 insert) obtained in one of three indepen-dent experiments, each performed in triplicate, showed high-affinity binding, the KD of which was estimated

to be 8.5 ± 0.9 nm, using all available data

To determine whether the binding of FXIIa to immobilized FN was mediated through the N-terminal surface binding sequence in FXIIa, investigations were performed to determine whether the presence of nega-tively charged compounds such as sulfatides would affect the binding of FXIIa to FN This showed that sulfatides neither inhibited nor enhanced the binding

to immobilized FN The apparently higher-affinity binding of FXIIa in the present experiment in the absence than in the presence of sulfatides was due to parallel higher nonspecific binding However, if FXIIa was exchanged with FXII, the presence of sulfatides induced binding of FXII, which in the absence of

0 0.5 1 1.5 2 2.5 3 3.5

20

Concentration of FXIIa, n M

FN Control

FN - Control

y = 1.1214x + 9.7607

R2 = 0.993

0 10 20 30 40 50 60 70

Concentration of FXIIa, n M

Fig 2 Concentration-dependent binding of FXIIa to FN The microtiter plate was coated overnight with FN (10 lgÆmL)1) and NaCl ⁄ P i (con-trol), respectively, and subsequently blocked with blocking buffer Then, it was incubated for 1 h with increasing concentrations of FXIIa in blocking buffer The amount of bound FXIIa was determined by sequential incubation with goat FXII IgG and HRP-conjugated rabbit anti-(goat IgG) and visualized by reactions with OPD as described in Experimental procedures d, total amount of FXIIa bound to wells coated with FN; s, total amount of FXIIa bound to control wells (devoid of FN but ‘coated’ overnight with NaCl ⁄ P i ; , binding of FXIIa to FN, calcu-lated as the difference between binding of FXIIa to the former and the latter Linear transformation of the results shown in the figure, which

is representative of three experiments performed in triplicate, gave a KDof 8.7 n M Results are means ± SD (n = 3), shown by vertical bars when extending beyond the symbols.

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sulfatides was negligible (Fig 3) The

sulfatide-depen-dent binding of FXII was most likely due to a

sulfat-ide-induced and sulfatide-enhanced autoactivation of

FXII [16,17] Accordingly, the presence of corn trypsin

inhibitor (CTI), which inhibits the activity of FXIIa,

and thus the autoactivation of FXII, almost

com-pletely blocked the sulfatide-induced binding of FXII

to FN As compared to FXIIa, a small amount of

FXII bound to FN Binding of the activated form of

FXII was shown by western blots of extracts of

immo-bilized FN incubated with FXII in the presence of

sulf-atides (Fig 4)

As FXII and FXIIa bind equally well to sulfatides

[5], the lack of binding to immobilized FN of FXII

and the lack of inhibition of FXIIa by sulfatides

indi-cate that the binding is not brought about by the

N-terminal surface-binding region in FXIIa To confirm

this, it was investigated whether the binding of FXIIa

to FN could be inhibited by the nine amino acid

pep-tide YHKCTHKGR(39–47), containing the

surface-binding sequence [18] The presence of this peptide did

not inhibit the binding of FXIIa to immobilized FN

(data not shown)

In plasma and in solution, FN adopts a compact soluble conformation in which the two subunits of the dimer are thought to be folded upon each other [7] Several studies have reported a change in the FN con-formation upon binding to plastic [11–14], exposing a cryptic binding site by transition from the soluble to the immobilized form [19,20] To determine whether these conformational changes were of significance for the binding of FXIIa, subsequent investigations were performed to determine whether the presence of solu-ble FN could inhibit the binding to immobilized FN This was shown not to be the case The amount of FXIIa that bound to immobilized FN was the same regardless of the presence of soluble FN In contrast, the presence of soluble FN reduced the sulfatide-induced binding of FXII (P < 0.001) (Fig 5) How-ever, as sulfatides had hardly any effect on the binding

of FXIIa to immobilized FN, and FXII did not bind

to immobilized FN in the absence of sulfatides (Fig 3), the inhibition could be due to an inhibition of the interaction between FXII and sulfatides To inves-tigate this further, the solid-phase binding assay was turned around and the microtiter plate was coated

0 0.5

1 1.5

2 2.5

FXII – sulfatide

FXII + sulfatide

FXII + sulfatide + CTI

FXIIa – sulfatide

FXIIa + sulfatide

FXIIa + anti- FXII antibody

Block buffer

Fig 3 The effect of sulfatide on the binding of FXIIa to immobilized FN The microtiter plate, coated overnight with FN (10 lgÆmL)1) and NaCl ⁄ P i (control), respectively, was blocked with blocking buffer and incubated for 1 h with FXII (20 n M ) and FXIIa (20 n M ) in the presence (+sulfatide) and absence ( )sulfatide) of sulfatides (20 lgÆmL )1) To ensure that possible sulfatide-dependent binding of FXII could not be explained by autoactivation of FXII, incubation of FXII in the presence of sulfatides was additionally performed in the presence of CTI (10 lgÆmL)1) FN was also incubated for 1 h with FXIIa, which had been preincubated for 1 h with a twofold molar excess of goat anti-FXII IgG At the end of the incubation, the incubation mixtures were removed, and the microtiter plate was washed extensively Then, the microtiter plate was incubated sequentially with goat anti-FXII IgG, and HRP-conjugated rabbit anti-(goat IgG) in 1% skimmed milk, and the amount of bound FXIIa was visualized by reaction with OPD The combination of primary and secondary antibodies did not bind to either FN-coated or control wells in the absence of FXIIa ⁄ FXII + sulfatides, as indicated by the column showing the binding of blocking buffer The total amount of FXIIa bound to FN and control wells is indicated by gray and white, respectively Results are means ± SD (n = 3), shown by vertical bars.

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with FXII and FXIIa instead of FN Then, the

bind-ing of soluble FN to immobilized FXII and FXIIa was

visualized by incubation with rabbit anti-(soluble FN)

IgG as the primary antibody and HRP-conjugated

swine anti-(rabbit IgG) as secondary antibody

Figure 6 shows that whereas almost no FN could bind

to immobilized FXIIa, it could bind to FXII The

presence of sulfatides increased only slightly the

bind-ing to both FXII and FXIIa Although it seemed most

unlikely, these differences in the amount of bound FN

could be due to differences in the amount of FXII and

FXIIa coated on the plate This was found not to be

the case, as the immunochemical response was

analyzed and observed to be identical using goat

anti-FXII IgG Moreover, to ensure that FXII had not been activated during the coating period, the wells were coated in the presence of CTI, which inhibits the activity of FXIIa and thus the conversion of FXII to FXIIa Furthermore, in order to prevent FXII from activation during the incubation with FN, CTI was added to the incubation mixture This did not affect the binding of FN (results not shown) Thus, these results clearly show that soluble FN interacts directly with FXII in the absence of sulfatides To determine whether this interaction also occurs in plasma, the presence of FXII was analyzed in immunoprecipitates

of FN Plasma was immunoprecipitated with antibod-ies against FN and adsorbed to protein G–Sepharose, from which FXII was extracted The plasma was not preabsorbed to protein G–Sepharose, as binding of FXII to the Sepharose could disturb the equilibrium for the binding of FXII to FN Instead, the amount of FXII bound to protein G–Sepharose in the absence of antibodies against FN was simultaneously analyzed (Fig 7) A much greater amount of FXII could be

2 1 FXIIa

80

50

Fig 4 Western blot of extracts of bound protein after incubation

of FXII on immobilized FN in the absence and presence of

sul-fatides The microtiter plate was coated overnight with FN

(10 lgÆmL)1) and subsequently blocked with blocking buffer Then,

it was incubated for 1 h with 20 n M FXII in blocking buffer in the

presence and absence of 20 lgÆmL)1sulfatide After washing, the

proteins bound to immobilized FN were extracted with SDS under

reducing conditions (SDS containing dithiothreitol) and subjected to

reduced SDS⁄ PAGE and western blotting FXII, FXIIa and standard

samples of molecular mass markers were run simultaneously

Anti-body-reacting bands were visualized by sequential incubation with

goat (human FXII) IgG (1 : 2500), HRP-conjugated rabbit

anti-(goat IgG) (1 : 2500) and SuperSignal West Femto Maximum

Sensi-tivity Substrate FXII; FXIIa; Lane 1: proteins extract from control

wells devoid of FN in which FXII had been incubated in the

absence of sulfatides Lane 2: proteins extracted from immobilized

FN after incubation with FXII in the absence of sulfatides Lane 3:

proteins extracted from control wells in which FXII had been

incu-bated in the presence of sulfatides Lane 4: proteins extracted from

immobilized FN after incubation with FXII in the presence of

sulfati-des The positions of 50 kDa and 80 kDa proteins are indicated to

the left The blot shows that only FXIIa binds to FN.

0 0.2 0.4 0.6 0.8 1 1.2 1.4 1.6 1.8 2

FXII – sulf

FXII – sulf

+ globular FN

FXII + sulf

FXII + sulf

FXIIa – sulf + globular

FN

FN Control

**

*

Fig 5 The effect of soluble FN on the binding of FXII and FXIIa to immobilized FN The microtiter plate was coated overnight with FN (10 lgÆmL)1) and NaCl⁄ P i , respectively Then, it was blocked with blocking buffer and incubated for 1 h with FXII (20 n M ) or FXIIa (20 n M ) in blocking buffer in the absence ( )sulf) and presence (+sulf)

of sulfatides (20 lgÆmL)1) and in the absence and presence of solu-ble FN (10 lgÆmL)1), as indicated The amount of FXIIa bound to FN was measured by sequential incubation with goat anti-FXII IgG and HRP-conjugated rabbit anti-(goat IgG) as described in Experimental procedures The amounts of FXIIa bound to FN and control wells are indicated by gray and white, respectively Statistically significant dif-ferences in binding of FXIIa to FN coated on the microtiter plate when incubated in the absence and presence of soluble FN are indi-cated by asterisks (*not significant and **P < 0.001) Results are means ± SD (n = 3), shown by vertical bars.

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extracted from FN immunoprecipitates of plasma than

from the plasma alone This indicates that FXII also

forms a complex with FN in plasma

Further characterization of FXIIa binding to

immobilized FN

The high-affinity interaction between FXIIa and

immobilized FN and the lack of interference by

solu-ble FN indicated that the binding site on FN for

FXIIa may be buried in the compact soluble form of

FN [7] FN has binding sites for a series of ligands

such as glycosaminoglycans, collagens or gelatine, fibrin and integrins [21–27] Figure 8 shows a sketch

of FN and the localization of the different binding sites used in our attempt to identify the binding site for FXIIa Thus, concentration-dependent inhibition

of FXIIa binding to immobilized FN was observed with gelatine and high concentrations of dextran sul-fate (DS) but not with heparin (Fig 9) As shown in Fig 8, FN has two binding sites for heparin The Hep-1-binding site is a low-affinity binding site, and the Hep-2-binding site is a high-affinity binding site [21–23] If FXIIa bound to the C-terminal high-affin-ity Hep-2-binding site, it would have been expected that its interaction with immobilized FN would be inhibited by heparin Thus, the lack of inhibition by heparin indicated that FXIIa did not bind to the C-terminal high-affinity heparin-binding domain in

FN (Hep-2) However, the inhibition by high concen-trations of DS and gelatine may indicate that FXIIa binds to the N-terminal region of FN, including the low-affinity Hep-1-binding domain DS is a heparin-like molecule and may, as such, be assumed to bind

to the heparin-binding sites on FN To investigate this further, the binding of FXIIa to commercially available proteolytic fragments of FN was analyzed Each of these fragments contains binding domains for heparin, gelatine and cells, respectively Surpris-ingly, the binding of FXIIa to these fragments showed that although heparin was unable to inhibit the binding of FXIIa to intact FN, FXIIa bound pri-marily to the 30 kDa low-affinity heparin-binding fragment (Hep-1), less to the 45 kDa gelatine-binding fragment, and not at all to the 120 kDa fragment containing the cell-binding domain (Fig 10) The amount of FXIIa that bound to the 30 kDa Hep-1-binding fragment was similar to the amount of FXIIa bound to FN The N-terminal 30 kDa Hep-1-binding domain has also been identified as a Hep-1-binding site for fibrinogen and fibrin [25,26] Further evidence for FXIIa binding to this domain was therefore provided, showing that the binding of FXIIa to immobilized FN was inhibited in a concentration-dependent manner by both fibrin generated by incu-bation of fibrinogen with thrombin and fibrinogen

As compared to the inhibition by fibrin, however, an approximately 100-fold higher fibrinogen concentra-tion was needed to yield an identical amount of inhibition (Fig 11)

Discussion

Although the presence in the blood of FXII has been known for more than 50 years, its physiological

func-0

0.5

1

1.5

2

2.5

3

FXII FXIIa

Fig 6 Binding of soluble FN to immobilized FXII and FXIIa The

microtiter plate was coated overnight as indicated with FXII (20 n M )

and FXIIa (20 n M ), respectively, diluted in NaCl ⁄ P i Then, the

micro-titer plate was blocked with blocking buffer and incubated for 1 h

with FN (10 lgÆmL)1) in blocking buffer or in blocking buffer

con-taining sulfatides (+sulf; 20 lgÆmL)1) The amounts of FN bound to

FXII and FXIIa, respectively, were determined by sequential

incuba-tion with rabbit anti-FN IgG, HRP-conjugated swine anti-(rabbit IgG)

and OPD, as described in Experimental procedures Results are

means ± SD (n = 3), shown by vertical bars.

Fig 7 Western blots of FXII present in FN immunoprecipitates of

plasma FN was isolated from plasma by immunoprecipitation with

a rabbit antibody against FN and protein G–Sepharose The

pres-ence of FXII in the immunoprecipitate (lane 2) was analyzed by

western blotting using goat anti-FXII IgG as primary antibodies and

HRP-conjugated rabbit anti-(goat IgG) as secondary antibody To

assure that the presence of FXII in the immunoprecipitate was not

due to adsorption of FXII to the protein G–Sepharose, the amount

of adsorbed FXII in the absence of the antibody against FN was

analyzed simultaneously (lane 1).

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tion is still not known For the past 15 years it has

been assumed that its function is connected with

Zn2+-dependent binding to a surface or a receptor

The present study has demonstrated that in purified

systems, activated FXII (FXIIa), but not its zymogen

(FXII), binds with high affinity to immobilized FN

The binding is independent of the presence of Zn2+, is

not affected by the presence of a negatively charged

surface represented by sulfatides, and is not inhibited

by soluble FN Accordingly, soluble FN did not

bind to immobilized FXIIa The binding of FXIIa to

immobilized FN occurs through type I modules in the

30 kDa N-terminal heparin (Hep-1)-binding and fibrin

(Fib-1)-binding domain of FN

Immunohistochemical visualization of the

interac-tion between FXIIa and FN deposited on the surface

of the culture dish during 3 days of growth of HUVECs clearly showed that FXIIa associated with

FN left behind on the plastic surface after removal of the cells The visualization showed that FN had been deposited in a sparse and patchy manner, which may reflect the conditions under which the cells had been cultivated and subsequently removed by EDTA extrac-tion Indeed, the majority of the deposited FN was attached to the cells and was thus removed during extraction of the cells Furthermore, experiments with cultures of arterial endothelial cells have shown that the amount of FN deposited on the surface of the cells varied dramatically when preconfluent, newly confluent and postconfluent cultures were analyzed Thus, whereas sparse patches of FN were generated in pre-confluent and newly pre-confluent cultures, a massive net

Fib-1/Hep-1

S

FXIIa binding

COOH

NH2

S

Type I Type II Type III

Fig 8 Schematic diagram of the modular

structure of the FN monomer The FN dimer

is formed through interchain disulfide bonds

at the C-terminus Each subunit consists of

type I, type II and type III repeating

modules Sets of repeats form domains of

regions implicated in adhesion of different

ligands The squares show the positions and

the sizes of the different fragments.

0.0 0.5 1.0 1.5 2.0

Block buffer Heparin, 20 µg·mL

–1 Heparin, 40 µg·mL

–1

Gelatine, 33 µg·

mL–1 Gelatine, 330 µg·mL

–1

DS, 2

0 µg·mL –1

DS, 40 µg·mL

–1

*

Fig 9 The effect of gelatine and heparin on binding of FXIIa to immobilized FN The microtiter plate was incubated overnight with FN (10 lgÆmL)1) and NaCl ⁄ P i , respectively, and blocked with blocking buffer Then, it was incubated for 1 h with FXIIa (20 n M ) in blocking buffer containing heparin (20 and 40 lgÆmL)1), gelatine (33 and 330 lgÆmL)1) or DS (20 and 40 lgÆmL)1) The amount of bound FXIIa was deter-mined by sequential incubation with goat anti-FXII IgG and HRP-conjugated rabbit anti-(goat IgG) and visualized by reactions with OPD as described in Experimental procedures Results are mean ± SD (n = 3), shown by vertical bars Statistically significant differences between FXIIa bound to FN incubated in the presence and in the absence of effectors are indicated by asterisks (*P < 0.001).The binding to control wells was less than 0.05 absorbance units.

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of FN covering the entire surface of the cells was

formed only in postconfluent cultures [28] It may be

claimed that the deposited FN originates from the

serum present in the cell culture medium However,

the lack of appearance of deposited FN on culture

dishes incubated with the medium using the same

con-ditions and periods of time as in the presence of cells

but in their absence showed that the deposited FN in

the present investigation was generated by a

cell-medi-ated process This process induces conformational

changes in FN, exposing cryptic sites of importance

for fibril generation and elongation [28–30]

The high-affinity binding of FXIIa to the ECM with

a KD of 12.8 nm [5] and the binding of FXIIa to the

immobilized FN with a KDof 8.5 nm make it probable

that FN, whether deposited during growth of

HUVECs or coated on a plastic surface, constitutes a

binding site for FXIIa Indeed, this binding site was

found not to be present in soluble FN, as soluble FN

was unable to inhibit the binding of FXIIa to

immobi-lized FN Together with the observed lack of

inhibi-tion by an antibody against soluble FN, this suggests

that the association between FXIIa and FN involves a

cryptic site in FN Such a binding site has been shown

to be also responsible for the interaction of FN with

fibrinogen and fibrin [27] Hence, fibrinogen and fibrin

inhibited the binding of FXIIa The binding of

fibrino-gen and fibrin has been mapped to type I modules of

FN present both N-terminally and C-terminally (Fig 8) Binding of FXIIa to the 30 kDa N-terminal fragment of FN indicates that FXIIa binds to FN through the type I modules in the cryptic N-terminal end of FN but does not exclude the possibility that FXIIa may also interact with the C-terminal Fib-2-binding site

The binding site in FXIIa is unknown, but lack of inhibition of the binding of FXIIa to FN by sulfatides and the surface-binding peptide of FXII strongly indi-cates that the binding does not involve the surface-binding region in FXIIa [18] The lack of inhibition of FXIIa binding to immobilized FN by the surface-bind-ing peptide strengthens the statement that FN is the target for the binding of FXIIa to the ECM, as this binding also could not be inhibited by the peptide [5] Thus, the affinities for FXIIa binding to ECM and to immobilized FN were the same, and neither one of the binding events could be inhibited by the surface-binding peptide of FXIIa

The binding to immobilized FN was specific for FXIIa, as FXII did not bind This indicates that the binding is of no physiological relevance for the

0.0

0.2

0.4

0.6

0.8

1.0

1.2

1.4

1.6

1.8

coating

Fig 10 The binding of FXIIa to immobilized fragments of FN The

microtiter plate was incubated overnight with the 30 kDa

heparin-and fibrin-binding fragment, the 45 kDa gelatine-binding fragment,

the 120 kDa cell-binding fragment, and FN, respectively The

frag-ments, as well as FN, were coated at a concentration of

10 lgÆmL)1in NaCl ⁄ P i The plate was then washed, blocked with

blocking buffer, and incubated for 1 h with FXIIa (20 n M ) in blocking

buffer The amount of bound FXIIa was determined by sequential

incubation with goat FXII IgG and HRP-conjugated rabbit

anti-(goat IgG) and visualized by reactions with OPD, as described in

Experimental procedures Results are means ± SD (n = 3), shown

by vertical bars.

0.0 0.5 1.0 1.5 2.0

Concentration of fibrinogen/fibrin, n M

Fig 11 Fibrin inhibition of FXIIa binding to immobilized FN The microtiter plate was incubated overnight with FN (10 lgÆmL)1) and NaCl ⁄ P i , respectively, and blocked with blocking buffer Mean-while, 1.74 l M fibrinogen dissolved in blocking buffer was incu-bated overnight with 90 mUÆmL)1 thrombin or blocking buffer at room temperature, and subsequently diluted with blocking buffer containing hirudin (100 UÆmL)1) to give the indicated final concen-trations of fibrinogen and fibrin after mixing with FXIIa (final con-centration: 20 n M ) The presence of hirudin did not affect the binding of FXIIa to FN, and the concentration of hirudin was suffi-ciently high to completely block the activity of thrombin The amounts of FXIIa bound to FN in the presence of fibrinogen ( ) and fibrin (d), and the amount of FXIIa bound to control wells (s), were determined by sequential incubation with goat anti-FXII IgG and HRP-conjugated rabbit anti-(goat IgG) and visualized by reac-tions with OPD, as described in Experimental procedures Results are mean ± SD (n = 3), shown by vertical bars when extending beyond the symbols.

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activation of FXII The binding of FXIIa to the

same domain as fibrin and fibrinogen indicates,

how-ever, that FXIIa may interfere with fibril formation

and elongation during fibrillogenesis and not with the

binding of FN to its cellular receptors Further

stud-ies are needed to determine whether and how the

binding of FXIIa to immobilized FN regulates these

processes

FXII was observed not to bind to immobilized FN,

but soluble FN bound to immobilized FXII, and

immunoprecipitates of plasma FN revealed the

pres-ence of FXII This indicates a role of FN in the

activa-tion and funcactiva-tion of FXII The general concept of the

function of FXII is connected to its binding to a

sur-face This generates FXIIa, which circumstantially can

cleave FXI and prekallikrein However, the mechanism

of this activation in vivo has still not been elucidated

Furthermore, the significance of FXIIa for the

activa-tion of FXI and prekallikrein in vivo has been

ques-tioned, as FXII deficiency is not associated with

hemophilia In addition, FXI can be activated by

thrombin [31], and prekallikrein by a

prolylcarboxy-peptidase [32] and the HSP90 protein [33] However,

recent investigations have shown that FXII in vivo

plays an important role in thrombus formation, being

activated on the surface of activated platelets [34] to

which FN binds [35] The mechanism for this

activa-tion is unknown, but although speculative, the present

investigation may be of importance in understanding

the impact of FXII in thrombus formation Thus, the

binding of FXII to soluble FN may be of relevance

for the activation of FXII on the surface of activated

platelets, but this remains to be established

Experimental procedures

Materials

FXII and thrombin were obtained as 50% glycerol

solu-tions from Haematologic Technologies Inc (Essex Junction,

band with a molecular mass of 80 kDa in reduced

Enzyme Research Laboratories (Swansea, UK) FXIIa was

dissolved in water as recommended by the company,

Siliconized test tubes were likewise used for subsequent

dilutions of FXII and FXIIa, and excess dilutions were

dis-carded Human plasma FN was from Gibco (Invitrogen,

Carlsbad, CA, USA) CTI, hirudin, the N-terminal 29 kDa

heparin-binding fragment and the 45 kDa gelatine-binding

fragment were from Sigma Chemicals (St Louis, MO,

USA) The 120 kDa cell-binding fragment was obtained

from Chemicon (AH Diagnostics, Aarhus, Denmark)

Wes-tern Reserve University, Cleveland, OH, USA) Fibrinogen from bovine serum was obtained lyophilized from citrate buffer (pH 7.4) It was purchased from Calbiochem (La Jolla, CA, USA) The concentration of fibrinogen in solution was determined at 280 nm absorbance using an

280 nm) of 15.1 Heparin [sodium salt; H3125; Grade 1 from porcine intestinal mucosa

Chemicals (Uppsala, Sweden) All other chemicals were of the purest grade commercially available

Affinity-purified goat anti-(human FXII) IgG (GAFXII-AP) was from Affinity Biologicals Inc (Hamilton, ON, Canada) Rabbit anti-FN IgG (ab 299) and monoclonal antibody to FN, (Fn-3, ab 18265), which reacts with human cellular fibronectin but not with plasma fibronectin, were from Abcam (Cambridge, UK) HRP-conjugated rabbit (goat IgG) (P-0449), HRP-conjugated swine anti-(rabbit IgG) (P-0399) and o-phenylenediamine (OPD) were

immunofluores-cence microscopy were from Invitrogen (Copenhagen, Denmark)

Solid-phase binding assay

The solid-phase binding assay was performed in 96-well maximum-binding polystyrene microtiter plates (NUNC, Roskilde, Denmark) The plates were coated with 150 lL

with Locke’s buffer (154 mm NaCl, 5.6 mm KCl, 3.6 mm

pH 7.4), and unoccupied binding sites were blocked by incubation for a minimum of 30 min at room temperature

Sigma Chemicals) dissolved in Locke’s buffer] The wells were then incubated for 60 min with FXII or FXIIa added

in a final volume of 100 lL in blocking buffer in the

antigens were measured following washing of the wells with

Tris, 0.15 mm NaCl, pH 8.0)] The wells were then incu-bated for 1 h with goat anti-(human FXII) IgG, diluted

for 1 h with HRP-conjugated secondary antibodies diluted

1 : 2500 in the skimmed milk solution Extensive washing with washing buffer was performed between each change of

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incubation conditions Finally, the plates were incubated

for 10–30 min with OPD, dissolved in water according to

the manufacturer’s recommendations The peroxidase

and the relative amount of bound FXII antigen was

determined as absorbance units at 490 nm All experiments

were performed in triplicate and repeated at least twice To

obtain estimates of affinity constants, the data were

analyzed according to the isotherm

where [FXIIa] is the molar concentration of FXIIa, A is

the absorbance of the oxidized HRP substrate, which is

assumed to be proportional to the amount of FXIIa bound,

concen-trations of FXIIa

Alternatively, the microtiter plate was coated with 20 nm

immobi-lized FXII or FXIIa was visuaimmobi-lized by sequential incubation

with rabbit FN IgG and HRP-conjugated swine

milk in washing buffer, and OPD, as described above

Immunoprecipitation

Ten microliters of rabbit anti-FN IgG was added to one of

two aliquots containing 200 lL of plasma, 2 lL of hirudin

slurry of protein G–Sepharose (Sigma-Aldrich, St Louis,

MO, USA) was added to each aliquot, and the rotation was

continued for another night Following centrifugation

(1 min, 2000 g) and 10-fold washing of the precipitate with

EGTA, 0.2 m NaCl, pH 7.4), the protein adsorbed to the

protein G–Sepharose was extracted by boiling for 10 min

SDS/PAGE and immunoblotting

For western blot analysis, bound proteins were extensively

washed with Locke’s buffer and then extracted with

dith-iothreitol Aliquots of the extracts and FXII, FXIIa and

molecular weight markers were run simultaneously

and transferred to poly(vinylidene difluoride) membranes

according to standard procedures The membrane was then

IgG) (diluted 1 : 5000) Dilutions of antibodies were

buffer Detection was carried out using the chemilumines-cence enhancer SuperSignal West Femto Maximum Sensi-tivity Substrate (Pierce Biotechnology, Rockford, IL, USA)

as recommended by the manufacturer, and the results were monitored on a Las Chemiluminator

Immunofluorescence microscopy

For immunofluorescence microscopy of FXIIa bound to the ECM, HUVECs were plated on eight chamber slides (Nalgene Nunc International Corp., Roskilde, Denmark) at

medium on the second day On day 4, the cells were detached by EDTA The ECM was incubated with 20 nm FXIIa in blocking buffer for 1 h After the washing proce-dure described above for the solid-phase binding assay, the slides were incubated with antibodies The primary anti-bodies were a mixture of goat anti-FXII IgG (diluted

1 : 100) and mouse anti-FN IgG (Fn-3) (diluted 1 : 100) The secondary antibodies were a mixture of Alexa 594-con-jugated donkey anti-(goat IgG) (diluted 1 : 800) and

Finally, the slides were mounted in antifade medium (DAKOCytomation, Ejby, Denmark) and examined in a Leica DM 4000 B microscope equipped with a Leica

DC 300 FX digital camera

Specificity analyses of the antibodies showed no reaction

of the secondary antibodies with the ECM incubated in the absence of the primary antibodies

Sulfatide preparation

Sulfatides extracted from bovine brain were from Sigma Chemicals Vesicles of sulfatides were prepared as previ-ously described [5]

Statistics

The results are shown as means ± SD, and statistically significant differences were calculated using Student’s t-test

Acknowledgements

The work was supported by grants 2005-1-192 and 2006-1-0247 from the Carlsberg Foundation

References

1 Hasan AA, Cines DB, Ngaiza JR, Jaffe EA & Schmaier

AH (1995) High-molecular-weight kininogen is exclu-sively membrane bound on endothelial cells to influence activation of vascular endothelium Blood 85, 3134– 3143

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