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We have isolated, from human normal amniotic fluid collected in the weeks 16–18, the intact nonphosphorylated IGFBP-1 and five electrophoretically distinct phospho-isoforms and have determ

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factor binding protein-1 phosphorylation sites and

propensity to proteolysis of the isoforms

Lorenzo Dolcini1, Alberto Sala1, Monica Campagnoli1, Sara Labo`1, Maurizia Valli1, Livia Visai1,2, Lorenzo Minchiotti1, Hugo L Monaco3 and Monica Galliano1

1 Department of Biochemistry ‘A Castellani’, University of Pavia, Italy

2 Center for Tissue Engineering (C I T), University of Pavia, Italy

3 Biocrystallography Laboratory, Department of Biotechnology, University of Verona, Italy

Introduction

The insulin-like growth factor binding proteins

(IG-FBP) are six homologous molecules (IGFBP-1–6) that

play critical roles in a wide variety of important

physi-ological processes Upon binding they regulate the

availability of both insulin-like growth factors I and II

(IGF-I and -II) and their affinity for these ligands is

modulated by several mechanisms: attachment to the

extracellular matrix and post-translational modifica-tions, such as proteolysis, phosphorylation and glyco-sylation Additionally, several studies have revealed that IGFBPs, as well as their proteolytic fragments, have IGF-independent biological activities in cell adhe-sion and migration and in the regulation of the cell cycle and apoptosis [1–4] Furthermore, although these

Keywords

IGFBP; insulin-like growth factor binding

protein-1; mass spectrometry;

phosphorylation; proteolysis

Correspondence

M Galliano, Department of Biochemistry ‘A.

Castellani’, University of Pavia, viale

Taramelli 3b, 27100 Pavia, Italy

Fax: +39 0382 423108

Tel: +39 0382 987724

E-mail: galliano@unipv.it

(Received 16 March 2009, revised 27 July

2009, accepted 19 August 2009)

doi:10.1111/j.1742-4658.2009.07318.x

Insulin-like growth factor binding protein-1 (IGFBP-1) is the major secreted protein of human decidual cells during gestation and, as a modula-tor of insulin-like growth facmodula-tors or by independent mechanisms, regulates embryonic implantation and growth The protein is phosphorylated and this post-translational modification is regulated in pregnancy and repre-sents an important determinant of its biological activity We have isolated, from human normal amniotic fluid collected in the weeks 16–18, the intact nonphosphorylated IGFBP-1 and five electrophoretically distinct phospho-isoforms and have determined their in vivo phosphorylation state The unmodified protein was the most abundant component and mono-, bi-, tri-and tetraphosphorylated forms were present in decreasing amounts The phosphorylation sites of IGFBP-1 were identified by liquid chromatogra-phy–tandem mass spectrometry analysis of the peptides generated with trypsin, chymotrypsin and Staphylococcus aureus V8 protease Five serines were found to be phosphorylated and, of these, four are localized in the central, weakly conserved, region, at positions 95, 98, 101 and 119, whereas one, Ser169, is in the C-terminal domain The post-translational modifica-tion predominantly involves the hydrophilic stretch of amino acids repre-senting a potential PEST sequence (proline, glutamic acid, serine, threonine) and our results show that the phosphorylation state influences the propensity of IGFBP-1 to proteolysis

Abbreviations

IGF, insulin-like growth factor; IGFBP, insulin-like growth factor binding protein; LC-ESI-MS, liquid chromatography-electrospray ionization-mass spectrometry.

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proteins are all secreted in the bloodstream, the

func-tional role of IGFBP-1 [5], IGFBP-3 [2] and IGFBP-5

[2] inside the cells has been well documented

IGFBP-1, the subject of the present study, is

predom-inantly expressed in the liver and, in adult mammals,

the synthesis of the protein is upregulated in a number

of catabolic or stressful conditions, such as fasting and

diabetes [6] Additionally, it has recently been shown

that a portion of intracellular IGFBP-1 localizes to

mitochondria, where it acts as a prosurvival factor and

protects the liver from apoptosis [5] IGFBP-1

repre-sents a minor IGF binding protein in the circulation of

nonpregnant adults, but it is regarded as the most

relevant member of the IGFBP family during gestation

[7–10] Several studies indicate that IGFBP-1 regulates

embryonic growth as a local modulator of IGF

bio-availability and stimulates trophoblast migration

through binding of its C-terminal domain, which

con-tains an Arg–Gly–Asp sequence recognized by the

integrin family of cell surface receptors [11–14]

Furthermore, the protein has also been shown to play

crucial roles in ovarian, endometrial, trophoblast and

fetal–placental physiology and pathology and its level

in maternal as well as in fetal circulation and in the liver

increases under hypoxic conditions in the uterus,

result-ing in intrauterine growth retardation [15,16]

Its mature polypeptide chain consists of 234 amino

acids and, as all other IGFBPs, contains an N-terminal

and a C-terminal domain linked by a mid-region,

which has a less ordered structure [1,17] This central

domain is more variable in the different members of

the family and contains most of the sites involved in

post-translational modifications IGFBP-1 is subject to

phosphorylation on serine residues and backbone

cleavage [1,17]

In the serum of nonpregnant adults, the protein

occurs as a highly phosphorylated single species [18],

whereas differently phosphorylated IGFBP-1 isoforms

are present in the amniotic fluid [19] Earlier reports

have indicated the presence of a variable number of

isomers [19], probably reflecting sampling at different

gestational periods We have recently shown that

nor-mal amniotic fluid, collected in the weeks 16–18 of

gestation, contains the unmodified protein and five

electrophoretically distinct phosphoisoforms [20] A

previous study has shown that IGFBP-1 expressed in

Chinese hamster ovary cells is phosphorylated at

Ser101, 119 and 169 [21,22] and a recent paper

described the modification of Ser98, which was

revealed in the highly phosphorylated protein isoforms

found in hypoxia-treated cells [23]

Post-translational modification influences the

inter-action of IGFBP-1 with IGF-I [21,22,24] and has been

associated with gestational and fetal abnormalities [24– 26] However, neither the phosphorylation state of the protein isolated from normal human amniotic fluid nor the biological properties of the homogeneous IGFBP-1 phosphoisoforms have been examined so far

It has been shown that, in vitro, several metallo-proteases recognize IGFBP-1 as a substrate [27] and a modulating effect of phosphorylation on the backbone cleavage of the protein has been recognized [8] Recently, a specific IGFBP-1 protease activity has been described in a patient with multiple myeloma and identified as azurocidin [28]

Here we describe the purification on a preparative scale of the six isoforms present in human normal amni-otic fluid collected in the weeks 16–18 The availability

of sufficient amounts of the pure protein allowed a detailed identification of the amino acid residues involved in the IGFBP-1 post-translational modifica-tions occurring in vivo An important result is that three

of the five phosphorylation sites identified in the present work are in a region enriched in proline, glutamic acid, serine and threonine, as it is known that phosphate addition represents a mechanism for activating a latent PEST sequence [29] Amniotic fluid contains a specific IGFBP-1 protease activity, yielding a stable, functional and well-structured C-terminal domain [30] and we have examined the propensity of the different isoforms of IGFBP-1 to backbone cleavage in the presence of this partially purified specific protease

Results

Purification of IGFBP-1 isoforms The amniotic fluid proteins were separated by gel filtration and the low molecular mass components, eluting after the albumin peak, were pooled and fur-ther purified by anion exchange chromatography In a previous study [30], we have shown that amniotic fluid contains a metalloprotease that cleaves the majority of IGFBP-1, yielding a stable C-terminal fragment Thus,

to prevent the proteolytic process, 10 mm EDTA was added during storage of the fluid and in all buffers used throughout the gel filtration separation The pro-teins were then subjected to anion exchange chroma-tography (Fig 1) and the fractions obtained, resolved

by SDS gel electrophoresis, were transferred to poly(vinylidene difluoride) membranes by electroblot-ting and probed with anti-IGFBP-1 IgGs (data not shown) Immunoblotting showed that IGFBP-1 eluted

in six peaks (I–VI) as a single positive band with identical molecular mass of  30 kDa and that no fragmentation of the protein had occurred during the

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purification procedure The peaks were pooled and

further purified by gel filtration chromatography Six

homogeneous polypeptide chains with the same

N-ter-minal sequence, APWQCAPCSA, corresponding to

the first 10 residues of intact mature IGFBP-1, were

obtained Nondenaturing gel electrophoresis (Fig 2A)

and immunoblotting (Fig 2B) showed that the

electro-phoretic mobilities of the IGFBP-1 isoforms increased,

but not progressively, as expected on the basis of the

anion exchange chromatography elution profile Peak IV

migrated more anodically than the preceding

chro-matographic fraction and the last eluting fraction, VI,

displayed a very similar mobility to that of fraction V

The six proteins were submitted to treatment with alka-line phosphatase and, when analysed by isoelectric focusing, all acquired the same isoelectric point of the polypeptide chain eluting under peak I (data not shown) This form thus represents the unmodified protein, whereas the other fractions contain distinct phosphoiso-forms These results showed that the nonphosphorylated protein, as well as the five distinct phosphoisoforms, were obtained in homogeneous form by anion exchange chromatography Furthermore, the addition of the chelating agent was effective in preventing proteolytic cleavage, as only the intact proteins were obtained

Mass determination of the IGFBP-1 isoforms The molecular mass of the six IGFBP-1 isoforms was probed by liquid chromatography-electrospray ioniza-tion-mass spectrometry (LC-ESI-MS) (Table 1) The mass value determined deconvoluting the multiple charged ions of the protein eluting in peak I was

25 252 Da and, following reduction, 25 270 Da These values match the theoretical molecular mass of the intact, unmodified IGFBP-1 and the difference (+18 Da) measured for the reduced sample accounts for the presence of nine disulfide bonds involving the

18 cysteine residues of the polypeptide chain Analysis

of peak II yielded a molecular mass of 25 332 Da and the increase of 80 Da over the previous component is consistent with the presence of one phosphate group Peaks III and IV displayed the same molecular mass increase of 160 Da over the unmodified protein, corre-sponding to the presence of two phosphates Peaks V (+240 Da) and VI (+320 Da) contain the tri- and tetraphosphorylated protein, respectively (Fig 3) The relative amount of each form was determined

by measuring the corresponding peak area in the chro-matographic profile and were in good agreement with the absolute amounts obtained from the absorbance at

280 nm of the pooled fractions (assuming an absor-bance value of 1.42 for a 1 mgÆmL)1 solution of IGFBP-1 as calculated on the basis of the amino acid

Fig 1 Purification of IGFBP-1 isoforms The low molecular mass

fraction obtained from human amniotic fluid was resolved by

Q-Sepharose ion exchange chromatography The elution was

carried out with a linear gradient from 0 to 100% using 6.25 m M

Bistris-propane, pH 7.5 as buffer A and 6.25 m M Bistris-propane,

pH 9.5, 0.35 M NaCl as buffer B The peaks I–VI, positive when

probed with anti-IGFBP-1 IgGs, were pooled and further purified by

gel filtration.

A

B

Fig 2 Nondenaturing gel electrophoresis and western blot analysis

of IGFBP-1 isoforms IGFBP-1 isoforms isolated by ion exchange

chromatography from peaks I–VI were (A) resolved on a 17%

non-denaturing polyacrylamide gel and stained with Coomassie Brilliant

Blue (lanes 1–6) and (B) following western blot were probed with

anti-IGFBP-1 IgGs (lanes 1–6).

Table 1 Molecular mass of the IGFBP-1 peaks isolated by anion exchange chromatography.

IGFBP-1 isoforms

Measured mass (Da)

Theoretical mass (Da)

Dmass (Da)

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composition) The most abundant nonphosphorylated

form, eluting as peak I, accounted for 69.5% of the total

protein; the monophosphorylated isoform, eluting in

peak II, was 14.9%; the doubly modified forms, eluting

as peaks III and IV, accounted for 6.5 and 5.5%,

respec-tively; the triphosphorylated protein, peak V,

repre-sented 2.7%, and peak VI, corresponding to the form

with four phosphate groups, was 0.9% These results

show that amniotic fluid IGFBP-1 is modified by up to

four phosphate groups that produce five

graphically distinct isoforms The different

chromato-graphic behaviour of the doubly modified protein is

probably due to the alternative positions of the charged

groups, resulting in different isoelectric points

Identification of IGFBP-1 phosphorylation sites

The examination of the amino acidic sequence of

human IGFBP-1 with the protein pattern database

NetPhos [31] predicted 12 serines, four threonines and one tyrosine, which could potentially serve as phosp-hoacceptor sites Thus, the assumption that the phos-phate groups can have alternative distribution within the polypeptide chain suggested that a low level of modification at each site could be expected Further-more, it is known that the ionization efficiency of phosphopeptides is generally slightly lower than that

of their unmodified counterparts and only relatively large fragments, between six and 25 amino acids, are suitable for mass spectral analysis [32] Therefore, to enhance sequence coverage and ensure that each phosphorylation site was contained in at least one peptide of suitable size and hydrophobicity, the IGFBP-1 isoforms were cleaved with three different proteolytic enzymes Reduction of disulfide bonds was carried out before each enzymatic digestion and the resulting peptide mixtures were immediately submitted

to LC-ESI-MS⁄ MS analysis The HPLC separation

1000

25+24+

23+

22+ 21+ 20+ 19+

18+

17+

16+

15+

14+ 13+

25+24+

23+

22+21+20+

19+ 18+ 17+

16+

15+

14+ 13+

25+ 24+

23+

22+

21+

20+ 19+

18+

17+

16+

15+ 14+ 13+

25+

26+

27+

24+

23+ 22+ 21+ 20+

19+

18+

17+

16+ 15+

14+ 13+

25+

26+

27+

24+

23+ 22+

21+

20+

19+ 18+

17+ 16+

1200 1400

m/z

1600 1800 2000

25251 Da

25331 Da

25410 Da

25572 Da

25493 Da

E

D

C

B

A

Fig 3 Multicharged mass spectra of the IGFBP-1 phosphoisoform isolated from human amniotic fluid The mass values reported on the y-axis were obtained by deconvoluting the multiply charged pattern obtained for (A) nonphosphorylated protein eluting as peak I; (B) the mono-1-phosphorylated form, peak II; (C) the biphosphorylated chains eluting in peaks III and IV; (D) the triphosphorylated protein, peak V and (E) the tetraphosphorylated molecule eluting as peak VI.

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was performed under acidic conditions in order to

maximize positive charge ionization and the elution

gradient was chosen in order to reduce the loss of

small and hydrophilic peptides during the desalting

step Phosphoric acid was added to the sample

solu-tion because it significantly enhances the detecsolu-tion of

mono- and multiphosphorylated peptides following

RP-HPLC [33] The identity of the fragments was

assessed by manual inspection and by comparing the

experimental fragmentation pattern with the theoretical

values obtained using the protein prospector

(http://prospector.ucsf.edu/prospector/mshome.htm)

and peaks studio software (Bioinformatic Solution

Inc., Waterloo, Canada) In addition, as

phosphoryla-tion can reduce the efficiency of cleavage [34], the data

were also examined for incompletely digested

frag-ments The phosphorylated peptides were identified by

the presence of species with a theoretical peptide mass

increased by 80 Da (plus one phosphate group) or

multiples thereof, and in all cases, ions corresponding

to the loss of H3PO4 ()98 for single charged ions and

)49 for doubly charged ions) dominated the

fragmen-tation pattern A complete sequence coverage of the

protein was obtained (data not shown) and Table 2

lists the phosphorylated peptides observed The V8

fragments spanning residues 93–100 (AGSPESPE)

from the unmodified protein, at m⁄ z 773.3, and from

the monophosphorylated one, at m⁄ z 853.2, displayed

a mass difference of 80 Da, which is consistent with

the presence of one phosphate group Fragmentation

in the ion trap showed that this peptide was phosphor-ylated at Ser95 and, to a much lower extent, also at Ser98 The presence of the fragment ion at m⁄ z 755.3

is due to the loss of phosphoric acid (98 Da), the ion

at m⁄ z 296.1 (b3+P) is consistent with the modifica-tion of Ser95 and the ions at m⁄ z 638.2, (y5+P) and

at m⁄ z 442.2 (b5) are indicative of the modification at Ser98 (Fig 4B) Figure 4A shows the MS⁄ MS spec-trum obtained for the unmodified peptide The two peptides coelute and the doubly modified fragment was not observed The MS⁄ MS spectra of the V8 fragment 93–103 (AGSPESPESTE) from the monophosphory-lated isoform, at m⁄ z 1172.1 (+80 Da shift) is shown

in Fig 5A The loss of phosphoric acid (98 Da) from the precursor yielded a charged species at m⁄ z 1072.1 and the presence of a product ion at m⁄ z 642.0 (y5+P) is consistent with phosphorylation at Ser101 The fragment ions at m⁄ z 562.2 and at m ⁄ z 649.0 correspond to unmodified y5 and y6, respectively, and indicate that Ser95 is phosphorylated as well The modification of Ser98 could not be distinguished in this peptide Analysis of the V8 digest of the trip-hosphorylated protein showed the presence of peptide 93–103 carrying two phosphate groups MS⁄ MS spec-tra (Fig 5B) of the charged ion at m⁄ z 1250.0 dis-played fragment ions at m⁄ z 1152.1 and at m ⁄ z 1054.1, consistent with the loss of one and two phosphate groups, respectively The ion at m⁄ z 642.0 (y5+P) indicates the phosphorylation of Ser101 and the ion at m⁄ z 955.0 (y8+P) indicates that in the

Table 2 List of the phosphopeptides revealed by LC-ESI-MS ⁄ MS Modified residues are underlined.

Calculated mass (Da)

Measured mass (Da)

Dmass (Da) Chymotryptic peptides

Glu C peptides

Tryptic peptides

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biphosphorylated fragment, Ser95 and Ser101 are

modified The triphosphorylated peptide was not

observed These results show that the sequence

span-ning residues 93–103 contains three serine residues

whose adjacent amino acids form consensus sites for

phosphorylation The V8 fragment spanning residues

109–121, at m⁄ z 1515.3, was found in the unmodified

protein and the fragmentation pattern of its

phosphor-ylated counterpart, at m⁄ z 1595.2, displayed ions

accounting for the modification of Ser119 (data not

shown) The modification of Ser169 was detected in

the V8 fragment spanning residues 161–172, at m⁄ z

1330.3, which showed the expected fragmentation ions

(data not shown) The MS⁄ MS spectrum of the V8

fragment TSMDGE, found in all the IGFBP-1

sam-ples, and spanning residues 219–224 at m⁄ z 654.9

(Fig 6), showed that the molecular mass increase of

15.9 Da over the theoretical value was due to the

oxidation of methionine to sulfoxide, a common

post-translational modification to proteins occurring

in vivo The nonoxidized sequence was also present at

m⁄ z 639.1

These results lead to the conclusion that the IG-FBP-1 phosphoisomers isolated from normal amni-otic fluid contain five phosphoacceptor sites The phosphorylation of the five serine residues was detected in all the chromatographically distinct isomers A complete sequence coverage was obtained for each polypeptide chain and Table 2 lists the molecular mass of the peptides with phosphorylated residues One novel phosphorylation site on Ser101 was revealed, and the phosphoacceptor sites Ser95, Ser98, Ser119 and Ser169, previously described in cultured cells [22,23], were confirmed in our natural samples of human amniotic fluid IGFBP-1 In agree-ment with previous data [22,35], we did not find any evidence of threonine and tyrosine phosphor-ylation For this reason, and because it is well

Fig 4 LC-MS ⁄ MS ion spectra of IGFBP-1 peptide 93–100 (A) MS ⁄ MS spectrum of the singly charged 773.3 Da ion correspond-ing to the nonphosphorylated AGSPESPE fragment (B) MS ⁄ MS spectrum of the singly charged 853.2 Da ion corresponding

to the monophosphorylated AGSPESPE sequence.

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known that the phosphopeptides are not eluted

with high efficiency from RP-HPLC, a quantitative

analysis of each phosphorylation site could not be

performed

Chromatographic separation of the V8 peptides

In order to decide whether a major phosphoacceptor serine is present in the monophosphorylated protein, it

A

B

Fig 5 LC-MS ⁄ MS ion spectra of IGFBP-1

peptide 93–103 (A) MS ⁄ MS spectrum of

the singly charged 1172.1 Da ion

corre-sponding to the monophosphorylated

AGSP-ESPESTE fragment (B) MS ⁄ MS spectrum

of the singly charged 1250.0 Da ion

corresponding to the triphosphorylated

AGSPESPESTE peptide.

Fig 6 LC-MS ⁄ MS spectrum of the

IGFBP-1 peptide 2IGFBP-19–224 The asterisk marks the

oxidized methionine in the sequence and in

the internal ions at m ⁄ z 234.5 and 262.9.

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was digested with endopeptidase V8 from

Staphylococ-cus aureus and compared with the unmodified form

The peptide mixtures were resolved by RP-HPLC

chromatography and Fig 6 shows the superimposition

of the UV traces monitored at 214 nm The peaks were

manually collected and submitted to N-terminal

sequencing and LC-ESI-MS⁄ MS analysis The

chro-matographic profiles, as well as the peak area and

shape, did not differ in a significant manner (Fig 7)

The only major discrepancy was the presence of a

peak, indicated as 1, in the trace of the unmodified

protein, which was absent in the profile of the

monophosphorylated molecule, where an additional

one, indicated as peak 2, was present The N-terminal

sequence analysis showed that peak 1 contains the

sequence AGSPESPE (93–100) and that peak 2

corre-sponds to the fragment AGSPESPESTE (93–103)

Mass analysis confirmed these data and showed that

the latter was phosphorylated These results indicate

that the presence of the phosphate group prevents the

enzymatic cleavage Furthermore, as no other evident

peak shift was appreciable, it can be argued that the

monophosphorylated IGFBP-1 form is predominantly

modified in the sequence spanning residues 93–103

Proteolysis

In a previous paper [30], we described the isolation of

the C-terminal domain of IGFBP-1 and suggested that

the proteolytic cleavage could be ascribed to a specific

amniotic fluid metalloprotease Therefore, prior to

examining the effect of phosphorylation on the

proteo-lytic process, we prefractionated the amniotic fluid

proteins by gel filtration and analysed the fractions

obtained for their IGFBP-1 protease activity As shown

in Fig 8A, unmodified IGFBP-1 incubated with the fraction containing components with molecular mass above 50 kDa, remained almost unchanged, whereas in

Fig 7 HPLC elution profiles of the V8 peptides Trace A was

obtained from the nonphosphorylated IGFBP-1 and trace B from

the monophosphorylated protein The arrows indicate the major

differences between the two chromatograms (peaks 1 and 2).

A

B

C

Fig 8 IGFBP-1 proteolysis (A) Aliquots of the nonphosphorylated protein (1 lg in 20 lL of 20 m M Tris pH 7.5 containing 20 m M

CaCl 2 ) were incubated overnight at 37 C with 5 lL of the amniotic fluid fractions containing proteins eluting ahead (lane 1) and after (lane 2) the albumin peak in gel filtration chromatography; as a con-trol, the isolated C-terminal fragment and the untreated protein are shown in lanes 3 and 4, respectively Following SDS gel electropho-resis, the proteins were electroblotted and probed with anti-IGFBP-1 IgGs (B) Aliquots of the nonphosphorylated IGFBP-1 (1 lg in 20 m M Tris pH 7.5 containing 20 m M CaCl 2 ) were incubated overnight with no addition (lane 1), and with the addition of increas-ing amounts of the amniotic fluid fraction displayincreas-ing proteolytic activity: 2 lL (lane 2); 4 lL (lane 3); 8 lL (lane 4); and 16 lL (lane 5) Sample volumes were adjusted to 30 lL and incubations were terminated after 48 h by the addition of 30 lL of sample buffer The samples were then submitted to SDS gel electrophoresis The proteins were electroblotted and probed with anti-IGFBP-1 IgGs (C) Substrate in gel electrophoresis of the amniotic fluid protease-containing fraction Gelatin (lane 1) and IGFBP-1 (lane 2) were added to a 10% polyacrylamide solution before gel casting at a con-centration of 1 mgÆmL)1 The asterisk indicates the lysis area observed in IGFBP-1 zymography.

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the presence of proteins eluting after the albumin peak,

IGFBP-1 was partially cleaved, yielding two major

bands, one corresponding to the C-terminal domain

and a second with an apparent molecular mass of

20 kDa No fragmentation was observed when the

incu-bation was performed in the presence of 10 mm EDTA

(data not shown) The fraction containing the IGFBP-1

protease activity was then used to assess the dose

dependence of the proteolytic process Figure 8B shows

that by increasing the amount added to the protein the

intensity of the 30 kDa band is progressively reduced

The protease-containing fraction was then submitted to

zymography using gelatine and IGFBP-1 as substrates

in order to examine the specificity of the IGFBP-1

degrading activity The gelatine substrate zymogram

showed the presence of several areas of lysis,

represent-ing gelatine-degradrepresent-ing proteinase activity in the sample

On the contrary, only one area was evidenced in the

IGFBP-1 substrate zymogram (Fig 8C) These results

indicate that a specific protease is involved in the

prote-olytic cleavage occurring in amniotic fluid We then examined the effect of phosphorylation on this process Each isoform was incubated with the proteinase-containing fraction and the samples were then submit-ted to western blot analysis, together with identical aliquots of the untreated proteins (Fig 9A) The degradation process was evaluated on the basis of the ratio between the integrated areas of the intact protein

in the treated and untreated samples This value was determined for each isoform and the results (Fig 9B) show that the monophosphorylated IGFBP-1 was cleaved almost to the same extent as the unmodified protein and that the susceptibility to proteolytic degradation of the isoforms increased with the number

of phosphates linked to the polypeptide chains The different position of the groups bound to the biphosph-orylated protein also played a role in the cleavage process

Discussion

The purification procedure used in this study permitted the isolation of the unmodified as well as of the five phosphoisoforms of IGFBP-1 previously detected by proteomic analysis in human amniotic fluid collected

in weeks 16–18 of gestation [20] To obtain a sufficient amount of the biological fluid, several hundred individ-ual samples were pooled and, therefore, the prepara-tion was assumed to be normal as every individual difference would be below the detectability score The molecular mass of the nonphosphorylated protein was determined under both reducing and nonreducing con-ditions The values obtained matched the molecular mass deduced from the amino acid sequence and were consistent with the presence of nine disulfide bonds In addition, the molecular mass increments of the other isoforms defined the exact number of phosphate groups linked to the chromatographically distinct IGFBP-1 phosphoisomers isolated The characteriza-tion of the phosphorylacharacteriza-tion sites of IGFBP-I isoforms revealed that the post-translational modification involves five serine residues (numbered as Ser95, Ser98, Ser101, Ser119 and Ser169 in the mature polypeptide chain) In a previous study, we examined the phos-phorylation of IGFBP-1 isolated from amniotic fluid following the selective extraction of phosphopeptides

on a titanium dioxide (TiO2) cartridge and only three modified serines were located [36] Enrichment meth-ods are well suited when only minute amounts of the phosphoprotein are available, but a detailed analysis

of phosphoptides demands relatively high quantities of the homogeneous molecules All the residues identified

in the present study were recognized by the protein

A

B

Fig 9 Proteolysis of IGFBP-1 isoforms (A) Aliquots of each

IGFBP-1 isoform (1 lg in 20 lL of 20 m M Tris pH 7.5 containing

20 m M CaCl 2 ) were submitted to SDS electrophoresis and

immuno-blotting following incubation at 37 C overnight with the addition of

a 4 lL aliquot of the amniotic fluid fraction containing IGFBP-1

pro-tease activity As a control, identical aliquots of the six isoforms

diluted to the same concentration with the buffer containing 20 m M

CaCl2were incubated separately at 37 C overnight and analysed.

For each isoform, indicated with the peak number, the left lane

contains the untreated protein and the right lane the sample treated

with the protease-containing fraction (B) Histogram representation

of the susceptibility to proteolytic degradation of IGFBP-1 isoforms.

The y-axis shows the percentage of the ratio between the spot

area of the degraded and that of the untreated protein measured

using IMAGE J 1.37V software.

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pattern database NetPhos [31] as phosphoacceptor

sites, with a score always higher than 0.99 and, in

agreement with previous data [22,35], no modification

occurring at tyrosine and threonine residues was

found Although a previous study, using the protein

expressed in Chinese hamster ovary cells, identified

three serines (Ser101, Ser119 and Ser169) as the only

modified amino acids and excluded the involvement of

other residues [22], our results, showing the presence

of the tetraphosphorylated isoform, revealed a more

extensive post-translational modification of the

amni-otic fluid protein Because the process can be the

con-sequence of a combination of biological variables,

including different activity levels of both the

intracellu-lar protein kinases and the extracelluintracellu-lar phosphatases,

this discrepancy is probably due to the different source

of the examined molecules Moreover, it has been

shown that the phosphorylation of IGFBP-1 is

con-trolled by placental steroid hormones [37] and by

IGF-II [38] Both factors are probably regulated differently

in Chinese hamster ovary cells and in human decidual

cells A recent paper revealed the modification of

Ser98 in the hyperphosphorylated IGFBP-1 expressed

in hypoxia exposed cells and the finding was suggested

to be associated with the condition [23] However, our

data show that this phosphoacceptor site is also

modi-fied in protein purimodi-fied from normal amniotic fluid

The kinases responsible for in vivo phosphorylation of

IGFBP-1 are as yet unknown, but it has been shown

that IGFBP-1 is a substrate for several protein kinases

[31,35] and the residues adjacent to all the modified

se-rines form consensus sequences for post-translational

modification by casein kinase II

A quantitative determination of the modification

occurring at each site could not be achieved because of

the poor ionization efficiency and fast degradation of

phosphopeptides In particular, the detection of

multi-phosphorylated peptides is reduced owing to their high

propensity to adsorption to exposed surfaces [34]

Fur-thermore, nonspecific and partial enzymatic cleavages

caused the presence of several fragments containing the

same phosphorylation site However, the examination

of the peptides obtained following V8 proteolytic

cleav-age indicated that the monophosphorylated IGFBP-1

form is predominantly modified within the

AGSPES-PESTE sequence containing three of the five

phosphor-ylated residues identified in the present study It is

interesting to note that this polypeptide sequence is

rich in proline, glutamic acid, serine and threonine, a

so-called PEST region, which is typical of rapidly

metabolized proteins Briefly, the PEST hypothesis

suggests that protein regions containing a high local

concentration of the amino acids proline, glutamic acid,

serine, threonine, and, to a lesser extent, aspartic acid, are suitable targets for proteolytic degradation [29] Examination of the amino acid sequence of IGFBP-1 with the protein pattern database PESTfind (available

at http://www.at.embnet.org/toolbox/pestfind/), a com-putational tool designed to predict potential PEST sequences, confirmed that the region spanning residues 89–114 (HAAEAGSPESPESTEITEEELLDNFH) is potentially one of those This region is located 27 residues far from the proteolytic site that leads to the formation of the C-terminal fragment previously isolated and characterized from amniotic fluid [30] Proteolysis of IGFBPs is an important mechanism that controls IGF bioavailability [39,40] and previous studies have focused on the cleavage of IGFBP-1 by matrix metalloproteases present in amniotic fluid and conditioned medium from decidualized endometrial cells [8,25] The effect of IGFBP-1 phosphorylation

on its susceptibility to enzymatic cleavage has been examined in vitro and Gibson et al [8] showed that the highly phosphorylated protein is resistant to the prote-ase activity in decidual conditioned medium and to plasmin, whereas Kabir-Salmani et al [37] suggested that matrix metalloproteinase-9 selectively degrades phosphorylated IGFBP-1 Recently, it has been reported that the IGFBP-1 specific protease activity identified as azurocidin cleaves both phosphorylated and nonphosphorylated IGFBP-1 [28] These discrep-ancies probably reflect the specificity of any enzymatic activity and, although the protein has been recognized

as a potential physiological substrate for several matrix metalloproteases, little is known about the susceptibil-ity of IGFBP-1 to enzymatic cleavage Because the only proteolytic degradation of IGFBP-1 occurring without the addition of endogenous reactants so far described under normal conditions is that observed in the amniotic fluid [41], we performed the assays using

a partially purified fraction of amniotic fluid displaying IGFBP-1 specific protease activity Our data clearly show that the post-translational modification increases the susceptibility to cleavage by the amniotic fluid pro-tease and suggest that not only the degree, but also the position of the phosphate groups influence the process

As for the other IGFBPs, the proteolytic cleavage of IGFBP-1 occurs within the central domain and we have shown that the process generates a C-terminal domain (residues 141–234) carrying a phosphate group linked to Ser169 [30] It is possible that the charge repulsion between the phosphate groups in the mid-region and the one located in the C-terminal portion

of the protein may cause a conformational change that makes the cleavage site more exposed to the protease Thus, the post-translational modification might act as

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