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Nội dung

Although BMPs are characterized by versatility in receptor binding, referred to as promiscuity, tremendous differences underlie in vivo signaling by either BMP-2⁄ 4 or BMP-6⁄ 7: whereas

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is dependent on N-glycosylation of the ligand

Stefan Saremba1,2,*, Joachim Nickel1,*, Axel Seher1, Alexander Kotzsch1,2, Walter Sebald1

and Thomas D Mueller1,2

1 Lehrstuhl fu¨r Physiologische Chemie II, Biozentrum der Universita¨t Wu¨rzburg, Germany

2 Lehrstuhl fu¨r Molekulare Pflanzenphysiologie und Biophysik, Julius-von-Sachs Institut der Universita¨t Wu¨rzburg, Germany

Bone morphogenetic protein (BMP)-6, BMP-5, BMP-7

and BMP-8 constitute a subgroup of the transforming

growth factor (TGF)-b superfamily proteins Besides

the ability of BMP-6 to induce bone formation at

ecto-pic and orthotoecto-pic sites, BMP-6 transcripts have been

localized in numerous studies to developing organs

and tissues, such as the heart, the brain, and

hyper-trophic cartilage, throughout the developing skeletal system, and also to adult tissues, such as brain and uterus [1–5] BMP-6 and its closest relative, BMP-7, show overlapping expression patterns as well as over-lapping functions For example, in the developing heart, BMP-6 and BMP-7 are required for cushion for-mation and septation [5] In the brain, BMP-6 and

Keywords

crystal structure; ligand–receptor specificity;

protein–protein interaction; recognition;

transforming growth factor-b superfamily

Correspondence

T D Mueller, Lehrstuhl fu¨r Molekulare

Pflanzenphysiologie und Biophysik,

Julius-von-Sachs Institut der Universita¨t Wu¨rzburg,

Julius-von-Sachs Platz 2,

D-97082 Wu¨rzburg, Germany

Fax: +49 931 888 6158

Tel: +49 931 888 6146

E-mail: mueller@biozentrum.

uni-wuerzburg.de

Website: http://www.bot1.biozentrum.

uni-wuerzburg.de

*These authors contributed equally

Database

The coordinates and structure factors for

the structures of wild-type BMP-6 and

B2-BMP-6 have been deposited with the

Pro-tein Data Bank, entry codes 2R52 and 2R53

(Received 3 September 2007, revised 6

November 2007, accepted 12 November

2007)

doi:10.1111/j.1742-4658.2007.06187.x

Bone morphogenetic proteins (BMPs), together with transforming growth factor (TGF)-b and activins⁄ inhibins, constitute the TGF-b superfamily of ligands This superfamily is formed by more than 30 structurally related secreted proteins The crystal structure of human BMP-6 was determined

to a resolution of 2.1 A˚; the overall structure is similar to that of other TGF-b superfamily ligands, e.g BMP-7 The asymmetric unit contains the full dimeric BMP-6, indicating possible asymmetry between the two mono-meric subunits Indeed, the conformation of several loops differs between both monomers In particular, the prehelix loop, which plays a crucial role

in the type I receptor interactions of BMP-2, adopts two rather different conformations in BMP-6, indicating possible dynamic flexibility of the pre-helix loop in its unbound conformation Flexibility of this loop segment has been discussed as an important feature required for promiscuous bind-ing of different type I receptors to BMPs Further studies investigatbind-ing the interaction of BMP-6 with different ectodomains of type I receptors revealed that N-glycosylation at Asn73 of BMP-6 in the wrist epitope is crucial for recognition by the activin receptor type I In the absence of the carbohydrate moiety, activin receptor type I-mediated signaling of BMP-6

is totally diminished Thus, flexibility within the binding epitope of BMP-6 and an unusual recognition motif, i.e an N-glycosylation motif, possibly play an important role in type I receptor specificity of BMP-6

Abbreviations

ActR, activin receptor; ALP, alkaline phosphatase; BMP, bone morphogenetic protein; BMPR, bone morphogenetic protein receptor; GlcNAc, N-acetylglucosamine; h, human; MPD, 2-methyl-2,4-pentanediol; TGF, transforming growth factor.

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BMP-7 have important effects at early and late stages

of nervous system development (e.g specification of

nervous system patterning [6] and decision of neuronal

fate [7]) BMP-6-deficient mice are viable and fertile

without displaying overt effects in tissues known to

express BMP-6 mRNA [3], implying also a functional

redundancy among the factors of this subgroup

BMPs exert their biological effects by inducing the

formation of a heteromeric receptor complex from

type II [activin receptor (ActR)-II, ActR-IIB, or BMP

receptor (BMPR)-II] and type I chains (BMPR-IA,

BMPR-IB, and ActR-I) [8–10] The constitutively

active type II kinase then phosphorylates and thereby

activates the type I chain [11], which subsequently

propagates the signal downstream by acting on

BMPR-regulated Smads-1⁄ 5 ⁄ 8 [12] Although BMPs

are characterized by versatility in receptor binding,

referred to as promiscuity, tremendous differences

underlie in vivo signaling by either BMP-2⁄ 4 or

BMP-6⁄ 7: whereas BMP-2 ⁄ 4 exert their function by initial

binding to the high-affinity receptors BMPR-IA or

BMPR-IB, it has been shown that ActR-I is the

pre-dominant type I receptor used by BMP-7 in a variety

of cell lines [13]

The overall fold and dimer architecture seem to be

highly conserved for the ligands of the TGF-b

super-family, when the receptor unbound conformation is

considered Several structures have been determined,

i.e TGF-b1, TGF-b2, TGF-b3, BMP-2, BMP-7, and

growth and differentiation factor (GDF)-5, all showing

the canonical fold Here, we present the crystal

struc-ture of BMP-6 and compare the strucstruc-ture with that of

other BMPs Differences resulting in possible

altera-tions of the type I receptor-binding profile were

inves-tigated Comparison of the receptor binding of

Escherichia coli and CHO-cell derived BMP-6 reveals

the importance of N-glycosylation for ActR-I binding

and activation For proteins other than TGF-b ligands

(e.g hormones), it has been shown that glycosylation

can ameliorate the receptor binding [14]; however, this

is the first report in which glycosylation of a TGF-b

superfamily ligand is essential for the versatility in

receptor binding

Results and Discussion

Structure of human BMP-6

Structures of BMP-6 and B2-BMP-6 were determined

by molecular replacement using the coordinates of

human (h)BMP-7 (Protein Data Bank entry 1BMP

[15]) as a start model Analysis of the unit cell content

suggested the presence of a complete BMP-6 dimer in

the asymmetric unit Assuming the presence of the BMP-6 dimer in the asymmetric unit, the Matthews coefficient VMis 3.94 A˚3ÆDa)1, corresponding to a sol-vent content of  70% With only one monomer pres-ent in the asymmetric unit, the solvpres-ent contpres-ent would exceed 86%, making this possibility unlikely Calcula-tion of a self-rotaCalcula-tion funcCalcula-tion confirmed the presence

of a two-fold noncrystallographic symmetry, which is distinct from all other structures of members of the TGF-b superfamily that have so far been determined (e.g BMP-2 [16], BMP-7 [15], GDF-5 [17,18], TGF-b2 [19,20], and TGF-b3 [21]) As a result, the biological dimer is not formed by a crystallographic dyad run-ning through the intermolecular disulfide bond, as is observed in all the other structures of TGF-b mem-bers, but by a noncrystallographic two-fold axis, indicating possible asymmetry in the homodimeric structure (Fig 1A,B)

Indeed, our structure data imply that loop regions, e.g the prehelix loop, in BMP-6 can adopt two differ-ent conformations (Fig 1C) Superimposing the struc-ture of free BMP-2 [16] onto the two different monomer conformers of BMP-6 yields rmsd values of 2.5 and 1.4 A˚ for the Ca positions, clearly indicating that one of the two BMP-6 conformers adopts a loop conformation similar to that of BMP-2 (Fig 1D) If only the Ca atoms of the b-sheet core of the BMP-2 dimer are considered (without helix, fingertip and pre-helix loops), an rmsd of 1 A˚ (0.8 and 0.6 A˚ for the individual monomer subunits) is observed, showing that the core b-sheet and the dimer architecture are almost identical between BMP-6 and BMP-2 (Fig 1D) The region exhibiting the largest difference between the two monomers is the prehelix loop comprising resi-dues Phe66 to Met72 of BMP-6 (Fig 1C) In con-former 1, the loop strongly deviates from the canonical backbone conformation observed in all other BMP (see Fig 1E,F for BMP-2 as an example) members [22] As compared with BMP-2, distances between the

Ca atoms of individual residues of up to more than

8 A˚, e.g between His71 (BMP-6) and His54 (BMP-2), are found The smallest distance (1.8 A˚) between two

Ca atoms of BMP-6 and BMP-2 within this loop seg-ment is observed for Leu68 (BMP-6) and Leu51 (BMP-2) (Fig 1E,F)

The prehelix loop, however, was shown to contain the main binding and specificity determinants for type I receptor recognition in BMP-2 [23] and GDF-5 [17] Structure analysis of receptor–ligand complexes of BMP-2 [23,24] and BMP-7 [25] suggested that receptor binding and recognition is accompanied by an induced

fit mechanism affecting the side chain and backbone conformation in this loop region Thus, the fact that

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BMP-6 shows two possible, largely different

conforma-tions for this loop segment suggests that recognition

and binding of type I receptors might be influenced by

this unique feature (Fig 1C) Superimposing the

struc-ture of the binary complex of BMP-2 bound to its

high-affinity receptor BMPR-IA indeed shows that the

noncanonical conformation of the prehelix loop of

BMP-6 would prevent binding of BMPR-IA, due to

steric hindrance, whereas the canonical loop

conforma-tion (BMP-2-like conformaconforma-tion) could form similar

noncovalent interactions with the type I receptor

(Fig 2) Although the noncanonical loop

conforma-tion of BMP-6 seems not to be able to form a stable

ligand–receptor interface using the type I receptor

structures known so far, the two different loop

confor-mations clearly show that the prehelix loop seems to

be dynamically disordered in the unbound ligand

Together with the fact that the a-helix of the type I

receptor BMPR-IA, which carries the main binding determinants for BMP-2 interaction (Phe85 and Gln86

of BMPR-IA), also seems not to be folded in the free receptor [26], a large portion of the core interface seems to be flexible and undergoes a disorder-to-order transition upon complex formation This induced-fit mechanism might explain the high degree of promiscu-ity in the BMP ligand–receptor interaction, as it allows the ligand as well as the receptor surfaces to adapt to the binding partner

Receptor binding and activity of BMP-6 depend

on the nature of the expression system Signaling of BMP-6 and BMP-7 has been shown to be mediated mainly via the ActR-I receptor in many cell types [9,13,27], although this receptor binds both BMPs only with weak affinities [25] In contrast,

C

D

Fig 1 The prehelix loop of BMP-6 adopts two conformations (A) Ribbon representa-tion of BMP-6 viewed from the top and from the side (B) The central intermolecular disulfide bond is indicated by ball-and-stick, secondary structure elements, and struc-tural features are marked The prehelix loop adopts two vastly different conformations, with the largest distances between the Ca atoms of the same amino acid residue in both segments (C) Stereoview of a super-position of the prehelix loop of conform-ers A and B of BMP-6 Residues occupying similar positions are indicated in black; resi-dues having different orientations in the two conformers are marked A and B according

to the conformer (D) Stereoview of a super-position of BMP-6 and BMP-2 (Protein Data Bank entry 3BMP), showing the differences

in the loop conformations of the fingertip loops as well as the prehelix loop (E) Super-position of the prehelix loops of BMP-2 (red carbon atoms) and BMP-6 (cyan carbon atoms) in its canonical loop conformation, which is very similar to that of BMP-2 (F) Same as in (E) except for the BMP-6 prehe-lix loop of conformer 1 (green carbon atoms), which adopts a noncanonical confor-mation, and is therefore different from BMP-2.

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BMP-2 uses the type I receptors BMPR-IA and

BMPR-IB, both of which are bound with high

affini-ties To further elucidate the molecular basis for the

different type I receptor specificity profiles of BMP-2,

BMP-6, and BMP-7, we used in vitro interaction

analy-sis (Table 1) Ligand proteins of BMP-2 (E coli),

BMP-6 (CHO cells) and BMP-7 (NS0 cells) were

immobilized onto a biosensor chip, and interaction

with the receptor ectodomain proteins BMPR-IA,

BMPR-IB and ActR-I was measured using surface

plasmon resonance spectroscopy (BIAcore technique)

As expected, immobilized BMP-2 showed high binding

affinities for BMPR-IA and BMPR-IB (KD= 10 and

95 nm, respectively), whereas binding to ActR-I was

below the detection level (KD> 400 lm) In contrast,

BMP-7 bound to BMPR-IA with a much lower affinity

of  10 lm and to BMPR-IB with a slightly higher

affinity of about 1 lm Binding of BMP-7 to ActR-I

yielded affinities (KD 50 lm) similar to those

described by Greenwald et al [25], and CHO

cell-derived BMP-6 showed a receptor binding profile

simi-lar to that of BMP-7 But, to our surprise, BMP-6

expressed from E coli did not bind to ActR-I

(Table 2), whereas basically identical binding

parame-ters for the type I receptors BMPR-IA and BMPR-IB

and for the type II receptor ActR-II were observed

(Table 2) Therefore, it can be ruled out that the

differ-ence in binding is caused by misfolding or unfolding of

the E coli-derived BMP-6 We thus investigated

whether E coli-derived BMP-6, which does not bind

ActR-I, is inactive in cell-based assays, as would be expected if ActR-I were the main signaling receptor for BMP-6 and BMP-7 Indeed, glycosylated BMP-6 and BMP-7 induced alkaline phosphatase (ALP)

A

Fig 2 Analysis of a BMP-6–BMPR-IA

com-plex model (A) A putative model of BMP-6

bound to BMPR-IA was built by docking the

BMPR-IA molecules of the BMP-2–BMPR-IA

complex (Protein Data Bank entry 1REW) to

BMP-6 (B) The noncanonical prehelix loop

is incompatible with complex formation, due

to several steric clashes between residues

of the BMP-6 prehelix loop and the

b 5 a 1 -loop of BMPR-IA (C) In its canonical

(or BMP-2 like) form, the prehelix loop

adopts a conformation that is very similar to

that of BMP-2 in the BMP-2–BMPR-IA

complex No severe steric clashes are

found, suggesting that this loop

conforma-tion might be adopted in a BMP-6–BMPR-IA

interaction.

Table 1 Receptor binding profile of BMP-6, BMP-7, and BMP-2 (BIAcore analysis) Biosensor analysis using surface plasmon reso-nance was performed to determine binding affinities of the BMP ligand–receptor interaction Ligands were immobilized onto the sur-face of a CM5 sensor chip, and receptor ectodomain proteins were used as analyte Thus, interaction analysis yields the 1 : 1 interac-tion of BMPs and their receptor ectodomain proteins NB, no bind-ing within detection limit (upper limit: KD> 400 l M ).

Receptor proteins

Ligands [affinity (l M )] a

Type I

Type II

a KD(eq) as deduced from the dose dependency of equilibrium bind-ing b KD(kin) as deduced from the association and dissociation rates of the interaction; analysis of dose dependency of equilibrium binding yields higher (three-fold) values for KD(eq), as real equilib-rium binding cannot be achieved, due to the slow association rates (k on  3 · 10 4

Æ M )1Æs)1) of the BMP-2–BMPR-IA and BMPR-IB 1 : 1

interaction In contrast, association, and especially dissociation, for interaction of BMPs with ActR-I and ActR-II are faster (k on > 10 5 Æ M )1Æs)1, k

off > 10)1Æs)1), impeding the analysis of the dis-sociation rate and thus requiring analysis of the equilibrium binding.

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expression in ATDC-5 cells in a dose-dependent

man-ner, with EC50 values of 9 and 57 nm (Fig 3A) In

contrast, BMP-6 derived from E coli was practically

inactive at ALP induction, even at the high

concentra-tions tested (Fig 3A) It is also interesting to note that

BMPR-IA seems to be not able to rescue activity of

BMP-6 in ATDC5 cells, despite the fact that in vitro

interaction analysis shows that BMP-6 (and BMP-7)

can bind to BMPR-IA and BMPR-IB with higher

affinity than ActR-I That signaling of BMP-6 and

BMP-7 is mediated via ActR-I can be seen from the inhibition of proliferation in the human myeloma cell line INA6, which expresses ActR-I but not BMPR-IA

or BMPR-IB [28] Both BMP-6 and BMP-7 showed high activity in this cell line, whereas BMP-2, which signals via BMPR-IA and BMPR-IB, did not (Fig 3B) This is the first time that such a large differ-ence in binding and activity has been observed for members of the TGF-b superfamily For example, BMP-2 derived from either prokaryotic or eukaryotic expression systems has similar biological activities when tested in various cells, e.g ALP induction in C2C12 or ATDC5 cells (W Sebald, unpublished results) Receptor binding is only marginally influ-enced, with a slightly decreased affinity of CHO cell-derived BMP-2 for the type II receptor ActR-IIB [29]

Recognition of BMP-6 by ActR-I depends on N-glycosylation

To determine the molecular basis for these differences between BMP-6 derived from E coli or CHO cells, we investigated whether post-translational modifications might play a role in receptor binding and activity The crystal structure analysis of recombinant BMP-7 expressed in CHO cells (Protein Data Bank entry 1LXI [25]) or complexes of BMP-7 (Protein Data Bank entries 1LX5 [25] and 1M4U [30]) showed that the N-glycosylation sequence Asn-X-Ser⁄ Thr in the cystine-knot motif, which is conserved among BMP ligands of the BMP-2⁄ 4 and the BMP-5 ⁄ 6 ⁄ 7 family, does indeed

Table 2 N-glycosylation of BMP-6 is required for ActR-I binding

(BIAcore analysis) Biosensor analysis using surface plasmon

reso-nance was performed to determine binding affinities of the BMP-6

ligand–receptor interaction BMP-6 proteins were immobilized onto

the surface of a CM5 sensor chip via amino-coupling, and receptor

ectodomain proteins were used as analyte Thus, interaction

analy-sis yields the 1 : 1 interaction of BMPs and their receptor

ectodo-main proteins NB, no binding within detection limit (upper limit:

KD> 400 l M ).

Receptor

proteins

Ligands [affinity (l M ) a ]

BMP-6

(CHO)

BMP-6 (CHO) PNGase F

BMP-6 (CHO) PNGase F3 ⁄ H

BMP-6 (E coli) Type I

Type II

a KD(eq) as deduced from the dose dependency of equilibrium

bind-ing.

0 2000 4000 6000 8000

10 000

12 000

14 000

BMP-2 (n.d.) BMP-6 (12.4 n M +/–0.8) BMP-7 (24.0 n M +/–2.2)

BMP-6 E.coli (n.d.)

control

0.0

0.5

1.0

1.5

2.0

2.5

BMP- 2 ( 5.3 n M +/–0.3)

BMP- 6 ( 9.3 n M +/–0.5)

BMP- 7 ( 56.7 n M +/–3.4)

BMP- 6 E.coli

A B

Fig 3 Biological activities of BMP-6, BMP-7, and BMP-2 (A) Induction of ALP expression in ATDC5 cells is stimulated by BMP-6, BMP-7, and BMP-2 BMP-6 derived from E coli expression is inactive in these cells, probably due to its lack of binding to ActR-I (B) Signaling of BMP-6 and BMP-7 via ActR-I is shown in the inhibition of proliferation in the myeloma cell line INA6, which lacks the type I receptors BMPR-IA and BMPR-IB Whereas BMP-6 and BMP-7 show high activity in this cell line, BMP-2 is almost completely inactive, due to its requirement for BMPR-IA or BMPR-IB E coli-derived BMP-6 is also inactive, due to its inability to bind ActR-I; the green dashed line indi-cates maximal proliferation in the absence of any BMP ligand.

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carry carbohydrate moieties The two putative

N-gly-cosylation sites in the N-terminus of the mature part

of BMP-7, which are also present in BMP-6, have been

shown not to be glycosylated [31,32] The binding site

of the type I receptors is located in the so-called wrist

epitope of BMPs, which comprises a part of both

fin-gers and the prehelix loop, suggesting that the

carbo-hydrate moieties linked to Asn73 of BMP-6 (Asn80 on

BMP-7) could contact the type I receptors and thus

modulate receptor binding To confirm this hypothesis,

we performed deglycosylation of CHO cell-expressed

BMP-6 and determined its receptor-binding properties

by BIAcore interaction analysis First, we removed all

N-linked carbohydrate by N-endoglycosidase F

treat-ment under nondenaturing conditions to ensure that

the folding of BMP-6 was not altered by the enzymatic

reaction Endoglycosidase F hydrolyzes the

N-glyco-sidic bond between the asparagine and the first

N-acet-ylglucosamine (GlcNAc) residue, resulting in a

nonglycosylated protein (Fig 4A) The completeness

of the deglycosylation was checked by SDS⁄ PAGE

(Fig 4B) and MS analysis; the BMP-6 was then

immo-bilized onto a biosensor, and the properties of binding

to BMP type I and type II receptors were determined

by BIAcore analysis Whereas binding to the type I

receptors BMPR-IA and BMPR-IB, as well as to the

type II receptor ActR-II, was essentially identical to

binding of fully glycosylated BMP-6, no binding to

ActR-I could be determined (Table 2) This clearly shows that binding of BMP-6 to ActR-I requires car-bohydrate moieties attached to Asn73 as binding deter-minants, whereas the other type I receptors do not As the parameters for binding to BMPR-IA, BMPR-IB and ActR-II are not influenced by the removal of the N-glycosylation, large, and even small, local structural changes can be excluded We examined how many car-bohydrate residues might be involved in the binding of ActR-I by using a mixture of N-endoglycosidase H and N-endoglycosidase F3 The latter cleaves the b1–4 glycosidic bond between the first and the second Glc-NAc residue, leaving the first carbohydrate (GlcGlc-NAc) attached to the protein (Fig 4A,B) Measurement of the binding affinities of this partially glycosylated BMP-6 for BMPR-IA and BMPR-IB confirms that binding to these two type I receptors is not altered by different N-glycosylation levels However, binding affin-ity for ActR-I is now very close (less than a factor of 2)

to that of CHO cell-derived BMP-6 with full N-glycosyl-ation (Table 2), showing that the first carbohydrate moiety at Asn73 is a main binding determinant for ActR-I interaction, whereas further carbohydrate resi-dues in the carbohydrate chain are not required The aspartyl side chain generated from Asn by deglycosyla-tion using endoglycosidase F cannot be responsible for this lack of activity, as the unglycosylated E coli BMP-6 containing an Asn at position 73 is also inactive

R 1

R 2

GlcN

β1-4 α1-4 GlcN GlcN

GlcN

GlcN

M M

M

β1-4

β1-2

β1-2

α1-3 α1-6

R 1

R 2

Asp

GlcN

β1-4

GlcN

R

PNGase F

PNGase F3/H

45 35

25

18.4 14.4

45 35

25

18.4 14.4

PNGases

Asp

Fig 4 Deglycosylation of BMP-6 expressed from CHO cells (A) Scheme to illustrate the restriction sites for the endoglycosidases used PNGase F hydrolyzes the N-glycosidic bond immediately after the asparagine residue, leaving a fully deglycosylated protein A mixture of PNGase F3 and PNGase H is used to trim complex carbohydrate structures to a single GlcNAc moiety attached to the asparagine residue (B) SDS ⁄ PAGE analysis under reducing conditions of the deglycosylation reactions of BMP-6, showing the completeness of the enzymatic reactions M: molecular weight marker Lane 1: BMP-6 derived from CHO cells Lane 2: BMP-6 after PNGase F treatment Lane 3: BMP-6 before PNGase F3 ⁄ H treatment Lane 4: BMP-6 after PNGase F3 ⁄ H treatment BMP-6 runs as two bands, i.e dimer and monomer.

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Modeling the interaction of BMP-6 and ActR-I

To gain insights into how ActR-I might interact with

the N-glycosylation site at Asn73 of BMP-6, we

con-structed a model of the binary complex of BMP-6

bound to ActR-I on the basis of our BMP-6 structure

and the structures of the BMP-7–ActR-II (Protein

Data Bank entry 1LX5 [25]) and BMP-2–BMPR-IA

(Protein Data Bank entry 1REW [23]) complexes A

putative carbohydrate chain was added to BMP-6 by

using the complex glycosylation structure present on

BMP-7 as identified in BMP-7–ActR-II and which also

presents a typical N-glycosylation from expression

in mammalian cells The model of the extracellular

domain of ActR-I is based on the structure of

BMPR-IA in its bound conformation to BMP-2 (Protein Data

Bank entry 1REW); insertions and deletions were built

manually using quanta2006 software The putative

complex model of BMP-6(glycosylated)–ActR-I was

then formed by superimposing BMP-6(glycosylated)

and ActR-I with the ligand and receptor structures in

BMP-2–BMPR-IA (Protein Data Bank entry 1REW)

The model of BMP-6–ActR-I shows that several

resi-dues in the N-terminus, the b1b2-loop and the short

loop before the a-helix of ActR-I are in close

proxim-ity to the carbohydrate chain of the BMP-6(glycosylat-ed) model (Fig 5A,B) Residues of ActR-I in these regions, namely Lys11 and Tyr54, can possibly form several hydrogen bonds with the first carbohydrate moiety (Fig 5C,D), showing how the first carbohy-drate plays an important role in recognition and the generation of binding affinity for the BMP-6–ACTR-I interaction

The model also gives some hints as to why the bind-ing affinity of BMP-6 for the type I receptors

BMPR-IA and BMPR-IB is not dependent on the presence of the carbohydrate structure The b1b2-loop of ActR-I is shortened by three residues in comparison to

BMPR-IA and BMPR-IB, possibly resulting in a less flexible loop in ActR-I Interactions between residues within this loop and the carbohydrate chain might thus con-tribute significantly to the binding free energy, whereas

in the more flexible b1b2-loop of BMPR-IA⁄ IB it does not

In summary, our analysis shows the first structure of

a BMP ligand member, which exhibits two vastly dif-ferent conformations for the prehelix loop, which has been shown to be important for BMP type I receptor interaction Although, due to the lack of other BMP ligand–receptor complex structures with type I receptors

Fig 5 Model of the binary complex of N-glycosylated BMP-6 bound to ActR-I Ribbon representation of the binary complex of BMP-6 bound

to its type I receptor ActR-I The carbohydrate chain [GlcNAcb1–4GlcNAcb1–4Man(a1,3Man)(a1,5Man)(b1,4Man)] – shown as thick lines – was added from a crystal structure analysis of BMP-7 (expressed in CHO cells) bound to the type II receptor ActR-II Several residues in the N-terminus, b1b 2 -loop or the loop in front of the a-helix of ActR-I are in close contact with the carbohydrate, namely the first two to three carbohydrate moieties, as would be predicted from our deglycosylation studies (A) Viewed from the top (B) Viewed from the side (C) BMP-6 (cyan ⁄ green) and ActR-I (magenta) are shown as surface representations to visualize the close packing of the carbohydrate in between the ligand–receptor interface; putative contact residues are indicated (D) Putative hydrogen bond interactions between ActR-I and the first GlcNAc residue of BMP-6 glycosylated at Asn73 Hydrogen bonds between the first carbohydrate residue (GlcNAc) and residues Lys11 and Tyr54 of ActR-I are shown.

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different from BMPR-IA, we cannot say whether the

second noncanonical loop conformation plays a direct

role in complex formation, however the second loop

conformation confirms that this loop is highly mobile

in BMP-6 and possibly also in other BMPs An NMR

relaxation study on TGF-b3has shown that this region

is dynamically disordered in solution [33] As the

bind-ing epitope of BMPR-IA also seems to be not fully

folded in solution, formation of complexes of BMP

ligands and receptor seem to involve a large induced

fit mechanism This conformational rearrangement

upon binding might explain the promiscuous binding

of BMPs, as it allows the binding epitopes to adapt to

various different binding partners Furthermore, so

far, functional analysis has highlighted the importance

of the central hydrogen bond pair in

BMP-2–BMPR-IA for BMP type I receptor recognition [23] However,

in the binding of BMP-6 to ActR-I, a new, so far

unknown, main binding determinant has been

discov-ered This new hot spot of binding involves an

N-gly-cosylation motif conserved between BMP-2, BMP-7,

and BMP-6, which is specifically required for binding

of BMP-6 (and possibly BMP-7) to ActR-I but does

not play a role in binding to the other type I receptors

BMPR-IA and BMPR-IB This finding suggests that,

in addition to the above-mentioned flexible binding

epitope, usage of different main binding determinants

might also add to the broad binding specificity

observed in the BMP family

Experimental procedures

Expression and purification of recombinant

proteins

The mature part of hBMP-6, comprising amino acids 375–

513 plus an N-terminal extension MAPT (single-letter

amino acid code) [34], was expressed in E coli

Alterna-tively, a BMP-6 variant with residues 375–410 replaced with

the sequence MAQAKHKQRKRLK was used

(B2-BMP-6) The protein was expressed in insoluble form in inclusion

bodies BMP-6 isolated from these inclusion bodies was

refolded and purified as previously described [35]

Recombi-nant hBMP-6 obtained by eukaryotic expression, i.e CHO

cells, was purchased from R&D Systems (Minneapolis,

MN, USA) The extracellular domains of the receptors

BMPR-IA and BMPR-IB were expressed as

thioredoxin-fusion proteins in E coli and purified as previously

described [36] The extracellular domains of hActR-I

(resi-dues 21–123 [37]) and hActR-II (resi(resi-dues 18–135 [38]) were

expressed in baculoviral-infected Sf9 insect cells as

previ-ously described [20] The receptor proteins hActR-I and

hActR-II were purified by metal affinity chromatography

using Ni–nitrilotriacetic acid–agarose (Qiagen, Hilden, Germany); the eluate was dialyzed against HBS buffer (10 mm Hepes, pH 7.4, 3.4 mm EDTA, 20 mm NaCl), and subjected to anion exchange chromatography The flow-through of the latter step contains the monomeric, biologi-cally active receptor protein, which was then finally purified

by RP-HPLC

Interaction analysis by surface plasmon resonance

A BIAcore2000 system (BIAcore Life Science; GE Health-care, Freiburg, Germany) was used for all biosensor experi-ments Ligand proteins were directly immobilized onto a CM5 biosensor chip at a density of about 800 resonance units (1 RU = 1 pgÆmm)2), using the amine coupling kit (BIAcore Life Science; GE Healthcare) according to the manufacturer’s protocol Sensor chips were first activated

by perfusing an ethyl-N-(3-diethylaminopropyl)carbodi-imide (EDC)⁄ N-hydroxysuccinimide (NHS) mixture for

7 min; ligands were dissolved in 10 mm sodium acetate (pH 4.5) at a concentration of 1 lgÆmL)1and perfused over the activated chip surface until the required surface density was achieved Sensor chips were subsequently deactivated with 1 m ethanolamine (pH 8.0) for 7 min

All interaction experiments were carried out using HBS500 buffer (10 mm Hepes, pH 7.4, 500 mm NaCl, 3.4 mm EDTA, 0.005% surfactant P20) Sensorgrams of receptor–ligand interaction were recorded at a flow rate of

10 lLÆmin)1at 25C The association and dissociation time was set to 5 min After each cycle, 4 m MgCl2was perfused for 2 min of regeneration

Evaluation of recorded sensorgrams

Apparent binding affinities were calculated using biaevalu-ation software 2.2.4 Bulk face effects, i.e unspecific bind-ing to the biosensor or buffer exchange, were removed by subtracting a reference flow cell (FC1) from all sensor-grams Briefly, equilibrium binding constants of the interac-tion of BMP-2 with the type I receptors BMPR-IA and BMPR-IB were calculated by fitting the kinetic data to a

1 : 1 Langmuir binding model [KD(kin)], and those of the interaction of BMPs with hActR-I and hActR-II as well as

of BMP-6 and BMP-7 with BMPR-IA and BMPR-IB were determined from the dose dependency of the equilibrium binding [KD(eq)] The relative standard deviations for mean

KD(eq) values were below 25%; those of mean KD(kin) val-ues were below 50%

Deglycosylation of BMP-6

Recombinant hBMP-6 obtained from a eukaryotic expres-sion system (R&D Systems, Minneapolis, MN, USA) was

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fully or partially deglycosylated using either the

endogly-cosidase PNGase F (New England Biolabs, Frankfurt,

Ger-many), or a mixture of the endoglycosidases PNGase F3

and PNGase H (New England Biolabs) For complete

deglycosylation under nondenaturing conditions, 1 U of

PNGase F was used per microgram of BMP-6, with a

reac-tion time of 8 h at 37C For glycosylation trimming, a

mixture of 1 mU of PNGase F3 and 1 mU of PNGase H

was used per microgram of hBMP-6, with a reaction time

of 24 h at 37C The completeness of the carbohydrate

removal was analyzed by SDS⁄ PAGE and MS

Crystallization and structure analysis of BMP-6

Lyophilized E coli-derived wild-type BMP-6 and the

vari-ant B2-BMP-6 were dissolved in water at a concentration

of 5–10 mgÆmL)1 and submitted to crystallization trials

using Hampton Crystal Screens I and II (Hampton

Research, Aliso Viejo, USA) Crystals were obtained using

several sets of conditions and organic solvents, i.e

2-propa-nol, 2-methyl-2,4-pentanediol (MPD) or dioxane, or

poly-ethylene glycols (polypoly-ethylene glycol 4000 to polypoly-ethylene glycol 6000) Wild-type BMP-6 and the B2-BMP-6 crystal-lized under identical conditions, however, due to the increased solubility of B2-BMP-6, reproducibly yielded lar-ger crystals Suitable crystals of B2-BMP-6 grew from 25% MPD and 0.1 m sodium citrate (pH 4.0), and for wild-type BMP-6, the largest crystals were obtained from 20% 2-pro-panol and 0.1 m sodium citrate (pH 4.0) Diffraction data for B2-BMP-6 were collected from a single crystal at 100 K

at the beamline XS06SA at the Swiss Light Source (SLS; Paul Scherrer Institute, Switzerland), and data for wild-type BMP-6 were acquired at 100 K using a home source (Rigaku RU300, MarResearch Imageplate 345, Osmic Con-focalBlue) Data were processed using xds software [39] or HKL2000⁄ Scalepack [40]; a summary of the processing statistics is given in Table 3

Structure analysis was performed by applying molecular replacement using cns software [41] and the structure of BMP-7 (Protein Data Bank entry 1BMP [15]) as a search model The initial models were refined by iterative manual model building using quanta2006 software (Accelrys Inc.,

Table 3 Processing and refinement statistics Statistical analyses for the highest-resolution shell are shown in parentheses.

c = 86.8 A ˚

a = b = 90, c = 120

a = b = 97.7 A ˚ ,

c = 85.1 A ˚

a = b = 90, c = 120

Refinement statistics

rmsd

Procheck analysis c

a

Wild-type BMP-6 was analyzed for comparison The structures of BMP-6T2 and wild-type BMP-6 are identical within the accuracy of the resolution (rmsd of 0.7 for all Ca atoms); residues in the segment differing between BMP-6T2 and wild-type BMP-6 show no electron den-sity, indicating a high degree of flexibility b Cut-off for reflections F > 0r c Number of residues is shown in parentheses.

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San Diego, CA, USA), and either Refmac5 [42] or CNS

[41] was used for subsequent refinement Progress of

refine-ment was monitored using the R-factors Rcryst and Rfree;

the latter was calculated from a test dataset comprising 5%

of randomly selected reflections In the final rounds of

refinement, electron density difference maps Fobs– Fcalc

were used to identify 78 water molecules (for wild-type

BMP-6, 53 water molecules) and four MPD molecules (for

wild-type BMP-6, 10 2-propanol molecules could be

identi-fied) The final minimization cycle yielded R-factors of 25.9

for Rcryst and 27.9 for Rfree for B2-BMP-6 (for wild-type

BMP-6, Rcrystis 23.5 and Rfreeis 27.0)

ALP induction

The teratocarcinoma AT508-derived cell line ATDC5

(RIKEN, Ibaraki, Japan, No RCB0565) was cultured in

DMEM⁄ F12 (1 : 1) medium containing 5% fetal bovine

serum, and antibiotics (100 UÆmL)1 penicillin G and

100 lgÆmL)1streptomycin) For ALP assays, the cells were

serum starved (2% fetal bovine serum) and exposed to

ligands for 72 h in 96-well microplates After cell lyses,

ALP activity was measured by p-nitrophenylphosphate

con-version using an ELISA reader at 405 nm

BMP-induced inhibition of INA6 cell proliferation

Cells of the human myeloma cell line INA6 were seeded in

DMEM in 96-well plates at densities of 5· 103

cells per well The dose-dependent inhibition of proliferation was

measured by adding increasing concentrations of BMP-6,

BMP-7 (R&D Systems, Minneapolis, MN, USA) or

BMP-2 After 72 h, 10 lL of [3H]thymidine (0.25 lCi;

GE Healthcare⁄ Amersham, Munich, Germany) was added

to each well The cells were immobilized after 24 h on fiber

mats (Skatron Instruments A⁄ S, Lier, Norway), and the

thymidine incorporation was determined using a RITA

counter (Raytest, Straubenhardt, Germany) All assays

were performed in duplicate, and the experiments were

repeated twice

Acknowledgements

The authors thank Maike Gottermeier and Christian

So¨der for excellent assistance We wish to acknowledge

access to the X-ray facility at TU Munich (A Skerra)

and the beamline XS06SA at the Swiss Light Source,

and thank C Schulze-Briese for local support

References

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