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Structure and potential C-terminal dimerization of a recombinant mutant of surfactant-associated protein C in chloroform/methanol Burkhard Luy1, Alexander Diener2, Rolf-Peter Hummel3, Er

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Structure and potential C-terminal dimerization of a recombinant mutant of surfactant-associated protein C in chloroform/methanol

Burkhard Luy1, Alexander Diener2, Rolf-Peter Hummel3, Ernst Sturm3, Wolf-Ru¨diger Ulrich4

and Christian Griesinger5

1

Institut fu¨r Organische Chemie und Biochemie, Technische Universita¨t Mu¨nchen, Garching, Germany;2Institut fu¨r Organische Chemie, Johann Wolfgang Goethe-Universita¨t Frankfurt, Germany;3Department of Physical Organic Chemistry and4Department of Chemical Research, Altana Pharma AG, Konstanz, Germany;5Max Planck Institut fu¨r Biophysikalische Chemie, Go¨ttingen, Germany

The solution structure of a recombinant mutant

[rSP-C (FFI)] of the human surfactant-associatedprotein [rSP-C

(hSP-C) in a mixture of chloroform andmethanol was

determined by high-resolution NMR spectroscopy

rSP-C (FFI) contains a helix from Phe5 to the rSP-C-terminal Leu34

andis thus longer by two residues than the helix of porcine

SP-C (pSP-C), which is reportedto start at Val7 in the same

solvent Two sets of resonances at the C-terminus of the

peptide were observed, which are explained by low-order

oligomerization, probably dimerization of rSP-C (FFI) in

its a-helical form The dimerization may be induced by

hydrogen bonding of the C-terminal carboxylic groups or

by the strictly conservedC-terminal heptapeptide segment with a motif similar to the GxxxG dimerization motif of glycophorin A Dimerization at the heptapeptide segment wouldbe consistent with findings basedon electrospray ionization MS data, chemical cross-linking studies, and CNBr cleavage data

Keywords: dimerization; NMR spectroscopy; surfactant; surfactant protein C (SP-C)

Surfactant-associatedprotein C (SP-C) is a

34–35-amino-acidpeptide which is highly conservedamong species

(Table 1) It is part of the protein–phospholipidcomplex

that is secretedinto the alveolar space [1] andis

responsible for lowering of the alveolar surface tension

Recombinant (r)SP-C (FFI) surfactant (Venticute) has

provedto be highly effective in animal experiments [2,3]

as well as in pilot clinical trials [4,5] The structure of

porcine SP-C (pSP-C) has been solvedin CDCl3/CD3OH/

0.1M HCl (32 : 64 : 5, v/v/v), andit has been found

that the peptide forms an a-helix from residue 7 to the

C-terminal residue 34 [6] The N-terminal structure as well

as the hydrophobic a-helix seems to be conservedin the

micellar environment as shown for the N-terminal 17

residues of pSP-C in fully deuterated

dodecylphospho-choline micelles [7] A secondset of resonances was found

for the full-length pSP-C peptide in chloroform/methanol

at the C-terminus, which was explainedby partial

oxidation of the methionine residue M32 In general,

samples of the lipophilic pSP-C are not completely stable

in chloroform/methanol mixtures andform a gel-like

b-sheet aggregate after several days at 10C [8] A mutant

of the human SP-C (hSP-C) has been produced recom-binantly by omitting the residue [Phe() 1)] that is only partially present andperforming the following substitu-tions: C4F, C5F andM32I The rationale behindthe substitutions is that the two cysteine residues are naturally palmitoylated, which would have been difficult to achieve for a bacterially expressedprotein The mutation of residue 32 was to prevent the undesired putative oxidation

of methionine In this article, we present the structure of the rSP-C (FFI) mutant in CDCl3/CD3OH (1 : 1, v/v) with a comparison with the structure of pSP-C A second set of C-terminal signals is explainedby the coexistence

of monomeric andoligomeric (probably dimeric) rSP-C (FFI)

Materials and methods

Preparation of the sample For the studies on rSP-C (FFI) (Altana Pharma AG, Konstanz, Germany; WO patent no 95/32992), we usedthe solidsubstrate consisting of the peptide (90%), HCl (4%), propan-2-ol (3%), water (2%), andmethyl ester (1%) Samples of rSP-C (FFI) were preparedby dissolving 3–12 mg of the powder in 600 lL CDCl3/CD3OH (1 : 1, v/v) or CDCl3/CD3OD (1 : 1, v/v) The resulting

rSP-C (FFI) concentration was 1.1–4.4 mM, respectively The solidpeptide was storedat )20 C, andthe prepared samples were storedin liquidnitrogen between NMR measurements Dissolvedsamples hada lifetime of 72 h

at 10C Over time, the dissolved peptide maintained identical NMR chemical shifts, but strongly reduced intensity, indicating similar aggregation to b-sheet-like

Correspondence to C Griesinger, Max Planck Institut fu¨r

Biophysi-kalische Chemie, Abt NMR basedStructural Biology,

Am Fassberg 11, 37077 Go¨ttingen, Germany.

Fax: + 49 551201 2202, Tel.: + 49 551201 2201,

E-mail: cigr@nmr.mpibpc.mpg.de

Abbreviations: SP-C, surfactant-associatedprotein C; hSP-C, human

SP-C; pSP-C, porcine SP-C; rSP-C, recombinant human SP-C; rSP-C

(FFI), FFI variant of recombinant human SP-C; TACSY, taylored

correlation spectroscopy.

(Received17 December 2003, revised1 March 2004,

accepted23 March 2004)

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structures as observedfor natural pSP-C in the solvent used.

Because of the limitedlifetime, samples were prepared

immediately before NMR measurements

NMR measurements

2D1H-NMR spectra were recorded on Bruker DRX 800,

DMX 600, AMX 600 andAMX 400 spectrometers in the

pure-phase absorption mode using the States-TPPI method

[9] All spectra were recorded at 10C, andprocessing

andbaseline corrections were performedusing the standard

Bruker softwareXWINNMR The complete set of experiments

recorded is given in Table 2

The1H-NMR chemical shifts were calibratedrelative to

trimethylsilane The residual water signal and the signal

of the hydroxy proton of CD3OH are degenerate at

4.8 p.p.m andwere reducedusing presaturation [10]

Before Fourier transformation, the time domain data were

multipliedwith shiftedsquaredsinebell window functions

The vicinal scalar coupling constants 3JNHa were

deter-minedusing the SIAM-TACSY andKeeler–Titman

approaches [11,12] using macros written by T Prasch for

the programFELIX(Felix 95; MSI, San Diego, CA, USA)

Signal overlap in the 800-MHz NOESY made peak

integration unreliable So, instead, signal height of the

cross-peaks was usedfor a conservative estimation of the

maximum distances and classification of cross-peaks as

weak, medium and strong For the calibration of the

intensities of the NOE peaks, a statistical analysis of the

daN(i,i+3) signals of residues 11–30 was performed using

typical values for an ideal a-helix [13] The a-helical

structure of this part of the peptide is clearly evident from

Hachemical shifts [14,15]

Results

NMR assignment Sequence-specific 1H-NMR assignment was achievedby standard procedures for small proteins [13] using the computer program NDEE (Spin Up, Lu¨nen, Germany) Owing to the high abundance of the amino acids valine, leucine andisoleucine in the sequence of rSP-C (FFI), there was extensive overlap in the homonuclear1H-NMR spectra Nevertheless, almost all spin systems (vide infra) couldbe assignedfrom the TOCSY spectra (Fig 1A) andthe DQF-COSY spectra (not shown) collected under identical conditions (Table 3)

The unique spin systems His8, Lys10, Arg11 andAla29, andthe pairs of Phe andPro residues andGly28 andGly33 were unambiguously identified, as well as 10 of the 11 valines The N-terminal Gly1 shows a single very broad

HN/Hacross-peak Although all 34 amino acids were found, the spin systems of seven leucines, five isoleucines andthe residual valine could only be unambiguously identified using sequential NOE information

The high dispersion of the 800-MHz NOESY spectrum made it possible to obtain the complete assignment of

rSP-C (FFI) (Fig 1B,rSP-C) Starting from the unambiguously identified residues, we were able to carry out the sequential assignment for residues 1–17 and 24–34 by daNanddNN cross-peaks As an a-helical secondary structure was assumedfrom chemical-shift arguments, daN(i,i+3) and

daN(i,i+4) NOE cross-peaks were used, leading to the assignment of the residual amino acids 18–23

We encountered special difficulties in identifying the following connectivities: the chemical shifts of the amide

Table 2 NMR experiments.

Spectrometer frequency (MHz)

Data matrix

Processed matrix

Mixing time (ms)

Total time (h) 1.1 m M rSP-C (FFI) in CDCl 3 /CD 3 OH (1 : 1) TOCSY 600 4096 · 768 4096 · 1024 70 11

DQF-COSY 600 4096 · 1024 4096 · 1024 – 12 4.4 m M rSP-C (FFI) in CDCl 3 /CD 3 OH (1 : 1) NOESY 800 8192 · 1024 8192 · 1024 50 24 1.1 m M rSP-C (FFI) in CDCl 3 /CD 3 OH (1 : 1) SIAM-TACSY 600 4096 · 400 4096 · 1024 70 12 1.1 m M rSP-C (FFI) in CDCl 3 /CD 3 OH (1 : 1) NOESY 400 4096 · 1024 4096 · 1024 50 12

Table 1 Amino-acid sequences of several SP-Cpolypeptides, including human, porcine and recombinant human SPCwith FFI substitution [rSP-C(FFI)].

Species Amino-acidsequence

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protons of Leu21, Val23 andLeu31 are degenerate so there

was a large overlap in the dNNcross-peaks As the amide

protons of Ile22 andLeu30 also overlapped, the assignment

was even more difficult The identical Hachemical shifts of

Leu12, Leu13 andLeu21 causedfurther problems in the

sequential assignment The same occurredfor the dNN connectivities to Val27 because Ile26 andGly28 have almost identical amide proton chemical shifts Except for some side chain protons of Ile14, Ile22 andIle26, all1H resonances of rSP-C (FFI) were assigned Stereochemical assignments for

Fig 1 NMR assignment (A) Assignment of the spin systems of 32 nonproline residues out of the 34 amino acids of rSP-C (FFI) illustrated in the TOCSY experiment with a mixing time of 70 ms Shown is the so-calledfingerprint region where the well-dispersedHNprotons are correlatedto the H a andside chain protons The spin systems of Lys10 andArg11 are indicatedby rectangles as both contain a secondH N in the side chain The N-terminal Gly1 appears as a weak andvery broadpeak All H a chemical shifts of residues 5–31 show an upfieldshift comparedwith random-coil data indicating an a-helical structure in an empirical pattern-recognition approach [13,16] (B) HN-HNregion of the 800-MHz NOESY experiment Sequential d NN (i,i+1) connectivities can be found for all nonproline amino acids For the C-terminal residues 31–34, a second set of resonances can

be sequentially assignedindicatedby the prime in the annotation of the corresponding NOE connectivity (C) H N -H a region of the 800-MHz NOESY experiment All resolved interresidual NOE connectivities are annotated In particular, the d Na (i,i+3) andd Na (i,i+4) connectivities are indicators of an a-helical secondary structure Intraresidual signals are not annotated (D) Summation of the experimental NMR data Shown are all resolvedNOE connectivities, where thin bars indicate distances > 4.0 A˚, medium bars distances of 3.0–4.0 A˚, andthick bars distances < 3.0 A˚ The d Na (i,i+3), d Na (i,i+4) as well as the d NN (i,i+2) andthe strong d NN (i,i+1) connectivities clearly show the a-helical structure of rSP-C (FFI) In addition,3J NHa coupling constants are summarized, with small circles indicating couplings < 5.0 Hz and large circles for constants > 6.0 Hz Pentagons classify the exchange properties of amide protons in weak exchange (filled pentagons), medium exchange (open pentagons) and strong exchange (no pentagon) as described in the text.

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the diastereotopic groups were inferred from NOEs

through floating chirality calculations

Second set of resonances

Closer inspection of the spectra revealedtwo sets of

resonances for Gly28, Leu30, Ile32, Gly33 andLeu34,

which differ mainly in the chemical shifts of the amide

protons andthe c protons of Ile32 andLeu34 A

compar-ison of the spectra showeddifferent relative intensities of the

two sets of resonances with respect to the concentration of

rSP-C (FFI) in CDCl3/CD3OH (1 : 1, v/v) andthe age of

the sample For a systematic analysis, freshly prepared

samples with concentrations of 0.7–3.5 mM were usedin

NOESY experiments with a mixing time of 50 ms At low

concentration, the two sets of signals were almost equally strong, whereas at higher concentrations of rSP-C, one of the signal sets was more predominant Attempts to fit the relative intensities of the two sets of resonances to a quantitative monomer–dimer equilibrium model failed (data not shown) However, the concentration dependence shown in Fig 5 can be considered an indication of intermolecular interaction The comparable linewidths of the signals of the two sets of resonances still suggest that monomeric and dimeric units are involved

Amide proton exchange The exchange properties of the amide protons were

rSP-C (FFI) in CDCl3/CD3OD (1 : 1, v/v) with the sample freshly preparedabout 1 h before the experiment All measurable Ha-HNcross-peaks were integratedandcom-paredwith the integrals of the 800-MHz NOESY spectrum The most intense signals were taken as 100% relative intensity, making the assumption that no significant exchange occurredin the given time frame within the center

of the well-ordered a-helix The relative intensities of the

Ha-HNcross-peaks of residues His8, Ala29 and Leu31 were about 50% of those recorded in the 800-MHz NOESY spectrum in CDCl3/CD3OH (1 : 1, v/v), andthe intensities

of residues 9–28 and 30 were 80% or higher From these estimates of the relative intensities, hydrogen bonds for the structure calculations were assumedfor His8 to Leu31 The amide protons of residues 1–7 and 32–34 could not be detected in the fully deuterated solvent

Structure of rSP-C (FFI) Using the empirical pattern-recognition approach [16], the combination of strong sequential dNNconnectivities, obser-vation of a significant number of daN(i,i+3), dab(i,i+3), anddaN(i,i+4) connectivities,3JNHacoupling constants of less than 5 Hz for all non-Gly residues in the polypeptide segment Phe5, Val7–Leu30, andretardedamide proton exchange for residues 8–31 indicate that rSP-C (FFI) forms

a long a-helix comprising approximately residues 5–34 For a more precise definition of the structure of

rSP-C (FFI), a set of 203 intraresidual, 201 interresidual and seven ambiguous NOE-derived upper distances were used together with 23 / angles derived from3J(HN,Ha) coupling constants as input data for a structure calculation using the programXPLOR[17] In addition, we introduced 24 hydro-gen bonds derived from the slow exchange rate of the amide protons No stereospecific assignments were usedin the floating chirality simulatedannealing protocol For residues 28–34, we usedonly the set of resonances with the stronger intensities because identical relative NOEs were observed for the two species

For the structure calculations, we useda standard simulatedannealing protocol designedfor proteins [18] After an initial energy minimization involving 50 optimiza-tion steps with conjugatedgradients, a high temperature phase with 2000 K was simulatedfor 32.5 ps in which all upper limits built the active constraints The following step was the first cooling phase from 2000 K to 1000 K in 25 ps with the dihedral angles as additional constraints After the

Table 3 Chemical shifts of rSP-C (FFI).

Residue H N H a H b Others

Gly1 8.23 3.73

Ile2 8.61 4.45 1.90 c1.66, 1.00; d1.23,0.95

Pro3 4.38 2.15, 1.99 c2.10; d3.95, 3.72

Phe4 8.08 4.49 3.18, 3.09 d7.17; e7.27; f7.19

Phe5 8.46 4.68 3.29 d7.28; e7.42; f7.36

Pro6 4.25 2.34, 2.00 c2.14; d3.65

Val7 7.62 3.69 2.28 c1.13, 1.01

His8 8.05 4.47 3.35, 3.29 d7.22; e8.74

Leu9 8.13 3.97 1.70, 1.60 c1.65; d1.03, 0.98

Lys10 7.95 3.91 2.03 c1.64, 1.50; d1.79;

e2.93; f2.92 Arg11 7.89 3.94 2.02, 1.99 c1.70; d3.30, 3.24; e7.50;

1g7.18; 2g6.68 Leu12 7.82 4.01 1.69 c1.81; d0.94

Leu13 8.04 4.01 1.89 c1.71; d0.95

Ile14 7.77 3.64 2.08 c1.94, 1.20; d0.97, 0.93

Val15 7.67 3.52 2.40 c1.17, 1.01

Val16 8.01 3.51 2.33 c1.15, 1.00

Val17 8.03 3.52 2.33 c1.16, 1.02

Val18 8.15 3.57 2.32 c1.14, 1.03

Val19 8.36 3.57 2.32 c1.15, 1.00

Val20 8.35 3.49 2.31 c1.15, 1.00

Leu21 8.25 4.01 1.99, 1.93 c1.75; d1.02, 0.94

Ile22 8.30 3.60 2.16 c1.17; d0.99

Val23 8.25 3.53 2.40 c1.16, 0.99

Val24 8.59 3.57 2.42 c1.17, 1.02

Val25 8.28 3.71 2.39 c1.17, 1.03

Ile26 8.45 3.70 2.09 c1.95, 0.98; d1.17

Val27 8.93 3.59 2.23 c1.14, 1.02

Gly28 8.45 3.88, 3.77

8.42 3.86, 3.77

Ala29 8.21 4.09 1.62

8.19 4.08 1.62

Leu30 8.25 4.18 2.12, 2.03 c1.60; d0.99

Leu31 8.30 4.14 2.07 c1.60; d0.99, 0.96

8.28 4.13 2.07 g1.60; d0.99,0.96

Ile32 7.70 4.35 2.18 c1.64,1.51; d1.02

7.64 4.38 2.18 g1.61,1.55; d1.02

Gly33 7.95 4.09, 3.87

7.91 4.09, 3,85

Leu34 8.02 4.51 1.73 c1.78; d1.00

8.08 4.55 1.64 g1.74; d1.00

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secondcooling phase from 1000 K to 100 K in 10 ps, a

secondenergy minimization was performedwith 200 steps

of conjugatedgradients for each structure The rmsdvalues

and the distance and dihedral angle violations for the best 10

out of 60 structures are given in Table 4 The final structures

shown in Figs 3 and 4 were determined by an additional

refinement in vacuo including the experimental restraints,

full charges, anda dielectric constant set to e¼ 4rijusing a

heating andcooling protocol

Figure 2 showsMOLMOLstereographic projections [19] of

the heavy atoms of C (FFI) The structure of

rSP-C (FFI) is a well-defined a-helix ranging from Phe5 to

Leu34 Note that the distribution of the / and w angles

indicates an a-helical structure up to Phe5, although residue

6 is a proline Strong evidence for this comes from the

unambiguously identified daN(i,i+3) anddaN(i,i+4)

cross-signals for Phe5 andPro6 (cf Fig 1D)

Discussion

Comparison of rSP-C (FFI) with pSP-C

The 34-residue peptide rSP-C (FFI) contains mainly

apolar amino acids, i.e 11 valines, seven leucines and

five isoleucines, andforms a well-defineda-helix along

residues 5–34 dissolved in CDCl3/CD3OH (1 : 1, v/v) The

solution structure of pSP-C with 76% sequence identity

(Table 1) in CDCl3/CD3OH/0.1MHCl (32 : 64 : 5, v/v/v)

was investigatedby Johansson et al [6] To compare the

structure of pSP-C with rSP-C (FFI), we show in Fig 3

the differences in chemical shifts of the HNandHasignals

of the corresponding residues It can be seen that the

chemical shifts for residues 10–29 are almost identical,

with slightly greater variations at nonidentical amino

acids Only the N-terminal nine residues show significant

chemical-shift differences mainly introduced by the

sequence deviations at residues 4, 5 and 8 This difference

at the N-terminus can also be seen when the two resulting

structures shown in Fig 4 are compared Whereas the

backbone of the central a-helix is very well defined in

both structures, the N-terminal variability for the pSP-C is

greater than that of rSP-C (FFI) This reflects the

NOE-data-based fact that rSP-C (FFI) has a defined a-helix

comprising residues 5–34, whereas for pSP-C an a-helical region at residues 7–34 has been reported [6]

However, the slow deuterium exchange for Leu9 and small distances dNa(i,i+3) anddab(i,i+3) for Pro6 andVal7 suggest that even pSP-C adopts an a-helix starting with capping at residue Cys5 [8] Substitution of acylated Cys with Phe in the polypeptide seems to influence the N-terminal a-helix formation including Pro6 in rSP-C (FFI) A possible explanation is the occurrence of aromatic interactions between Phe5 andHis8 which may leadto stabilization of the extendeda-helix The structures

of both pSP-C andrSP-C (FFI) were determinedin chloroform/methanol, an environment in which hydropho-bic elements can move freely Membranous environments such as the surfactant, however, have a directional effect on the hydrophobic palmitoylatedCys andPhe residues andon the chargedLys andArg residues at positions 10 and11, which probably results in slightly different N-terminal structures for the SP-C variants in their biologically active form

The central helix of pSP-C has a slightly lower rmsdvalue than that of rSP-C, probably because of the longer stretch of Val residues, leading to extremely stable stacking In

rSP-C (FFI) this homogeneous stacking is interruptedby Ile14 andIle22, which may introduce slight mobility into the hydrophobic a-helix However, this increasedmobility still leaves the central helix quite rigidanddoes not seem to be important, as it was shown in mutation studies that SP-C retains its function even after the replacement of all valines

by leucines or other a-helical amino-acidsequences [20,21] Two sets of resonances

Two sets of resonances were foundfor rSP-C (FFI) at the C-terminal residues Gly28, Leu30, Ile32, Gly33 and Leu34 Similar duplication of resonances has been reported for pSP-C, affecting residues Val27, Ala29, Leu30, Leu31 and Met32 [6] In the case of pSP-C, the additional signals were explainedby partial oxidation of Met32 to methionine sulfoxide In the case of rSP-C (FFI), a different explan-ation must be foundfor the secondset of resonances because Met32 is substitutedby Ile32 The careful studies on pSP-C show a variation of 20–50% of the minor populated

Table 4 Analysis of the 10 best calculated structures before and after the refinement.

E tot (kcalÆmol)1) 165.9 ± 12.7 (142.4.181.6) ) 265.4 ± 7.5 ()266.9 … )246.1) Distance violations

Torsion-angle violations

Rmsds (A˚)

Heavy atoms (8–33) 1.05 ± 0.18 (0.90.1.44) 0.82 ± 0.13 (0.67.1.00) Backbone (18–28) 0.23 ± 0.08 (0.14.0.41) 0.07 ± 0.02 (0.04.0.10) Heavy atoms (18–28) 0.61 ± 0.08 (0.51.0.77) 0.45 ± 0.12 (0.35.0.68)

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set of resonances among samples preparedfrom different

batches We observedthe same variation even in samples

preparedfrom the same batch A closer look at the acquired

spectra indicates a dependence of the relative population

of the signals on the overall SP-C concentration As a

consequence, we acquireda set of 2D NOESY spectra with

identical mixing times but different concentrations of

rSP-C (FFI) in rSP-CDrSP-Cl3/CD3OH (1 : 1, v/v) The relative

popu-lations of the two sets of resonances in these spectra with

respect to the overall SP-C concentration are shown in

Fig 5 The dependence observed is a clear indication of

intermolecular interaction The relatively narrow linewidths

of the observedsignals ledto the conclusion that oligomers

of low order are present, probably monomeric and dimeric

units, but trimeric or tetrameric units may also be possible; larger oligomers can be excluded because the linewidths wouldhave to be significantly broader than observed The linewidths of the two sets of resonances do not differ significantly, therefore the two oligomers must be of comparable size, anda monomer/tetramer equilibrium, for example, cannot explain the observedsignals The absence of further resonances implies that we are observing specific oligomers Finally, chemical shifts of the Ha resonances are a clear indication that both oligomers are mainly a-helical andthat their structures differ only slightly The NMR data therefore point to the coexistence of

a monomeric andhomodimeric a-helical form of rSP-C (FFI)

Fig 2 Stereographic projection of the best 10

out of 60 structures of rSP-C(FFI) (A) Side

view of the heavy atoms of the full-length

peptide (B) View from the bottom along

residues 15–27 of the tightly packed a-helix.

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The literature on SP-C describes many oligomerization

processes, most of which are either aggregates with mainly

b-sheet-like or undetermined structure Specific

oligomeri-zation, i.e dimerioligomeri-zation, is only reported in a few cases:

MS data provide evidence for dimeric SP-C [22,23], and

chemical cross-linking studies also show mainly a specific

dimer of mature SP-C (Fig 8C in [24]) Yet unpublished

high-resolution Fourier-transform ion-cyclotron-resonance

MS, light-scattering andCD experiments reveal the

exist-ence of an a-helical dimer at acidic pH ([25]; A Seidl,

G Maccarone, N Youhnovski, K P Schaefer and

M Przybylski, unpublished data) CNBr cleavage data

even put the dimerization site near Met32 at the C-terminus,

i.e at the site at which the dual resonances are observed [23]

The coexistence of monomeric andhomodimeric

rSP-C(FFI) as derived from the NMR data therefore

corres-ponds well to other reported experimental observations

Fibril formation

The data from Fig 5 couldnot be fittedto a simple

monomer–dimer equilibrium model, but this is not

surpri-sing considering that rSP-C (FFI), like pSP-C, shows a

complete transition to b-sheet fibrils over time [8,26,27]

Immediately after rSP-C (FFI) is dissolved in chloroform/

methanol, short, fiber-like impurities of up to 1 mm length

are observedin solution andon the glass walls of the NMR

tube on visual inspection This indication of already formed

fibrils makes it necessary to describe rSP-C (FFI) by at least

a three-state model with two a-helical states, probably

monomer anddimer, andb-sheet fibrils that cannot be

observedby high-resolution NMR because of their high

molecular mass A three-state model with monomeric, nonhelical and b-fibril states has already been presented [8] Interestingly, the existence of an a-helical transition state (SPC#in [8]) was proposedin that publication, which would

Fig 4 Comparison of the 10 best structures of rSP-C (FFI) (left) and pSP-C(right) The backbone of the a-helix is shown Clearly visible

is the better defined secondary structure of rSP-C (FFI) near the N-terminus.

Fig 5 Concentration dependence of the relative integrals of the two sets

of resonances observed at the C-terminus Ratios are given for well-resolvedresidues Ile32, Gly33 andLeu34.

Fig 3 Differences in the chemical shifts of rSP-C(FFI) compared with

pSP-C[6] for the H N (A) and the H a protons (B) Whereas residues

10–29 show almost identical chemical shifts, residues at the N-terminus

andC-terminus differ more strongly.

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match the potential a-helical dimer found here The

interpretation of the potential dimeric state as the transition

state to b-fibril formation wouldalso match recent

solid-state andliquid-solid-state NMR results, which suggest that the

smallest fibril diameter in b-amyloidfibrils is due to a

parallel b-sheet dimer [28,29] and also that the minimum

unit needed for fibril growth of a-synuclein is a dimer [30]

The disappearance of high-resolution NMR signals after

several days at 10C in chloroform/methanol shows that

the equilibrium state of rSP-C (FFI) is the b-sheet-like

multimer The a-helical states are therefore not equilibrated,

and, in addition to the observed concentration dependence,

a dependence on the age of the prepared samples can be

predictedin the given solvent It shouldbe notedthat

neither rSP-C (FFI) nor pSP-C [8] show any transition to

b-fibrils in dodecylphosphocholine micelles even after

several weeks at room temperature

Sample handling and the situationin vivo

SP-C is very difficult to handle In general, basic conditions

shouldbe avoidedandproperties of the molecule depend

strongly on the conditions for synthesis, the kind of

purification used, and the aggregation states it was

trans-ferred to In this study, we relied on the elaborate procedure

developedby Altana Pharma andonly suspendedthe

powder provided directly in chloroform/methanol The

NMR spectra yieldedgoodresults andtherefore there

appearedto be no needto change the method Whether

oligomerization can be avoided by different sample

treat-ment remains to be proven

The local environment of the molecule also has a large

impact on its behavior Wild-type SP-C, like rSP-C (FFI),

is solely monomeric at micromolar concentrations in pure

organic solvents but has a strong tendency to aggregate in

more hydrophilic environments Relatively high

concentra-tions can be obtained in dodecylphosphocholine micelles in

which SP-C is stable for months in its a-helical form [8] The

surfactant consists of 1% by weight of SP-C [31] The

concentration of rSP-C (FFI) andextractedpSP-C in

the NMR studies is therefore similar to the concentration

of SP-C in its natural environment, although it shows a slow

transition to b-sheet fibrils However, whether the

homo-dimer in chloroform/methanol is representative of the

biologically active SP-C in the surfactant cannot be judged

from the experiments presented A hint may be gained from

chemical cross-linking data on mature SP-C in cytosolic

vesicles of A549 cells (Fig 8C in [24]), which provide

evidence of dimer formation during trafficking

Potential dimerization site

The evidence suggests dimerization of SP-C, and it might be

allowedto speculate on the potential dimerization site The

C-terminus of rSP-C (FFI) only contains apolar side chains

andit can be assumedthat it is situatedat the hydrophobic

palmitoyl chains of the surfactant phospholipids In this

environment, hydrogen-bonding interactions and strong

hydrophobic associations are most likely to be the source of

intermolecular attraction A minor dimerization motif can

be foundin the C-terminal carboxylic group Similar to the

dimer formation of acetic acid, SP-C may form a dimer via

hydrogen bonding (Fig 6A) The acidic conditions of the NMR sample as well as the natural environment of SP-C would allow such a dimer formation However, in the acidic NMR sample, relatively fast hydrogen exchange rates are expectedwhich do not match the slow exchange regime observedfor the two sets of resonances Therefore, hydro-gen bonding of the carboxylic group is unlikely to be the cause of the observeddimerization, but we cannot exclude it

An alternative dimerization motif can be found in the strictly conservedC-terminal heptapeptide segment-span-ning residues Gly28 to Leu34: the heptapeptide segment

of rSP-C (FFI), as well as all other SP-C variants, has an AxxxG pattern that perfectly matches the requirements for helix–helix association as described in [32] Interestingly, the residues for which double resonances are observed are all within the strictly conservedheptapeptide segment with the AxxxG motif (Fig 6B) Attempts to model two distinct structures for the two sets of resonances failedbecause of massive overlap of the side chain resonances in the region

of interest in particular However, as mentionedabove, we can conclude from chemical-shift arguments that the two structures shouldbe very similar andare a-helical in character For the same reasons, it was impossible to obtain

a structure of the potential dimer based on intermonomeric NOEs A theoretical model based on the monomeric structure presentedin this paper andcomputational dock-ing studies is derived in the followdock-ing paper [33]

Conclusion

We have derived by NMR spectroscopy the high-resolution 3D structure of rSP-C (FFI) dissolved in CDCl3/CD3OH (1 : 1, v/v) The lipophilic peptide forms a tight a-helix for residues 5–34 which is two residues longer than the a-helix

Fig 6 Potential dimerization motifs for rSP-C(FFI) (A) Hydrogen bonding at the C-terminal carboxy group may lead to dimerization (B) Comparison of the amino-acidsequences of glycophorin A and rSP-C (FFI) shows a potential AxxxG dimerization motif similar to the van der Waals dimer of glycophorin A [33–35] at the strictly conservedheptapeptid e segment where two sets of resonances are observed.

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observedin pSP-C, with 76% sequence identity in the same

solvent Both peptides show two sets of resonances for a

number of C-terminal residues Because of the lack of Met

we can exclude oxidation to methionine sulfoxide as the

cause of the secondset of resonances for rSP-C (FFI),

which was previously assumedin the case of pSP-C [6]

Studies on the concentration dependence of the dual

resonances together with the narrow linewidth of the

NMR signals suggest the coexistence of a monomeric and

dimeric a-helical structure in the given solvent There are

two potential dimerization sites in SP-C: the C-terminal

carboxylic group may form a dimer via hydrogen bonding;

the C-terminal heptapeptide segment, which is conserved in

all known SP-C species, contains an AxxxG motif that

closely resembles the GxxxG helix–helix dimer motif of

glycophorin A Even though the latter dimerization motif is

consistent with other experimental results andtherefore

highly likely, additional studies such as point mutations at

the potential dimerization site are necessary to

unambigu-ously determine the origin of the intermolecular interaction

that leads to the second set of resonances

Acknowledgements

C.G gratefully acknowledges support from the DFG, the MPG, and

the Fonds der Chemischen Industrie B.L andA.D were supportedby

the Fonds der Chemischen Industrie B.L is also supportedby the DFG

(Emmy Noether LU 835/1–1) We thank Bettina Elshorst for help with

NDEE , Michael Nilges for help with the XPLOR protocols, andMichael

Przybylski (University of Konstanz) for providing his results before

publication Special thanks go to Michael K Gilson (CARB, Rockville,

MD, USA) for many detailed scientific discussions.

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