Biochemical Journal
Biochem. J. (1996) 316, 923‚935 (Printed in Great Britain)
Structural composition and functional characterization
of soluble CD59: heterogeneity of the oligosaccharide
and glycophosphoinositol (GPI) anchor revealed by
laser-desorption mass spectrometric analysis
Seppo MERI*ß, Timo LEHTO*, Chris W. SUTTONð, Jaana TYYNELŸ›
and Marc BAUMANN›
*Department of Bacteriology and Immunology, Haartman Institute, University of Helsinki,
Finland, ðThermo Bioanalysis Ltd, Hemel Hempstead, U.K., and ›Department of Medical
Chemistry, FIN-00014, University of Helsinki, Finland
Abbreviations used: MAC, membrane attack complex of complement; MALDI-MS,
matrix-assisted laser desorption mass spectrometry; RP-HPLC, reverse-phase HPLC; GPI,
glycophosphoinositol; HRF, homologous restriction factor; CD59E, CD59U, erythrocyte and
urinary forms of CD59; DAF, decay accelerating factor; GPE, guinea pig erythrocytes; PNGase
F, peptide N-glycosidase F; TCC, terminal complement complex; uPAR, receptor for
urokinase-type plasminogen activator; NHS, normal human serum; TFA, trifluoroacetic acid;
ACH, -cyano-4-hydroxycinnamic acid.
ß To whom correspondence should be addressed.
CD59 (protectin) is a glycophosphoinositol (GPI)-anchored inhibitor of the membrane attack
complex of complement found on blood cells, endothelia and epithelial cells. In addition to the
lipid-tailed CD59, soluble lipid-free forms of CD59 are present in human body fluids. We
have investigated the detailed structural composition of the naturally occurring soluble
urinary CD59 (CD59U) using peptide mapping, anion-exchange chromatography, sequential
exoglycosidase digestion and matrix-assisted laser-desorption mass spectrometry
(MALDI-MS). CD59U exhibited an average Mr of 12444 in MALDI-MS. Mass analysis of the
isolated C-terminal peptide (T9) indicated that a GPI-anchor (at Asn-77) without an
inositol-associated phospholipid was present in soluble CD59U. By using residue-specific
exoglycosidases, chemical modification and MALDI-MS structures of seven different
GPI-anchor variants were determined. Variant forms of the anchor had deletions and/or
extensions of one or more monosaccharide units. Sialic acid linked to an
N-acetylhexosamine-galactose arm was found in two GPI-anchor variants. The N-linked
carbohydrate side chain of CD59U (at Asn-18) also displayed considerable heterogeneity. The
predominant oligosaccharide chains were fucosylated biantennary and triantennary complexes
with variable sialylation. Mono Q anion-exchange chromatography resolved urinary CD59
into nine different fractions that bound equally well to the terminal complement SC5b‚8
complexes. Despite binding to C5b‚8, soluble CD59U inhibited complement lysis at an approx.
200-fold lower efficiency than erythrocyte CD59. These results document the structural
heterogeneity of both the GPI anchor and N-linked oligosaccharide of CD59 and demonstrate
that the phospholipid tail is needed for the full functional activity of CD59. The site of cleavage
between the diradylglycerol phosphate and inositol suggests that a mammalian phospholipase D
could be involved in the solubilization of GPI-anchored proteins.
INTRODUCTION
Activation of the plasma complement (C) system leads to formation of a cytolytic membrane
attack complex (MAC) composed of the terminal C components C5b, C6, C7, C8 and multiple
copies of C9 (reviewed in ref. [1]). While the main purpose of MAC is to destroy invading
micro-organisms, the cells of the host have to be protected against self-destruction. Damaging
effects of MAC are controlled by regulatory molecules which act either in the fluid phase or on
the cell membrane. The human plasma protein, S-protein (vitronectin), is a multifunctional
protein that acts as an acceptor for the aggregating late complement components and keeps
them as a soluble non-cytolytic terminal complement complex (TCC) [2]. S-protein binds to
the C5b67 complex and inhibits C9 polymerization by SC5b‚8 [3]. Another body fluid
protein, clusterin (SP40,40), binds to the TCC and appears to be capable of keeping it soluble
in a manner similar to S-protein [4,5].
The CD59 antigen (MACIF, MIRL, HRF-20 or protectin) is a major inhibitor of the MAC
present on human cell membranes [6‚10]. CD59 inhibits complement lysis by preventing
C5b‚8-catalysed insertion and polymerization of C9 into cell membranes [11,12]. The
homologous restriction factor (HRF, C8bp, MIP) is another proposed inhibitor of MAC that
binds to C5b‚8 [13‚15]. CD59 and HRF are anchored to cell membranes via a
glycophosphoinositol (GPI) anchor. Soluble forms of HRF have been found in human urine,
plasma and cerebrospinal fluid, where they appear to exert a limited complement inhibitory
function [15,16]. A soluble form of CD59 was initially reported in human urine [7]. CD59 is
strongly expressed in the epithelia of many secretory organs [17] and has been detected in
several body fluids [18‚22a]. The presence of a lipid tail has been demonstrated for CD59 in
amniotic fluid [18], seminal plasma [19] and breast milk [22]. In blood plasma CD59 has
been detected in association with lipoprotein particles [23]. Recently, we observed that both
soluble and lipid-tailed forms of CD59 are present in human urine [24].
The three-dimensional structure of soluble urinary CD59 [25] or of a recombinant CD59
composed of amino acid residues 1‚70 [26] has recently been determined by two-dimensional
NMR spectroscopy. The overall folding of CD59 resembles that of snake venom neurotoxins
[27]. The disulphide bonding pattern of the ten cysteines of CD59 [26,28] defines a distinct
domain that is found, for example, in the Ly-6 family of putative T-cell activation antigens
[29] and in triplicate in the urokinase-type plasminogen activator receptor (uPAR, CD87)
[30]. The CD59 domain has two antiparallel -sheets, one with three strands and another
with two, that create a disc-shaped structure with extending loops [25,26].
The GPI anchor of CD59 is attached to the C-terminal Asn-77 residue and links the CD59
polypeptide to phospholipid. Initial biochemical analysis has indicated that the GPI-anchor
structure resembles that of Thy-1 [31] in having a core structure of
ethanolamine-PO4-(±Man1-2)Man1-2Man1-6Man(PO4-ethanolamine;
±GalNAc)-GlcNH21-6myo-inositol [32]. Using sequential exoglycosidase digestion Nakano
et al. [32] have also examined the Asn-18-linked oligosaccharide of CD59 and report a
biantennary complex-type structure. Detailed structural analysis of the oligosaccharide is
warranted because glycosylation of CD59 has been suggested to be important for its
complement lysis-inhibiting [33] and putative T-cell co-stimulating or signalling activities
[34,35].
In the present study we have characterized in detail structural and functional properties of
soluble urinary CD59 (CD59U). CD59U was carboxyamidomethylated, digested with trypsin,
and the resulting peptides separated by reverse-phase HPLC (RP-HPLC). The N-linked
glycopeptide and GPI-anchor peptide conjugates were hence isolated from each other to allow
separate structural analysis using residue-specific exoglycosidases, chemical modification
and matrix-assisted laser desorption mass spectrometry (MALDI-MS).
The results reveal a whole spectrum of structures of both the oligosaccharide linked to
Asn-18 and of the GPI anchor (at Asn-77). The latter include previously undescribed
variants containing sialic acid. The fact that the GPI anchor of CD59U does not have an
inositol-associated phospholipid suggests that mammalian phospholipase D could solubilize
GPI-anchored glycoproteins that have become shed from cell surfaces. The soluble isoforms of
CD59 retain their specific binding activity towards the TCCs but, because of the absent
phospholipid tail, they only have a limited ability to inhibit MAC assembly on cell membranes.
MATERIALS AND METHODS
Antibodies and reagents
Rat hybridoma cells producing an anti-CD59 mAb (YTH53.1; kindly obtained from Professor
H. Waldmann, Department of Pathology, University of Cambridge, Cambridge, U.K.) were
grown in Dulbecco's modified Eagle's medium supplemented with 10% (v/v) IgG-free fetal
calf serum (Gibco). The IgG fraction was collected from the culture supernatants by protein
G‚Sepharose chromatography (Pharmacia Biotechnology, Uppsala, Sweden). Mouse anti-CD59
(BRIC 229) monoclonal antibody was from International Blood Group Reference Laboratory
(Bristol, U.K.). The following supplies were purchased as indicated: Lubrol PX, Triton
X-100, Nonidet P-40, n-octyl glucoside, EDTA, hydrofluoric acid, inulin, trypsin (from
bovine pancreas), Arthrobacter ureafaciens sialidase (Sigma Chemical Co., St. Louis, MO,
U.S.A.); endoproteinase Lys-C (Boehringer Mannheim, Mannheim, Germany),
Flavobacterium meningosepticum peptide N-glycosidase F (PNGase F), Streptococcus
pneumoniae galactosidase, galactosidase from bovine testes, S. pneumoniae
N-acetylglucosamidase, chicken liver N-acetylglucosamidase, jack bean -mannosidase,
Helix pomatia mannosidase, jack bean N-acetylhexosaminidase (Oxford Glycosystems, Oxon.,
U.K.). Sera depleted of the terminal complement components (C7DS, C8DS and C9DS) were a
kind gift from Dr. B. P. Morgan, University of Wales College of Medicine, Cardiff, U.K. C56
complexes were isolated from a pool of acute-phase sera by euglobulin precipitation,
gel-filtration and anion-exchange chromatography on a DEAE-Sephadex column (Pharmacia
Biotechnology). The purity of C56 was examined by SDS/PAGE and activity titrated in a
reactive lysis system using normal human serum (NHS)‚EDTA and guinea pig erythrocytes
(GPE).
Isolation of soluble and membrane forms of CD59
The erythrocyte membrane (CD59E) and urinary forms of CD59 were purified by
YTH53.1-Sepharose 4B affinity chromatography [7,11]. To obtain soluble urinary CD59
(CD59U), the possibly remaining lipid-tailed CD59 associated with small micelles or
vesicles in the urinary CD59 preparation was removed by centrifugation at 100000 g for 1
h followed by gel filtration on Superose 6 (Pharmacia Biotechnology) [24]. Proteins were
radiolabelled with 125I using the Iodogen method (Pierce Chemicals, Rockford, IL, U.S.A.). The
initial specific radioactivities of the radiolabelled proteins in representative samples were:
CD59E, 2x107 c.p.m./g; and CD59U, 1.3x107 c.p.m./g respectively. As compared with
unlabelled CD59E the 125I-labelled CD59E retained 80% of its complement lysis inhibitory
activity in a reactive lysis assay system [11].
Mono Q anion-exchange chromatography and SDS/PAGE
Affinity-purified urinary CD59 was chromatographed on an HPLC Mono Q column (HR 5/5,
Pharmacia Biotechnology) equilibrated with 20 mM Tris/HCl, pH 7.2, and eluted with a
linear gradient of 0 to 200 mM NaCl in the equilibration buffer. The eluting proteins were
monitored on-line by absorbance at 218 nm. SDS/PAGE was performed according to Laemmli
[36] using 12% or 15% Protean II minigels (Bio-Rad Laboratories, Richmond, CA, U.S.A.).
Enzymic digestions and amino acid sequence analysis
Urinary CD59 was subjected to A. ureafaciens sialidase, 1.0 IU/ml, treatment in 50 mM
Tris/HCl, pH 5.5, at 37 ƒC overnight, diluted with 2 vols. of Milli-Q water and applied on to
the Mono Q column. PNGase F digestion of CD59U (for PNGase F digestion of N-linked
glycopeptide see Table 1) was carried out in 50 mM potassium phosphate buffer, pH 6.1,
containing 1% n-octyl glucoside, 0.1% SDS and 5 mM EDTA at 4 ƒC for 16 h (in the presence
or absence of -mercaptoethanol). Endoproteinase Lys-C and trypsin digestions were carried
out in 1% NH4HCO3 solution. Purified CD59 (CD59E or CD59U) was incubated with 1.5%
(w/w) of enzyme for 2 h at 37 ƒC followed by addition of another 1.5% (w/w) of the same
enzyme. The incubation was continued at 37 ƒC for 2‚4 h. The released peptides were
separated by RP-HPLC. Sequence analysis was performed by using automated Edman
degradation and a modified Applied Biosystems 477A/120A sequencer in the pulsed liquid
mode [37].
Table 1 Specificities and compositions of glycosidase mixtures
Abbreviations: GlcNAcase, -N-acetylglucosaminidase; PNGase F, peptide N-glycosidase F;
HexNAcase, N-acetylhexosaminidase.
(a) N-linked glycopeptides
Exoglycosidase digests (volumes in l)
Enzyme
Source
Linkage
specificity*
(unit/ml)
pH
optimum
PNGase
F
digest
1
2
3
4
5
6
7
8
PNGase F
Flavobacterium
meningosepticum
20
7.0‚8.0
0.4
Sialidase
Arthrobacter
ureafaciens
2-6 > 3,8
1
5.0‚5.5
0.1
0.1
0.1
0.1
0.1
0.1
0.1
0.1
Galactosidase
Streptococcus
pneumoniae
1-4
0.2
5.5‚6.5
0.1
0.1
0.1
0.1
0.1
0.1
0.1
GlcNAcase
Streptococcus
pneumoniae
1-2
0.004
5.0‚5.5
0.1
0.1
GlcNAcase
Chicken liver
1-2,3,4,6
1
4.0‚4.5
0.1
0.1
0.1
0.1
Mannosidase
Jack bean in 10
mM ZnCl2
1-3
0.1
4.0‚4.5
0.1
Mannosidase
Jack bean in 10
mM ZnCl2
1-2,3,6
10
4.0‚4.5
0.1
0.1
0.1
Mannosidase
Helix pomatia
1-4
1
4.0‚4.5
0.1
HPLC
fraction/sample
0.5
0.5
0.25
0.25
0.25
0.25
0.25
0.25
0.25
Buffer Að
0.1
Buffer B›
0.1
0.1
0.1
0.1
0.1
0.1
0.1
0.1
10% Methanol
0.3
0.45
0.35
0.35
0.15
0.25
0.25
0.15
(b) GPI anchor peptide conjugates
Reactions (volumes in l)
Enzyme
Source
Linkage
specificity*
Concn.
(unit/ml)
pH
optimum
1
2
3
4
5
Sialidase
Arthrobacter
ureafaciens
2-6 > 3,8
1
5.0‚5.5
0.1
Galactosidase
Bovine testes
1-3,4 > 6
1
4.0‚5.0
0.2
HexNAcase
Jack bean
1-2,3,4,6
3.4
6.0‚8.5
0.2
Mannosidase
Jack bean in 10
mM ZnCl2
1-2,3,6
10
4.0‚4.5
0.1
48%
Hydrofluoric
acid
0.5
HPLC fraction
0.25
0.25
0.25
0.25
0.25
Buffer Bð
0.1
0.1
0.1
0.1
10% Methanol
0.55
0.45
0.45
0.55
0.25
* Specificity data were taken from Oxford Glycosystems Ltd. data sheets and product
information.
ð Buffer A = 20 mM ammonium bicarbonate, pH 8.0, 50% methanol.
› Buffer B = 50 mM sodium citrate/phosphate, pH 5.0.
Prior to digestion with trypsin CD59U was carboxyamidomethylated as follows: CD59U (113
g in 200 l of one-tenth diluted PBS) was lyophilized without desalting and resuspended
in 25 l of 8 M urea, 100 mM ammonium bicarbonate (prepared fresh and deionized on a
mixed-bed resin). Dithiothreitol (1 l, 450 mM) was added and the mixture incubated at
50 ƒC for 15 min. Carboxyamidomethylation was performed using 10 l of iodoacetamide
(100 mM) and incubated at room temperature for 15 min. Water (64 l) was added to
dilute the urea prior to trypsin digestion. Modified sequence-grade trypsin (Promega Corp.,
Madison, WI, U.S.A.) was added (5 l of a 2 mg/ml solution) and incubated at 28 ƒC for 24 h.
The final protein to protease ratio was 11.3:1 (w:w).
Prior to digestion with endoproteinase Lys-C (Wako Chemicals, CA, U.S.A.) CD59U was
vinylpyridylated as follows: 10 g of the protein was incubated with the alkylation buffer (6
M guanidium hydrochloride, 0.5 M Tris/HCl, 2 mM EDTA) and reduced with 5 l of
dithiothreitol at 22 ƒC for 10 min. After incubation 1 l of 4-vinylpyridine (Aldrich
Chemicals, Steinheim, Germany) was added and the mixture was incubated at 22 ƒC for 10
min. The reduced and alkylated protein was desalted by HPLC and subjected to digestion (2x2 h
at 37 ƒC) with endoproteinase Lys-C (1.5%).
Digested CD59U (8.8 nmol in 200 l) was applied to a Brownlee RP-300 column (C8; 100
mmx2.1 mm). Peptides were separated by RP-HPLC on an Applied Biosystems 130A System
with a linear gradient of acetonitrile [solvent A = 0.1% trifluoroacetic acid (TFA); solvent B
= 0.085% TFA in acetonitrile; flow rate, 100 l/min] over a 100 min cycle. Peptide peaks
were detected at a wavelength of 220 nm, and collected, lyophilized and stored at -20 ƒC until
required for analysis.
Glycosidase digestion of T2-glycopeptide and T9-GPI-anchor peptides
Digestions were performed in a total volume of 1 l (see Tables 1a and 1b for digestions of
the N-linked oligosaccharide and the GPI anchor, respectively) [38]. After the final addition
to each digest, the tubes were vortexed briefly and centrifuged at 18000 g for 1 min. The
incubations were performed at room temperature and aliquots removed after 18‚24 h. Each
aliquot was used immediately to prepare sample slides for MALDI-MS.
MALDI-MS analysis
The mass spectrometer used in this work was a Finnigan LASERMAT 2000 (Finnigan MAT Ltd.,
Hemel Hempstead, U.K.) using a pulsed nitrogen laser (337 nm), 0.5 m drift tube and
multi-channel plate detector. The detector signal was digitized at a sampling rate of 100 MHz
and transferred to a personal computer for storage and report generation.
Native CD59U and sialidase-treated CD59U were analysed in 0.5 l of
-cyano-4-hydroxycinnamic acid (ACH) at 10 mg/ml in acetonitrile/water (70:30, v/v),
in the absence of an internal calibrant. HPLC fractions were analysed in the presence of an
internal calibrant (substance P, [M+H]+ = Mr 1348.7), T2 N-glycosylation exoglycosidase
digests in the presence of PNGase F-treated (deglycosylated) T2 ([M+H]+ = Mr 1804) and
T9-GPI digests in the presence of CD59 T5 peptide ([M+H]+ = Mr 1540.6). ACH (0.5 l
at 10 mg/ml in acetonitrile/water (70:30, v/v) was used as a matrix throughout.
Cell binding and haemolysis assays
Binding of soluble and membrane forms of CD59 to GPE was analysed by mixing serial
dilutions of radiolabelled proteins with GPE (2x107 cells/ml) and incubating them for 60
min at 37 ƒC. Proteins were diluted in PBS in the presence (CD59E) or absence (CD59U) of
0.001% Lubrol PX detergent. Radiolabelled sheep IgG was used as a control for non-specific
binding. After washing with PBS, the cells were counted for bound radioactivity.
In the reactive haemolysis assay 107 GPE were mixed with C56 euglobulin and increasing
concentrations of CD59E or CD59U. Cells were incubated with EDTA plasma (25%) for 30
min at 37 ƒC and haemolysis was quantified as release of haemoglobin (A412). All lysis
experiments were performed in duplicate.
Sucrose density-gradient ultracentrifugation
Binding of CD59 to TCCs was investigated by incubating (60 min, 37 ƒC) 125I-CD59U (50
ng) with 60 l of C7DS, C8DS, C9DS or NHS in the presence or absence of 4% (w/v) inulin
[39]. Bound radioactivity was separated from unbound ligand by sucrose density
ultracentrifugation (10‚50% gradients in PBS, centrifugation for 16 h at 40000 g) and
binding was calculated as percentage of label incorporated in the high-molecular-mass
complexes.
RESULTS
Comparison of binding properties of soluble and lipid-tailed CD59
Earlier studies have demonstrated the presence of both soluble and lipid-tailed forms of CD59
in various human body fluids including sweat, tears, saliva, breast milk, seminal plasma,
amniotic fluid and blood plasma [18,19,22,22a,23]. In the present study soluble urinary
CD59 (CD59U) was chosen for closer structural and functional studies. CD59 was isolated
from human urine by YTH53.1 monoclonal antibody affinity chromatography. N-terminal
sequencing of urinary CD59 yielded the sequence LQCYNCPNPTADCK, which is identical to the
N-terminal sequence derived from the cDNA sequence of CD59 [7]. When freshly isolated
urinary CD59 was radiolabelled and subjected to a Triton X-114 phase separation procedure,
approx. 2.4% of the radiolabel was observed to partition with the detergent phase. In a
separate study [24] employing Triton X-114 phase separation and a specific ELISA to quantify
CD59 we observed that 90.1% and 9.1% of CD59 in a urine pool from healthy donors
partitioned into the aqueous and detergent phases, respectively.
The glycolipid-anchored CD59E has previously been shown to be capable of inserting into
erythrocyte membranes [11]. In contrast with 125I-CD59E, soluble 125I-CD59U did not
enter the cell membrane when mixed with human or guinea pig erythrocytes (Figure 1a). On a
molar basis the binding of 125I-CD59U to red blood cells did not exceed that of the soluble
radiolabelled control protein (sheep IgG).
Figure 1 Functional comparison of soluble CD59U and lipid-tailed CD59E
(a) Binding of CD59U and CD59E to GPE membranes. Various amounts of radiolabelled CD59E
(2x107 c.p.m./g, in PBS with 0.001% Lubrol PX), CD59U (1.3x107 c.p.m./g) or
sheep IgG (1.2x107 c.p.m./g; control) were mixed with 2x107 GPE in PBS and incubated
for 60 min at 37 ƒC. Bound radioactivity was determined after centrifugation of cells through
20% sucrose. (b) Inhibition of complement lysis by soluble and membrane forms of CD59.
107 GPE were mixed with C5b6 and increasing concentrations of CD59E or CD59U. Lysis was
developed with EDTA plasma (25%) and quantified as release of haemoglobin (A412). CD59U
inhibits lysis of GPE at an approx. 200-fold lower efficiency than CD59E.
Functional activity of soluble CD59: inhibition of complement lysis and
binding to TCCs
The MAC-inhibiting activities of CD59E and CD59U were compared in a reactive lysis system.
As shown in Figure 1(b), CD59U inhibited C56+C7+C8+C9-induced haemolysis of GPE.
Approx. 1 M of CD59U versus 5 nM of CD59E was required for 50% inhibition of
haemolysis under the conditions used.
CD59E has previously been shown to interact with the terminal C5b‚8 and C5b‚9 complexes
[11]. To see if soluble CD59U had equivalent activities, fluid-phase TCCs SC5b67, SC5b‚8
and SC5b‚9 were prepared by activating sera depleted of C8, C9 or NHS with 4% inulin (60
min, 37 ƒC). 125I-CD59U was added to the sera prior to activation. In line with results
obtained earlier [39] approx. 42% and 19% of offered 125I-CD59U bound to the SC5b‚8 and
SC5b‚9 complexes, respectively. No binding to the C5b6 or SC5b67 complexes or to any
high-molecular-mass complexes in zymosan-activated rabbit serum occurred.
Molecular heterogeneity of urinary CD59
When affinity-purified urinary CD59 was applied on a Mono Q strong anion-exchange
chromatography column it was found to resolve into nine distinct peaks, which in SDS/PAGE
showed heterogeneity in the apparent molecular mass (Figure 2). When the same sample of
urinary CD59 was treated with A. ureafaciens sialidase prior to Mono Q chromatography the
molecular heterogeneity was observed to diminish and a novel peak in the Mono Q
breakthrough fraction appeared (Figure 3a). The apparent molecular mass of
sialidase-treated urinary CD59 in SDS/PAGE was only slightly smaller than that of untreated
CD59U (Figure 4b). Examination of the individual radiolabelled fractions from Mono Q
separation, including fraction N1 after sialidase treatment, showed that each fraction had a
quantitatively similar capacity to bind to the SC5b‚8 complexes generated in inulin-activated
C9DS (Table 2 and Figure 3c).
Figure 2 Heterogeneity of urinary CD59 revealed by anion-exchange
chromatography
(a) Affinity-purified urinary CD59 was applied on a Mono Q column equilibrated with 20 mM
Tris/HCl, pH 7.2, and eluted with a linear gradient of NaCl. Elution was monitored by A218
(AU, absorbance units). (b) SDS/PAGE analysis (silver staining) shows fractions 1, 2, 3, 5,
6 and 9 run under reducing conditions. t, time.
Figure 3 The effect of sialidase on urinary CD59
(a) Mono Q profile of urinary CD59 after treatment with A. ureafaciens sialidase. Note
appearance of fraction N1 and decrease in the other fractions. (b) SDS/PAGE analysis (silver
staining) compares fractions N1‚N3 with fractions 1 and 9 (from Figure 2). No protein in
fraction N3 becomes visualized in the silver-stained SDS/PAGE gel. Because of its ability to
incorporate into cell membranes and TCC the material in fraction N3 may represent a trace of
a phospholipid-tailed form of CD59 with a disproportionately high absorbance at 218 nm. (c)
Comparison between CD59U (fraction 1 from Figure 2) and sialidase-treated CD59U
(fraction N1) for their ability to bind to SC5b‚8. To generate fluid-phase SC5b‚8
C9-depleted human serum was activated (60 min, 37 ƒC) with 4% inulin in the presence of
125I-CD59U (50 ng) and subjected to sucrose density-gradient analysis. IgG (7 S) and IgM
(19 S) were used as migration markers. Top of the gradient is to the left. t, time.
Figure 4 MALDI-MS spectra of native CD59U (a), sialidase- (b) and PNGase
F- (c) treated CD59U
(a) Approx. 0.4 pmol of soluble CD59U was applied on a Lasermat 2000 mass spectrometer.
The native spectrum gives two major and three minor peaks with indicated average mass
values. The broad mass peaks are due to molecular mass heterogeneity of the sample. The
identity of the two major peaks is based on mass calculations that consider structures of the
N-linked oligosaccharide (CHO) and C-terminal GPI‚anchor (see Figures 9 and 11). (b)
MALDI-MS analysis of CD59U after treatment with A. ureafaciens sialidase. The broad peaks
of A are resolved into distinct molecular mass species. A small reduction in the apparent
molecular mass of CD59U after sialidase treatment can be seen in a silver-stained SDS/PAGE
gel (inset). (c) On treatment with PNGase F the dominant peak of CD59U still exhibits
heterogeneity due to the GPI anchor. [M+H]+ values indicate the calculated expected mass
values of protonated products. The masses of the major forms of the GPI anchor (C, C¥, D) are
from Figure 11. A Coomassie Blue-stained SDS/PAGE gel in the inset shows the effect of
PNGase F on CD59U in the absence or presence of a reducing agent.
Table 2 Incorporation of Mono Q fractions of 125I-labelled urinary CD59 into
SC5b‚8 complexes
Binding is expressed as a percentage of specific incorporation of 125I-labelled urinary CD59
fractions into the terminal SC5b‚8 complex during incubation of C9DS with 4% inulin for 60
min at 37 ƒC.
Mono Q fraction number
Incorporation (%)
1
20.1
2
21.4
3
25.9
4
25.7
5
31.4
6
25.9
7
21.9
8
22.2
9
31.3
In line with earlier results with CD59E [6] the treatment of CD59U with PNGase F reduced its
apparent molecular mass by approx. 6 kDa in SDS/PAGE (Figure 4c). Removal of the
N-linked carbohydrate was successful only in the presence of a reducing agent. This prevented
the re-analysis of the molecule by RP-HPLC (but not MALDI-MS). On the other hand, PNGase
F was not able to cleave the non-reduced molecule as shown in the inset of Figure 4(c).
Therefore only the isolated and repurified tryptic peptide (T2) has been cleaved with PNGase
F. Examination of the effect of N-deglycosylation on the functional activity of CD59U was not
possible in this approach, since reduction of disulphides in CD59U rendered it incapable of
binding to SC5b‚8.
Peptide mapping and mass spectrometric analysis of CD59U
Purified soluble CD59U was reduced, alkylated and digested with endoproteinase Lys-C and
trypsin into peptides that were separated by RP-HPLC (Figure 5). The isolated peptides were
subjected to automated Edman degradation and amino acid sequence analysis. All peptide
sequences obtained matched the reported amino acid sequence of CD59 [7]. Only one amino
acid, Asn-18, could not be identified due to the glycosylation of this residue [33]. The
C-terminal amino acid was confirmed as Asn-77 by automated N-terminal sequence analysis
of the isolated C-terminal peptide T9.
Figure 5 RP-HPLC profile of peptides from CD59U after digestion with
endoproteinase Lys-C
Approx. 10 g of CD59U was digested with endoproteinase Lys-C (1.5%) for 2x2 h at 37 ƒC
and the resulting peptides were separated by RP-HPLC. Amino acid sequences of the selected
peptides were determined by automated Edman degradation.
MALDI-MS analysis of native soluble CD59U gave two major broad mass signals with average
Mr values of 12444 and 11164 (Figure 4a). The spectrum showed additional
lower-molecular-mass peaks which may be due to [M+2H]+ ions. After treatment of CD59
with Arthrobacter ureafaciens sialidase to remove terminal sialic acid residues more clearly
defined peaks for CD59U were observed (Figure 4b). The broad peaks were thus principally
due to variable sialylation. The multiplicity of the peaks, however, indicated significant
remaining heterogeneity in the CD59 preparation.
After treatment with PNGase F to remove the Asn-18-linked oligosaccharide, CD59U still
exhibited heterogeneity in its MALDI-MS spectrum (Figure 4c). As shown below, this was
apparently due to heterogeneity in the GPI anchor, of which two major variants (C+C¥, D)
linked to the CD59 polypeptide could be deduced from the mass values.
MALDI-MS analysis of N-linked oligosaccharides of CD59U
RP-HPLC fractions of the separated CD59U tryptic peptides were analysed by MALDI-MS.
Three fractions exhibited complex spectra associated with glycosylation heterogeneity.
Detailed inspection of the spectra revealed mass differences between adjacent signals
corresponding to monosaccharide residues, e.g. Mr 162 corresponding to a hexose (glucose,
mannose, or galactose), Mr 146 to fucose, Mr 203 to an N-acetylhexosamine
(N-acetylglucosamine or N-acetylgalactosamine), or Mr 291 to N-acetylneuraminic acid
(Figures 6A and 6B).
Figure 6 Mass analysis of RP-HPLC fractions containing CD59U tryptic
fragments
(A) Native T2 glycopeptide separated by RP-HPLC. Further analysis of the heterogenous T2
glycopeptide is shown in Figure 7. (B) C-terminal T9-GPI-anchor peptide. Interpretation of
signals A‚F is illustrated in Figure 11. The 1540.6 mass peak corresponds to the T5 peptide
that served as an internal standard for the mass analysis.
When treated with PNGase F (Table 1a), and re-analysed by MALDI-MS, the complex spectra
of two of the fractions (one shown in Figure 6a) were replaced by a single mass of MH+ =
1803.7 (Figure 7a). This signal corresponds to the carboxyamidomethylated T2 peptide (with
Asn converted into Asp by PNGase F) containing the N-linked recognition sequence
-TAVN18C¥SSD (C¥ = carboxyamidomethylated cysteine). The third fraction (Figure 6B) was
not affected by PNGase F treatment.
Figure 7 Mass analysis of the T2 glycopeptide after digestion with
glycosidases (Table 1a)
(a) PNGase F digestion, (b) digest 1, Arthrobacter ureafaciens sialidase, (c) digest 2, A.
ureafaciens sialidase and Streptococcus pneumoniae galactosidase, (d) digest 3, A.
ureafaciens sialidase, Strept. pneumoniae galactosidase and Strept. pneumoniae
N-acetylglucosaminidase, (e) digest 4, A. ureafaciens sialidase, Strept. pneumoniae
galactosidase and chicken liver N-acetylglucosaminidase, (f) digest 5, A. ureafaciens
sialidase, Strept. pneumoniae galactosidase, chicken liver N-acetylglucosaminidase and jack
bean -mannosidase, (g) digest 6, A. ureafaciens sialidase, Strept. pneumoniae
galactosidase, Strept. pneumoniae N-acetylglucosaminidase and jack bean -mannosidase,
(h) digest 8, A. ureafaciens sialidase, Strept. pneumoniae galactosidase, chicken liver
N-acetylglucosaminidase, jack bean -mannosidase and Helix pomatia -mannosidase.
The two fractions containing the N-linked glycopeptide were combined and used for sequence
analysis of the oligosaccharide structures as described by Sutton et al. [38]. The calculated
mass value of the free T2 peptide (Mr 1803.0) was subtracted from each of the mass values
in the native glycopeptide spectrum to give the residue masses of the oligosaccharide mass.
Using the residue masses of monosaccharides that occur naturally in mammalian glycoproteins
it was possible to assign a probable composition to each glycoform and tentatively identify it
as belonging to a particular class of oligosaccharides. The T2 oligosaccharide compositions
indicated that fucosylated bi- and tri-antennary complexes predominated, with a smaller
contribution from non-fucosylated bi- and tri-antennary complexes. To confirm these
assignments and obtain more sequence information, the T2 glycopeptide was treated with
specific exoglycosidases and subjected to MALDI-MS [38].
The T2 glycopeptide fraction was split into eight aliquots and digested using the enzyme
mixtures listed in Table 1(a). The digests were run simultaneously and then analysed by
MALDI-MS (Figures 7b‚7h). The MALDI-MS results from each digest have been interpreted
in terms of progressive digestion of the oligosaccharide structures and represented as a flow
chart (Figure 8). Because of the sugar and linkage specificity of the exoglycosidases the shift
in masses with inclusion of each new enzyme enabled the sequencing of the oligosaccharides
attached to the T2 peptide. Previous experiments [38] had shown that after sialic acid had
been removed with Arthrobacter ureafaciens sialidase, the mass signal intensities of each
glycoform attached to the peptide were proportional to site occupancy for each glycoform.
Quantification was based on the intensity of each signal as a fraction of the integral intensity of
all signals assigned to glycopeptides after sialidase digestion.
Figure 8 Composite glycoforms identified in each exoglycosidase digest of
CD59U tryptic fragment
Each structure was based on the mass signal shifts identified by MALDI-MS before and after
digestion with a specific exoglycosidase in digests 1 to 8 of Table 1(a).
The data collected from treatment of the T2 glycopeptide with specific exoglycosidases,
MALDI-MS analysis and quantification from the sialidase-treated spectra enabled the
structure of the glycoforms to be determined (Figure 9).
Figure 9 Structures of oligosaccharides linked to Asn-18 of soluble urinary
CD59
The linkage information was determined by a combination of MALDI-MS analysis with
sequential treatment of the desialylated T2-CHO peptide with linkage-specific exoglycosidases
(Table 1a; Figures 7 and 8). The percentages indicate the approximate proportions of each
structure determined from peak heights from the spectra of sialidase-treated T2-CHO
glycopeptide (Figure 7b).
Characterization of the C-terminal GPI-anchor moiety of CD59U
A single HPLC fraction gave a complex spectrum in the Mr range 2400‚3400 (Figure 6B).
When treated with 48% hydrofluoric acid (to cleave labile phosphate bonds that link the GPI
anchor to the peptide), the putative T9-GPI-anchor signals (A to F) disappeared and were
replaced by a signal of Mr 1440.3 (Figure 10A) which corresponded to the T9 peptide with a
single ethanolamine group attached.
Figure 10 Mass analysis of the T9-GPI anchor
T9-GPI peptide was treated with (A) 48% hydrofluoric acid or glycosidases (Table 1b), (B)
digest 4, jack bean -mannosidase, (C) digest 2, bovine testis -galactosidase, or (D)
digest 1, A. ureafaciens sialidase.
The most prominent signal, C (Mr 2817.4), of the HPLC fraction (Figure 6B) had a mass
consistent with T9 peptide conjugated to a Thy-1-type GPI anchor, but lacking the
phosphatidyl group. The HPLC fraction was split into several aliquots and treated with a range
of exoglycosidases (Table 1b). Incubation with S. pneumoniae N-acetylglucosaminidase,
chicken liver N-acetylglucosamidase or coffee bean -galactosidase did not result in any
mass changes of any of the signals (results not shown). Jack bean -mannosidase, however,
resulted in Mr changes of 162 for signals C, D and F, indicating the presence of an exposed
mannose (Figure 10B). Bovine testis -galactosidase reduced the signal intensity of C and
resulted in the complete loss of D (Figure 10C). With both these enzymes there was a
concomitant increase in signal B intensity.
The information from the digests was used to deduce possible structures of the GPI-anchor
moiety (Figure 11). We have assumed that the Man-Man-Man
(ethanolamine-phosphate)-glucosamine-inositol core is the same as that observed in the
well-characterized Thy-1 GPI anchor [31] and that the position of the exposed sugar, cleaved
by exoglycosidases, is analogous to that observed in the scrapie prion protein PrpSc [41].
Signal C comprises two possible structures containing either an exposed mannose (structure
C) or a galactose (structure C¥) residue. Signal B constitutes structure C without the exposed
mannose residue and signal A lacks the mannose and a putative N-acetylhexosamine residue.
In Thy-1, the latter residue is an exposed N-acetylgalactosamine, but in CD59 the respective
residue was not affected by jack bean N-acetylhexosaminidase (results not shown). It is
possible that jack bean hexosaminidase does not recognize N-acetylhexosamine in the context
of the GPI structure or that neither N-acetylglucosamine nor N-acetylgalactosamine is
present. Signal D was modified by both -mannosidase and -galactosidase, suggesting the
presence of exposed residues on the same molecule (structure D). A. ureafaciens sialidase
digestion resulted in loss of E and F signals, indicating the presence of exposed sialic acid
residues (Figure 10D). Signal F was also lost on -mannosidase digestion, indicating the
presence of an exposed mannose residue on structure F.
Figure 11 Putative GPI-anchor structures of CD59U deduced from
exoglycosidase treatment of the C-terminal peptide T9-GPI and MALDI-MS
Aliquots of the T9-GPI peptide were treated with various exoglycosidases as shown in Table
1(b) and subjected to MALDI-MS. The [M+H]+ values indicate mass peaks observed after
each treatment. Treatment of all peptides with 48% hydrofluoric acid [31] generated an Mr
peak of 1440 corresponding to the T9 peptide with an attached ethanolamine (Figure 10a).
Structure of the GPI-core oligosaccharide and location of the second ethanolamine-phosphate
group are based on analogy with previously published reports [31,42,43,45]. The suggested
variant structures were deduced from analysis of mass shifts after treatment with the
linkage-specific exoglycosidases. The percentages indicate the approximate proportions of
each structure (A‚F) present in the original T9-GPI peptide (Figure 6B). Structures C and C¥
were both present but their relative quantitative ratio could not be determined by MALDI-MS.
DISCUSSION
In addition to cell membranes the complement MAC-inhibitor CD59 (protectin) is present in
human body fluids and secretions. As indicated in the present study a high proportion of CD59
in human urine represents a soluble, phospholipid-free isoform (CD59U) that binds to the
TCCs and has limited complement haemolysis-inhibiting activity. CD59U was found to have an
average Mr of 12444, of which the main N-linked oligosaccharide accounts for 19% and
the GPI anchor for 11% depending on the degree of their structural modifications.
The 77-amino-acid-long CD59 was found to express considerable heterogeneity both in its
oligosaccharide side chain and in the C-terminal GPI anchor. Affinity-purified urinary CD59
resolved into at least nine different peaks in Mono Q chromatography (Figure 2a). As Mono Q
is a strong anion-exchange column the various forms of urinary CD59 separated must have
different charged groups exposed on their surfaces under the buffer conditions used. Mono Q
fractionation of the sialidase-treated urinary CD59 yielded a major breakthrough peak,
indicating that the charge heterogeneity was due to differential sialylation of CD59. A similar
result was obtained using MALDI-MS, where the diffuse major mass peak of CD59U resolved
into distinct peaks after sialidase treatment (Figure 4b).
Variable sialylation in the Asn-18 oligosaccharide of CD59 was directly demonstrated by
MALDI-MS analysis of the T2-CHO peptide. Treatment of T2 with sialidase significantly
simplified the spectrum and allowed assignment of core structures of the oligosaccharide
(Figure 7). Heterogeneity in the oligosaccharide backbone was due to the presence of either
bi- or tri-antennary complexes. Treatment of T2 with PNGase F abolished the heterogeneity
by removing the oligosaccharide side chain from the peptide.
Our results differ from those of Nakano et al. [32] in that we detected a significant proportion
(22%) of triantennary structures and a broader distribution of various oligosaccharide
structures in CD59. In accordance with this study we observed that the most common
oligosaccharide type is a biantennary complex with fucose linked to the proximal
N-acetylglucosamine residue.
The sialylation heterogeneity of urinary CD59 was not found to significantly affect its binding
activity towards the terminal SC5b‚8 complex (Table 2). Thus, in accordance with
observations made by Ninomiya et al. [33], it is unlikely that the degree of sialylation would
affect the functional activity of CD59. On the other hand, the removal or chemical modification
of the backbone of the N-linked sugar was suggested to lead to a substantial reduction in the
complement lysis inhibitory activity, but not in the TCC binding activity of erythrocyte CD59
[33]. The mechanism of the possible contribution of the oligosaccharide to the MAC inhibitory
function of CD59 remains to be established.
The presence of the GPI anchor is a characteristic feature of the complement regulators decay
accelerating factor (DAF) and CD59, as well as of many non-complement molecules. Mass
analysis of the GPI anchor on the C-terminal peptide of CD59U yielded a main signal of Mr
2817.4, which is close to a theoretical protonated mass, Mr 2818.0, of structures C and C¥
in Figure 11. The principal putative GPI structure of CD59 (C in Figure 11) is similar to
that of mammalian Thy-1 GPI anchor [31] without an inositol-associated phosphatidate
group. The apparent absence of the diradylglycerolphosphate group from CD59U could be due
to enzymic cleavage of the GPI anchor in vivo. Phospholipase D in human plasma [40] is an
enzyme capable of cleaving GPI anchors between inositol and phosphate. Solubilization of
GPI-anchored molecules could thus provide a physiological function for plasma phospholipase
D, for which the identity of natural substrates is still unknown. In order not to affect
GPI-anchored molecules on cell membranes the detachment of GPI proteins from their lipid
tails should occur only after the molecules have become shed from cell membranes. How this
precisely occurs and whether any regulatory mechanisms are involved in this process remain
to be investigated.
The mass signals for the T9-GPI-anchor peptide also showed significant heterogeneity in the
Mr range 2400‚3400 (Figure 6B). The different signals are due to structural modifications
of the anchor structures. The two major forms (C and C¥) with the same mass value differ in
having either an exposed mannose (C) or galactose residue. Further heterogeneity is due to the
absence (B) or presence (D) of both of these residues, an absent N-acetylhexosamine (A) or
the presence of a sialic acid residue (E, F). In the present study it was not possible to
determine the exact locations of the sialic acid residue or of the
N-acetylhexosamine-galactose-sialic acid arm. The presence of a trisaccharide arm on GPI
anchors is a relatively novel observation and has previously been described only once before,
on a scrapie prion protein [41]. In this study it was determined that the trisaccharide was
linked to the third mannose from the peptide [41]. Therefore, an analogous putative position
for the trisaccharide is shown in Figure 11.
Similarities in the GPI anchors of Thy-1 [31], human erythrocyte acetylcholine esterase
[42], placental alkaline phosphatase [43] and CD59 [32] suggest a consensus structure for
mammalian GPI anchors, which with slight modifications extends to that of single cell
eukaryotes and protozoal parasites [44,45]. With the currently available glycosidases it was
not possible to determine the sugar linkages of the core GPI structure of CD59. The mass value
data obtained in the present study are consistent with the consensus structure but do not prove
it. However, when combined with the biochemical data available [32] the GPI glycan core
structure shown in Figure 11 appears highly likely. The main uncertainty concerns the
second phosphoethanolamine, whose location remains to be confirmed.
At present the origin of urinary CD59 is unknown. It could originate from the epithelial cells
of kidney or from plasma. Using indirect immunofluorescence CD59 has been detected in the
epithelial cells of distal tubules, collecting ducts and glomeruli of kidneys [17,46]. Both
CD59 and DAF may become shed from epithelial cells. Shedding could occur in the form of
small membrane vesicles, with the phospholipid tail left intact, or after cleavage of the
anchor by the phospholipase C or phospholipase D enzymes. Recent studies have verified that
CD59 is present in small membrane microparticles in the urines of normal individuals as
well as of patients with membranous glomerulonephritis [24]. As indicated above, after CD59
has become shed from cell surfaces it may lose the phospholipid portion of the anchor and
transform into a soluble hydrophilic form. Alternatively, for a portion of the synthesized
CD59 molecules the addition of the lipid tail may have failed and resulted in the secretion of
non-lipid-tailed forms. Considering the fact that CD59 is present on all blood cells and on
vascular endothelial cells only a little CD59 is present in normal human plasma [23]. As a
small and hydrophilic molecule soluble CD59 could become filtered through kidney glomeruli
and concentrate into urine, whereas the lipid-tailed forms would remain in the vasculature
and reincorporate into cell membranes or plasma lipoprotein particles [23,24]. The
heterogeneity of the oligosaccharide and GPI-anchor structures suggests that soluble urinary
CD59 may originate from multiple sources.
The haemolysis-inhibiting activity of soluble CD59U was notably weaker than that of the
membrane form (Figure 1b). The lower activity of the soluble form is consistent with its
weaker binding to the cell membrane (Figure 1a), and suggests that quantification of the
difference is dependent on conditions used in the assay. Despite showing low
haemolysis-inhibiting activity soluble CD59 bound to the TCCs in equivalent quantities to
erythrocyte CD59 (Table 2) [11,39]. In recent studies we have demonstrated that CD59U is
also capable of binding to a nascent form of membrane-associated MAC [39]. These results
indicate that the specific protein‚protein interaction of soluble CD59 with the TCCs does not
require the glycolipid anchor. The lipid tail, however, is important for the full cell lysis
inhibitory activity, apparently because it facilitates the incorporation of CD59 into the outer
leaflet of the lipid bilayer and the intrinsic inhibition of MAC on the cell membrane.
Comparison of the amino-acid sequences of human [7], primate [47] and rat [48] CD59 with
mouse Ly-6 antigens [29], snake venom neurotoxins [27], a putative CD59 homologue in
Herpes virus saimiri [49] and uPAR [30] suggests that a common folding pattern based on
conserved cysteine residues exists within this family of proteins [7,10,49‚52]. This
proposal has been substantiated by the recent NMR spectroscopic determination of the
three-dimensional structure of the polypeptide backbone of human CD59 [25,26] which
resembles that of neurotoxins determined earlier by X-ray crystallography [27]. Although
the actual positions of the side chains relative to the protein backbone are not known the
N-linked oligosaccharide is attached to the external N-terminal portion of CD59 where it may
have an important role in stabilizing the overall structure of the molecule. At the other end,
the attachment of CD59 to cell membranes via a GPI anchor is likely to provide the molecule
with flexibility that is needed in its interaction with the TCCs.
This study was supported by the Academy of Finland, the Sigrid Juselius Foundation and the
University of Helsinki.
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Received 19 January 1996; accepted 22 February 1996
The Biochemical Society, London © 1996
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