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In addition, de novo biosynthesis of glycosphingolipids was shown by metabolic incorporation of [14C]palmitic acid and [14C]glucose in the three intraerythrocytic stages of the parasite.

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Glycosphingolipids in Plasmodium falciparum

Presence of an active glucosylceramide synthase

Alicia S Couto1, Carolina Caffaro1, M Laura Uhrig1, Emilia Kimura2, Valnice J Peres2, Emilio F Merino2, Alejandro M Katzin2, Masae Nishioka3, Hiroshi Nonami3and Rosa Erra-Balsells1

1

CIHIDECAR, Departamento de Quı´mica Orga´nica, Facultad de Ciencias Exactas y Naturales, Universidad de Buenos Aires, Argentina;2Departamento de Parasitologia, Instituto de Cieˆncias Biome´dicas, Universidade de Sa˜o Paulo, Brazil;3College of Agriculture, Ehime University, Matsuyama, Japan

Malaria remains a major health problem especially in

trop-ical and subtroptrop-ical regions of the world, and therefore

developing new antimalarial drugs constitutes an urgent

challenge Lipid metabolism has been attracting a lot of

attention as an application for malarial chemotherapeutic

purposes in recent years However, little is known about

glycosphingolipid biosynthesis in Plasmodium falciparum

In this report we describe for the first time the presence of

an active glucosylceramide synthase in the intraerythrocytic

stages of the parasite Two different experiments, using

UDP-[14C]glucose as donor with ceramides as acceptors, or

UDP-glucose as donor and fluorescent ceramides as

accep-tors, were performed In both cases, we found that the

parasitic enzyme was able to glycosylate only

dihydrocera-mide The enzyme activity could be inhibited in vitro with

low concentrations of D,L

-threo-phenyl-2-palmitoylamino-3-morpholino-1-propanol (PPMP) In addition, de novo

biosynthesis of glycosphingolipids was shown by metabolic incorporation of [14C]palmitic acid and [14C]glucose in the three intraerythrocytic stages of the parasite The structure

of the ceramide, monohexosylceramide, trihexosylceramide and tetrahexosylceramide fractions was analysed by UV-MALDI-TOF mass spectrometry When PPMP was added

to parasite cultures, a correlation between arrest of parasite growth and inhibition of glycosphingolipid biosynthesis was observed The particular substrate specificity of the malarial glucosylceramide synthase must be added to the already known unique and amazing features of P falciparum lipid metabolism; therefore this enzyme might represent a new attractive target for malarial chemotherapy

Keywords: dihydroceramide; glucosylceramide synthase; glycosphingolipids; malaria; Plasmodium falciparum

Malaria is the most serious and widespread parasitic disease

in humans Each year, approximately 300 million people

become infected and 2–3 million people die as a result In

addition there is considerable morbidity associated with this

disease [1]

The glycobiology of Plasmodium falciparum has been

causing an increasing amount of interest in recent years The

presence of N-linked glycoproteins in relation to schizogony

of the intraerythocytic stages [2] and

glycosylphosphatidyl-inositols as the major carbohydrate protein modification

have been described in the human malaria parasite [3–6] In addition, lipid metabolism has also been attracting a lot

of attention with respect to basic biology and applications for malarial chemoterapeutic purposes [7] However, little

is known about glycosphingolipids (GSLs), a group of ceramide-based lipids that in other systems regulate inter-actions of the cell with its environment and play a role in cell signalling [8,9] The first evidence of the presence of GSLs in

P falciparum was obtained by metabolic incorporation

of [3H]serine and [3H]glucosamine After labeling with the carbohydrate precursor, hydrophilic glycosphingolipids migrating slower than the penta-glycosylated ceramide standard were detected [10] More recently, the synthesis

of chloroplast galactolipids in apicomplexan parasites was reported [11]

Biosynthesis of complex GSLs in mammalian cells involves sequential glycosyltransferase reactions, starting with the formation of glucosylceramide (GlcCer), and it has been assumed that the various transferases used are functionally organized within the Golgi [12,13] It is known that the key step involves the transfer of glucose to ceramide from UDP-glucose, catalyzed by the action of a glucosyl-ceramide transferase [EC 2.4.1.80: glucosylglucosyl-ceramide syn-thase (GCS)] With regards to localization, as far as it is known, GlcCer is special because it is the only glyco-sphingolipid synthesized on the cytosolic leaflet in the early Golgi but it is used for the synthesis of higher sphingolipids

Correspondence to A S Couto, CIHIDECAR, Departamento de

Quı´mica Orga´nica, Pabello´n II, Facultad de Ciencias Exactas y

Nat-urales, Universidad de Buenos Aires, Buenos Aires, 1428, Argentina.

Fax/Tel.: + 54 11 4576 3346, E-mail: acouto@qo.fcen.uba.ar

Abbreviations: GSLs, glycosphingolipids; GlcCer, glucosylceramide;

GCS, glucosylceramide synthase; DHCer,

BODIPY-dihydroceramide; BODIPY-Cer, BODIPY-ceramide;

D , L -threo-PPMP, D , L

-threo-phenyl-2-palmitoylamino-3-morpholino-1-propanol, d18:0, 4-hydroxysphinganine; d20:0,

4-hydroxyicosa-sphinganine; C10:0, etc., decanoic acid, etc.; C10h:0, etc.,

hydroxydecanoic acid; C10-2h:0, dihydroxydecanoic acid;

C10-3h:0, trihydroxydecanoic acid, etc.

Enzyme: glucosylceramide synthase (EC 2.4.1.80).

(Received 4 December 2003, revised 26 February 2004,

accepted 6 April 2004)

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in the lumenal leaflet [14] Because glucosylceramide is a

pivotal precursor of numerous GSLs, this enzyme is

extremely important for understanding GSL function

In this report, we describe for the first time the presence

of an active glucosylceramide synthase in the

intraerythro-cytic stages of P falciparum Two different experiments,

using UDP-[14C]glucose as donor or fluorescent ceramides

as acceptors were performed In both cases, the enzyme

showed specificity for dihydroceramide as substrate

The enzyme activity could be inhibited in vitro with

D,L

-threo-phenyl-2-palmitoylamino-3-morpholino-1-pro-panol (PPMP) In addition, GSLs were shown by

meta-bolic incorporation of [14C]palmitic acid and [14C]glucose

in the three intraerythrocytic stages of the parasite

UV-MALDI-TOF mass spectrometry proved that four

fractions analysed corresponded to ceramides,

monohexo-sylceramides, trihexosylceramides and

tetrahexosylcera-mides, respectively When PPMP was added to parasite

cultures, a correlation between arrest of parasite growth

and inhibition of GSL biosynthesis was shown The

particular substrate specificity of the malarial GCS suggests

that this enzyme might represent a new attractive target for

malarial chemotherapy

Materials and methods

Materials

Lipid standards and BSA were purchased from Sigma

AlbuMax I was obtained from Gibco BRL Life

Tech-nologies (New York, NY, USA) All solvents were of

analytical or HPLC grade Percoll was purchased from

Pharmacia Chemicals (Uppsala, Sweden).D,L

-threo-phenyl-2-palmitoylamino-3-morpholino-1-propanol (PPMP) was

from Matreya (Pleasant Gap, PA, USA) and ceramide

glycanase from GlyKo, BODIPY-sphingolipids used were

from Molecular Probes Polyclonal antibodies against

human GCS were a kind gift of D L Marks and R E

Pagano, Mayo Clinic Foundation, Rochester, MN, USA

TLC was performed on silica gel 60 precoated plates

(Merck) using the following solvent systems: (a)

chloro-form/methanol/water (65 : 25 : 3, v/v/v); (b) chloroform/

methanol/0.25% KCl (80 : 30 : 2, v/v/v); (b), chloroform/

methanol/1M NH4OH (40 : 10 : 1, v/v/v); (d)

chloro-form/methanol/water (65 : 25 : 3, v/v/v); (e) chloroform/

methanol/water (80 : 20 : 2, v/v/v) In all cases, radioactive

samples were located by fluorography at )70 C using

EN3HANCE (NEN) and KodakX-OMAT AR films

Cultures ofP falciparum and metabolic labeling

An isolate (S20) of P falciparum obtained from a patient

living in Porto Velho (Rondoˆnia, Brazil) was used [15]

Parasite cultures of P falciparum were performed as

described [2]

[U-14C]Palmitic acid (Amersham 822 mCiÆmmol)1,

2.91 mCiÆmg)1) originally supplied in toluene was dried

under nitrogen, redissolved in ethanol, and coupled with

defatted BSA at a 1 : 1 (v/v) molar ratio The final labeling

medium contained 6.25 lCiÆmL)1of the radioactive

pre-cursor, 0.5% (w/v) Albumax and 0.05% (w/v) BSA

Parasites were labeled for 18 h

D-[U-14C]glucose (Amersham, 291 mCiÆmmol)1, 1,54 mCiÆmg)1) was incorporated at a concentration of 6.25 lCiÆmL)1 in RPMI 1640 medium without addition

of 11 mM of glucose Parasites (5.9% ring forms, 5.4% trophozoites, 3.7% schizonts) were labeled for 18 h

D-[U-14C]galactose (Amersham, 306 mCiÆmmol)1, 1,61 mCiÆmg)1) was incorporated at a concentration of 3.2 lCiÆmL)1in RPMI 1640 medium without addition of

11 mM of glucose Parasites (9.8% ring forms, 3.0% trophozoites, 1.6% schizonts) were labeled for 18 h The viability of the parasites was verified by microscopic evaluation of Giemsa stained smears Each stage was purified on a 40/70/80% (w/v) discontinuous Percoll gradient (15 000 g, 30 min, 25C) This procedure yielded

an upper band (40%) containing schizonts, another band with trophozoites (70–80% interface) and a pellet of ring forms [2]

A control containing a similar number of uninfected erythrocytes was incubated with the different radioactive precursors and further processed under the same conditions

In order to evaluate biosynthesis of proteins, synchronous

P falciparum ring-stage cultures, with a parasitemia of around 5%, untreated or treated with 5 lMPPMP for 48 h, were labeled with 25 lCiÆmL)1 of L-[35S]methionine (> 1000 CiÆmmol)1) (Amersham) in 10 lM methionine-deficient RPMI medium, at the beginning or after 24 h

of treatment Aliquots were collected at different times (0–48 h), precipitated with 12% (w/v) trichloroacetic acid, and radioactivity was measured with a Beckman 5000 b-counter

Treatment of parasites withD,L -threo-phenyl-2-palmitoylamino-3-morpholino-1-propanol Parasite cultures (6.4% ring forms, 2.4% trophozoites, 1.2% schizonts) were incubated with 5 lM D,L -threo-phenyl-2-palmitoylamino-3-morpholino-1-propanol (D,L -threo-PPMP) After 24 h of treatment, parasites were labeled with [14C]palmitic acid or [14C]glucose for 18 h in the presence of the drug After the labeling period, each stage was purified on a Percoll gradient as described above and freeze-dried prior to lipid extraction The effect

on parasite development was monitored by microscopy of Giemsa-stained blood smears in two independent experi-ments

In all cases, control cultures without the inhibitor and

a similar amount of uninfected erythrocytes were labeled under the same conditions

Isolation and purification of glycosphingolipids Each intraerythrocytic stage of P falciparum was extracted with chloroform/methanol 1 : 1 (3· 1 mL) Each extract was fractionated by anionic exchange chromatography on a DEAE-Sephadex A-25 (acetate form) column, which was eluted with chloroform/methanol/water (30 : 60 : 8, v/v/v)

to recover neutral GSLs and zwitterionic lipids Anionic lipids were bulkeluted with chloroform/methanol/0.8M

NaAcO (30 : 60 : 8, v/v/v) The unbound fraction was evaporated to dryness and treated with 0.1M NaOH in methanol (500 lL), at 37C for 3 h The mixture was neutralized with HCl 1 in the presence of 1 phosphate

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buffer pH 7 (50 lL) to avoid over-acidification After

evaporation, salts were removed by reverse-phase

chro-matography, using a Sep-PackC-18 cartridge (Worldwire

Monitoring, Horsham, PA, USA) Acidic lipids were also

concentrated through a Sep-Packcartridge Purification of

neutral glycosphingolipids was achieved by

chromatogra-phy on silicic acid The sample was dissolved in chloroform

and loaded into a column of Unisil (7· 50 mm) which was

eluted with chloroform (20 mL), chloroform/methanol

(98 : 2, v/v, 20 mL) and chloroform/methanol (1 : 3, v/v,

25 mL) [16]

In another experiment, total lipids from schizont forms

were extracted and purified as described above The purified

neutral GSL fraction was analysed in parallel with an

analogous fraction obtained from [U-14C]palmitic acid

labeled parasites by TLC in solvent B Spots corresponding

to the ceramide fraction (I), monohexosylceramide fraction

(II), trihexosylceramide fraction (IV) and

tetrahexosylcera-mide fraction (V) were extracted from the plate and

analysed by UV-MALDI-TOF MS

Acid methanolysis and methylation

The sample was hydrolysed for 18 h at 80C with 12M

HCl/MeOH/water (3 : 29 : 4, v/v/v) The hydrolysate was

dried and the acid eliminated by several evaporations with

addition of water Methylation of fatty acids was carried out

with BF3/MeOH in dry toluene under nitrogen at 80C for

90 min [17]

Ceramide glycanase digestion

Samples were dissolved in 250 mMphosphate buffer pH 5.0

(100 lL) containing 1% (w/v) sodium cholate Ceramide

glycanase (from Macrobdella decora) (0.3 mU) was added

and digestion was performed at 37C for 18 h Lipids

were extracted with chloroform/methanol (1 : 1, v/v) and

analysed by TLC

Glucosylceramide synthase assay

Parasite homogenates were prepared in 0.1M sodium

phosphate buffer (pH 7.4) containing 5 mM MgCl2,

25 mM KCl, 1 mMphenylmethanesulfonyl fluoride, 1 mM

N-a-tosyl-L-lisine chloromethyl ketone hydrochloride

(TLCK) and 10 lgÆmL)1 leupeptine by probe sonication

three times with 10 pulses while on ice Liposomal substrate

was performed with dipalmitoylphosphatidylcholine

and ceramide (palmitoyldihydrosphingosine or

palmitoyl-sphingosine) (10 : 1, v/v) containing 0.1 nmol of ceramide

The constituent lipids were dissolved in

chloroform/meth-anol (1 : 1, v/v), vortexed and dried under nitrogen Lipids

were dispersed in 0.1M sodium phosphate buffer pH 7.4

by sonication at 0C

The reaction mixture consisted of UDP-[14C]glucose

(1 lCi, 319 mCiÆmmol)1, Amersham), 2 mM b-NAD and

the liposomal substrate (600 nmol lipid phosphorous) in

0.1Msodium phosphate buffer (pH 7.4) The cell

homo-genate (50–100 lg protein per tube) was added making a

total volume of 15 lL The mixture was incubated at 37C

for 5 h with shaking Incubations were stopped by freezing

and the mixtures were cleaned by passage through C18

cartridges Lipids were eluted with chloroform/methanol (1 : 1, v/v) and further analysed by TLC When the inhibition test was performed, PPMP (5 lM) was added to the reaction mixture Spots were quantified using a phos-phoimager (Molecular Analyst, Bio-Rad) withMOLECULAR ANALYSTsoftware

In another experiment, BODIPY analogues of ceramides were used BODIPY-dihydroceramide (BODIPY-DHCer) was synthesized from dihydrosphingosine and BODIPY acid according to Kok& Hoekstra [18] The enzyme assay was performed in tubes precoated with dipalmitoylphos-phatidylcholine (15 nmoles added in chloroform and dried down under nitrogen) by adding the fluorescent ceramide (300 ng per tube) precoupled to BSA, the parasite lysates (50–100 lg protein) and the assay buffer (0.1M sodium phosphate buffer pH 7.4 containing 25 mM KCl, 5 mM

MgCl2, 2.5 mMUDP-glucose and 2 mMb-NAD) in a final volume of 150 lL The reaction was incubated at 37C for

5 h with shaking The mixture was extracted with chloro-form/methanol (1 : 1, v/v) and analysed by TLC Spots were visualized using a Fuji LAS1000 densitometer equipped with

IMAGE GAUGE3.122, software, Fuji Film, Japan

All protein determinations were performed using Brad-ford’s method [19]

Immunoprecipitation Parasite lysates (1–2 mg protein) were incubated with GCS 1.2 antibody (which recognizes a region near the GCS C-terminus) [20] in buffer Tris/HCl pH 8.0 containing

150 mMNaCl, 0.5% (w/v) sodium deoxycholate and 0.1% (w/v) SDS, for 2 h at 5C Protein A-Sepharose (10% in the same buffer, 100 lL) was added and it was incubated for a further 60 min The mixture was centrifuged at 10 000 g and the immunoprecipitate was washed (3· 100 lL) The immunoprecipitates were dissolved in sample buffer and subjected to SDS/PAGE in 10% gels Western blot to poly(vinylidene difluoride) membrane was performed and blots were probed with anti-peptide polyclonal antibodies GS-5.1 (1/1500) which recognizes a region near the GCS N-terminus [20] followed by an anti-rabbit horseradish peroxidase secondary antibody and visualized using ECL (Amersham) enhanced chemiluminescence reagent UV-MALDI-TOF MS analysis

Matrices for UV-MALDI-TOF MS The b-carboline (9H-pyrido[3,4-b]indole), nor-harmane and 2,5-dihydroxy-benzoic acid were obtained from Aldrich Chemical Co Calibrating chemicals for UV-MALDI-TOF analysis a-Cyclodextrin (cyclohexaamylose, Mr972.9), b-cyclodex-trin (cycloheptaamylose, Mr1135.0), c-cyclodextrin (cyclo-octaamylose, Mr 1297.1), angiotensin I (Mr 1296.49), neurotensin (Mr1672.96) and bovine insulin (Mr5733.5) were purchased from Sigma-Aldrich

Solvents Methanol, ethanol, acetonitrile (Sigma-Aldrich HPLC grade) and trifluoroacetic acid (Merck) were used as purchased without further purification Water of very low conductivity (Milli Q grade; 56–59 nSÆcm)1with PURIC-S (ORUGANO Co., Ltd, Tokyo, Japan) was used

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UV-MALDI-TOF-MS experiments Measurements were

performed using a Shimadzu Kratos, Kompact MALDI 4

(pulsed extraction) laser-desorption time-of-flight mass

spectrometer (Shimadzu, Kyoto, Japan) equipped with a

pulsed nitrogen laser (kem¼ 337 nm; pulse width ¼ 3 ns),

tunable pulse delay extraction (PDE), post source decay

(PSD) (MS/MS device) and a secondary electron multiplier

Experiments were first performed using the full range

setting for laser firing position in order to select the optimal

position for data collection, and secondly fixing the laser

firing position in the sample sweet spots The samples were

irradiated just above the threshold laser power for obtaining

molecular ions and with higher laser power for studying

cluster formation Thus, the irradiation used for producing

a mass spectrum was analyte-dependent with an

acceler-ation voltage of 20 kV Usually 50 spectra were

accumu-lated

All samples were measured in the linear and the reflectron

modes, in both the positive- and the negative-ion mode

The stainless steel polished surface 2 sample-slides were

purchased from Shimadzu Co., Japan (P/N 670-19109-01)

Polished surface slides were used in order to get better

images for morphological analysis with a stereoscopic

microscope (NIKON Optiphot, Tokyo, Japan;

magnifica-tion·400) and with a high-resolution digital microscope

(Keyence VH-6300, Osaka, Japan; magnification·800)

Sample preparation Matrix stocksolutions were made by

dissolving 1 mg of the selected compound in 0.5 mL of 1 : 1

(v/v) methanol/water Analyte solutions were freshly

pre-pared by dissolving the samples (0.05 mg) in chloroform/

methanol, 1 : 1 (v/v) (0.025 mL)

To prepare the analyte-matrix deposits two methods were

used Method A; thin-film layer method (sandwich

method) Typically 0.5 lL of the matrix solution was placed

on the sample probe tip, and the solvent removed by

blowing air at room temperature Subsequently, 0.5 lL of

the analyte solution was placed on the same probe tip

covering the matrix and partially dissolving it, and the

solvent was removed by blowing air Then, two additional

portions (0.5· 2 lL) of the matrix solution were deposited

on the same sample probe tip, producing a partial

disso-lution of the previously deposited thin-film matrix and

analyte layers The matrix to analyte ratio was 3 : 1 (v/v)

and the matrix and analyte solution loading sequence was:

(a) matrix, (b) analyte, (c) matrix and (d) matrix Method B;

mixture method The analyte stocksolution was mixed with

the matrix solution in 1 : 1–1 : 12 (v/v) ratio A 0.5 lL

aliquot of this analyte-matrix solution was deposited onto

the stainless steel probe tip and dried with a stream of forced

room temperature air Then, an additional portion of 0.5 lL was applied to the dried solid layer on the probe, causing it to redissolve partially, and the solvent was removed by blowing air

The resulting solid partially crystalline layers were found to be relatively homogeneous in both cases nor-Harmane and 2,5-dihydroxybenzoic acid as matrices showed signals of higher quality by using Method A Thus, the results shown and discussed in the present article are those obtained using this sample preparation method for each analyte, in the optimum experimental conditions

Spectra were calibrated using external calibration rea-gents: (a) commercial proteins (neurotensin; angiotensin I; bovine insulin) and (b) a-, b- and c-cyclodextrins with nor-harmane as matrix, in positive- and in negative-ion mode TheKRATOS KOMPACTcalibration program was used

Results

Metabolic labeling of GSLs Cultures of P falciparum with parasitemia around 10% (4.5% ring forms, 2.7% trophozoites and 1.6% schizonts) were metabolically labeled with [14C]palmitic acid for 18 h The different stages were purified on a Percoll gradient and extracted with chloroform/methanol (1 : 1, v/v) A control

of uninfected erythrocytes was analysed in parallel (Table 1) The different extracts were further fractionated

by DEAE-Sephadex A-25 (ACO–) column chromatography into neutral and acidic lipids TLC analysis of the unbound fraction showed that the radioactive precursor was mainly incorporated into diacyl-phospholipids (phosphatidylcho-line, phosphatidylethanolamine and their lyso-derivatives)

as reported previously [21] (not shown) The acidic fraction corresponding to the schizont stage showed a significantly high incorporation in comparison with ring and trophozoite stages (Table 1); thus similar amounts of radioactivity of each fraction was applied to the TLC plate Acidic lipids analysed in solvent A showed main spots corresponding

to phosphatidylinositol, phosphatidic acid and fatty acids (Fig 1A)

The unbound fraction of each stage was treated with 0.1MNaOH in methanol to hydrolyse non ceramide-based lipids and after purification, the samples were analysed

by TLC in solvent B (Fig 1B) These lipidic components migrated close to standards of GSLs A spot with the mobility similar to a standard of sphingomyelin was also shown Even though the sample of control erythrocytes used was enhanced, only faint bands were observed In

Table 1 Incorporation of radioactive precursors (CPM per 108parasites) in the different fractions of lipids obtained fromthe three intraerytrocytic stages of P falciparum C, control uninfected erythrocytes; R, rings; T, trophozoites; S, schizonts.

[ 14 C]palmitic acid [ 14 C]glucose

Total lipids Neutral lipids Acidic lipids Sphingolipids Total lipids Neutral lipids Acidic lipids Sphingolipids

S 6269500 5192900 73800 115900 87854 71200 2500 3800

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order to ensure that the labeled components corresponded

to ceramide-based lipids, the spot comigrating with the

standard of GbOse3Cer (IV) from the schizont fraction

(Fig 1B, lane 4), was eluted from the plate and digested

with ceramide glycanase As expected, hydrolysis was not

complete, however, a new spot comigrating with a standard

of ceramide was obtained (Fig 2A)

A sample of the saponified neutral lipids obtained from schizont stages, was further purified by Unisil column chromatography Three fractions (CHCl3, CHCl3/MeOH (98 : 2, v/v), and CHCl3/MeOH (1 : 3, v/v)) were eluted The latter, containing the glycosphingolipids, was hydro-lysed with HCl/MeOH/water (3 : 29 : 4, v/v/v), treated with BF3/MeOH to methylate the rest of the fatty acids that could interfere, and analysed by TLC in solvent C (Fig 2B) Two spots, migrating in the region where long chain bases are resolved, were detected One of them (RF 0.33) with the mobility of an authentic standard of C18 -sphinganine, the other one (RF 0.38) migrating slightly above, would correspond to C20-sphinganine A similar result was obtained when the spot comigrating with GbOse3Cer was analysed under the same conditions (not shown)

In another experiment a [14C]glucose incorporation was tried The three sugar labeled stages were extracted as above and the extracts were fractionated by DEAE-Sephadex column chromatography and saponified Although recov-eries in ring and trophozoite forms extracts were low, the purified glycosphingolipid fraction obtained from the schizont stage showed a significant higher incorporation

of the radiolabeled sugar than uninfected erythrocytes (Table 1) This extract was analysed by TLC in solvent B

in comparison with an analogous [14C]palmitic acid labeled fraction (Fig 3A) Four spots with RF similar to those obtained by [14C]palmitic acid labeling were detected Cerebroside (RF 0.85) was clearly resolved in the sugar labeled sample (Fig 3A, lane 2) When a similar experiment was performed using [14C]galactose as precursor, a faint band corresponding to galactosylceramide was also detected (Fig 3B)

In order to further analyse each GSL fraction, extracts obtained from schizont stages were fractionated as above and the neutral GSL fraction was subjected to TLC in parallel with an analogous [14C]palmitic acid labeled

Fig 1 Incorporation of [14C]palmitic acid into lipids of Plasmodium falciparum (A) TLC analysis in chloroform/methanol/water (65 : 25 : 3, v/v/v) of the acidic lipids Samples obtained from 4.8 · 10 7 ring forms (lane 1), 2.06 · 10 7 trophozoites (lane 2) and 4.38 · 10 6

schizonts (lane 3) were spotted in order to apply similar amounts of radioacti-vity PtdGr, phosphatidyl glycerol; PtdH, phosphatidic acid; PtdIns, phosphatidylinosi-tol; PtdSer, phosphatidylserine; lysoPtdIns, lysophosphatidylinositol (B) The unbound fractions of the DEAE-Sephadex column were saponified and analysed by TLC Samples corresponding to: 2.4 · 10 8 ring forms (lane 1); 1.0 · 10 8

trophozoites (lane 2); 0.4 · 10 8

schizonts (lane 3); 7.0 · 10 8

non-infected erythrocytes (lane 4) were analysed in chloroform/methanol/0.25% KCl (80 : 30 : 2, v/v/v); I, ceramide; II, glucosylceramide; III, lactosylceramide; IV, globotriaosylceramide;

V, globotetraosylceramide; VI, sphingo-myelin.

Fig 2 Hydrolysis of the GSLs The spot comigrating with GbOse 3 Cer

(IV) from Fig 1B (lane 4) was incubated with ceramide glycanase for

18 h, extracted with choroform/methanol and further subjected to

TLC in chloroform/metanol/0.25% KCl (80 : 30 : 2, v/v/v) Cer,

cer-amide; GbOse 3 Cer, globotriosylceramide The glycosphingolipid

fraction metabolically labeled with [14C]palmitic acid was subjected to

methanolysis, further treated with BF 3 /methanol and analysed by TLC

in chloroform/metanol/1 M NH 4 OH (40 : 10 : 1, v/v/v).C 18 -Sph, C 18

-sphingosine; C -sSph, C - dihydrosphingosine.

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fraction Spots corresponding to the ceramide fraction (I),

monohexosylceramide fraction (II), trihexosylceramide

fraction (IV) and tetrahexosylceramide fraction (V) were

extracted from the plate and analysed by UV-MALDI-TOF

MS (Fig 4) Table 2 shows the m/z values (mass numbers)

and possible sphingoid-fatty acid-sugar combinations of

ceramides for the signals obtained from each fraction,

taking into account the results obtained by TLC analysis of

the long chain bases

Presence of an active glucosylceramide synthase

In a first approach, the activity of the enzyme in a parasite

lysate was examined using UDP-[14C]glucose as donor and

two different ceramides, palmitoyldihydrosphingosine and

palmitoylsphingosine as acceptors (Fig 5A) A spot

comi-grating with glucosylceramide was obtained but,

interest-ingly, although dihydroceramides are poor substrates for

the mammalian enzymes [22], P falciparum enzyme seemed

to be active only with the saturated compound (Fig 5A,

lane 2) In order to confirm the substrate specificity of the

plasmodial enzyme, the enzymatic assay was performed

using fluorescent ceramides and UDP-glucose as donor

Lysates from each stage were assayed and the products were analysed by TLC As expected, in the three stages, only parasite lysates incubated with BODIPY-DHCer as accep-tor synthesized fluorescent glucosylceramide (Fig 5B, lanes 1–3) No fluorescent product was obtained when the unsaturated ceramide was used (Fig 5B, lanes 4–6) The special substrate specificity assures the parasite origin of the detected enzyme activity

In order to show the presence of the GCS, immunopre-cipitation of parasite lysates from each stage was performed using polyclonal GCS 1.2 antibody [20] The immunopre-cipitates were subjected to SDS/PAGE and electrotrans-ferred to poly(vinylidene difluoride) membranes When the membranes were developed with the GCS 5.1 antibody, a band at a molecular mass of 48 kDa was detected in the three intraerythrocytic stages (Fig 5C, lanes 2–4) Never-theless, the possibility that the 48 kDa band can be due to a cross-reacting parasite protein not related with the GCS cannot be ruled out

In mammalian cells, low concentrations of D,L -threo-PPMP have no effect on sphingomyelin synthase but can inhibit the synthesis of glucosylceramides [23–26] In order

to establish if the plasmodial enzyme activity was affected, the experiment was performed in the presence of 5 lM

PPMP (Fig 5D) TLC analysis revealed that the presence of threo-PPMP efficiently inhibited the synthesis of glucosyl-ceramides (52%) Additionally, the primary effect of PPMP seemed to be specifically on GSLs, because no difference in bulkprotein synthesis was seen when comparing whole [35S]methionine labeled precipitate of identical number of parasites that were left untreated or treated with 5 lM

PPMP (Fig 5D)

Previous reports showed that treatment of parasite cultures with PPMP resulted in a potent inhibition of the intraerythrocytic development of P falciparum [27–30]

In order to determine the effect of the inhibitor in GSLs synthesis, treatment of parasite cultures with threo-PPMP for 24 h was performed followed by incorporation of [14C]glucose or [14C]palmitic acid in the presence of the drug Parasite development was monitored by microscopy

of Giemsa-stained blood smears (Table 3) As expected, treatment with threo-PPMP showed inhibition of the intraerythrocytic development at the ring stage as described

by Haldar et al [26] Each labeled stage from treated and nontreated parasites was purified by Percoll gradient and further fractionated as above to achieve purified GSLs Comparison of the incorporation of [14C]glucose in the same number of treated and nontreated parasites, showed a clear reduction in the ring stage (Fig 6A) As a result of the arrest on development, a low amount of treated trophozoite and schizont stages were obtained, this fact joined to a low incorporation of the sugar precursor precluded further analysis of these stages Fractions corresponding to the same number of [14C]glucose-labeled ring forms were analysed by TLC in solvent A (Fig 6B) While the fraction obtained from nontreated ring forms showed spots corres-ponding to the labeled GSLs, no spots were detected in the fraction obtained from PPMP-treated ring forms

As regards the palmitic acid labeled parasites, the same analysis was carried out In accordance, when the incor-poration of palmitic acid was compared in treated and nontreated parasites, inhibition of the precursor

incorpor-Fig 3 Incorporation of [ 14 C]glucose and [ 14 C]galactose into

glyco-sphingolipids of Plasmodium falciparum (A) TLC analysis in

chloro-form/methanol/0.25% KCl (80 : 30 : 2, v/v/v) of the unbound

fractions after mild alkaline treatment Lane 1, [14C]glucose labeled

control erythrocytes (7 · 10 8

cells); lane 2, [14C]glucose labeled gly-cosphingolipids from schizonts (4 · 10 8 cells); lane 3, [ 14 C]palmitic

acid labeled glycosphingolipids from schizonts (0.4 · 10 8

cells).

I, ceramide; II, glucosylceramide; III, lactosylceramide; IV,

globo-triaosylceramide; V, globotetraosylceramide; VI, sphingomyelin (B)

TLC analysis in chloroform/methanol/0.25% KCl (80 : 30 : 2, v/v/v)

of the glycosphingolipid fraction purified from schizonts (1.4 · 10 8

parasites) after metabolic incorporation of [ 14 C]galactose GalCer,

galactosylceramide.

Trang 7

ation in the three stages was also observed (Fig 6C) The lipidic precursor is incorporated more efficiently probably

as a result of the development of the membrane network Consequently, even when a low amount of schizont stages was obtained, the comparison on TLC could be carried out (Fig 6D) Interestingly, treated schizonts showed no gly-cosphingolipid components in contrast with the nontreated samples

Discussion

Glycosphingolipids seem to be a general feature of eukary-otic cells However, the physiological functions of these glycolipids have only been documented in mammalian cells, whereas very little information is available of their roles in other systems [31] In this report we show for the first time the presence of an active glucosylceramide synthase in the intraerythrocytic stages of P falciparum Incorporation of [14C]palmitic acid and [14C]glucose allowed the analysis of purified glycosphingolipids When the long chain base component of these GSLs was investigated, using [14 C]pal-mitic acid as a precursor, labeled sphinganine was obtained (Fig 3) in contrast with the major long chain base present in erythrocytes, indicating clearly the parasite origin of the detected compound Degradation of host sphingomyelin to produce ceramide for parasite growth has been suggested, supported by the existence of sphingomyelinase in P falci-parum[30,32,33] However, although the amount of

cera-Fig 4 UV-MALDI-TOF mass spectra in positive ion mode of the different GSLs frac-tions Values indicate m/z of sodium adducted molecular ions, [M + Na] + , in nominal mass Posible ceramide species are listed in Table 2 (A) UV-MALDI-TOF MS of cera-mides (fraction I) in reflectron mode; matrix: nor-harmane; (B) UV-MALDI-TOF MS of monohexosylceramides (fraction II) in linear mode; matrix: nor-harmane; (C) UV-MALDI-TOF MS of globotriaosylceramides (frac-tion IV) in reflectron mode; matrix: 2,5-di-hydroxybenzoic acid; (D) UV-MALDI-TOF

MS of globotetraosylceramides (fraction V) in reflectron mode; matrix: 2,5-dihydroxybenzoic acid.

Table 2 Mass numbers and possible sphingoid-fatty acid-sugar

combi-nations of ceramides in the different fractions of GSLs obtained from

schizont forms after TLC analysis Molecular related ions, [M+Na]+

are expressed as nominal mass Listed ceramide species were deduced

from UV-MALDI-TOF MS spectra (Fig 4) Spectra are shown in

Fig 6 m/z, Data from UV-MALDI-TOF MS.

Spectra m/z Proposed structures

A 494.9 d18:0-C10h:0

522.9 d20:0-C10h:0 d18:0-C12h:0

537.2 d20:0-C10-2h:0 d18:0-C12-2h:0

550.8 d18:0-C14h:0 d20:0-C12h:0

553.2 d20:0-C10-3h:0 d18:0-C12-3h:0

569.0 d18:0-C14-2h:0 d20:0-C12-2h:0

B 522.5 d20:0-C10h:0 d18:0-C12h:0

550.7 d18:0-C14h:0 d20:0-C12h:0

568.0 d18:0-C14-2h:0 d20:0-C12-2h:0

656.6 d18:0-C10h:0(+hex)

686.3 d20:0-C10h:0 (+hex) d18:0-C12h:0 (+hex)

C 1036.5 d18:0-C14h:0(+3hex) d20:0-C12h:0(+3hex)

1052.3 d18:0-C14-2h:0(+3hex) d20:0-C12-2h:0(+3hex)

1068.3 d18:0-C14-3h:0(+3hex) d20:0-C12-3h:0(+3hex)

D 1038.2 d18:0-C14h:0(+3hex) d20:0-C12h:0(+3hex)

1052.8 d18:0-C14-2h:0(+3hex) d20:0-C12-2h:0(+3hex)

1185.0 d18:0-C10h:0

(3hex+hexNAc)

Trang 8

mide produced is low, our results confirm that the de novo

biosynthetic pathway of ceramides is active in this parasite

Nevertheless, the last step of the ceramide biosynthesis,

involving the dehydrogenation of N-acylsphinganine to

N-acylsphingenine would be absent in P falciparum

Addi-tionally, [14C]galactose incorporation showed the presence

of galactosylceramide as reported recently [11]

The sphingolipid structure of the different products obtained in the GSL fraction was proven by UV-MALDI-TOF mass spectrometry The spectra showed that the predominant components of Fraction I were ceramides involving long chain bases d18:0 or d20:0 and hydroxy fatty acids C10:0, C12:0 and C14:0 bearing one, two or three hydroxy residues (Fig 4A, Table 2) This

Fig 5 Glucosylceramide synthase analysis (A) The enzymatic assay was performed using UDP-[14C]glucose as marker in 0.1 M sodium phosphate buffer (pH 7.4), 2 m M b-NAD and a liposomal substrate consisting in dipalmitoylphosphatidylcholine and ceramide (10 : 1) (containing 0.1 nmol

of ceramide) The mixture was purified and analysed by TLC in chloroform/methanol/water (65 : 25 : 2, v/v/v) Lane 1, palmitoylceramide; lane 2, palmitoyldihydroceramide (B) The enzyme assay was performed using the fluorescent ceramide precoupled to BSA, UDP-glucose (2.5 m M ) and

2 m M b-NAD The parasite lysates (50–100 lg protein) in 0.1 M sodium phosphate buffer (pH 7.4) The mixture was extracted with chloroform/ methanol (1 : 1) and analysed by TLC in chloroform/methanol/water (65 : 25 : 2, v/v/v) Lanes 1, 2 and 3 are rings, trophozoites and schizonts, respectively, using BODIPY-dihydroceramide; lanes 4, 5 and 6, the same using BODIPY-ceramide (C) Immunoprecipitation of parasite lysates performed using polyclonal GCS 1.2 antibody The immunoprecipitates were subjected to SDS/PAGE and electrotransferred to poly(vinylidene difluoride) membranes The membranes were developed with the GCS 5.1 antibody followed by ECL 1, control erythrocytes; 2, ring forms; 3, trophozoites; 4, schizonts Molecular mass of markers is indicated (in kDa) at the right side of the figure The arrow at the left side shows the band at

48 kDa (D) The enzymatic assay was performed using schizonts as enzymatic source as in (A), without (lane 1) or with (lane 2) 5 l M PPMP as inhibitor GlcCer, glucosylceramide; R, ring forms; T, trophozoites; S, schizonts (E) Incorporation of L -[ 35 S]methionine in proteins obtained from parasites treated or nontreated with 5 l M PPMP Aliquots were collected at different times (0–48 h), precipitated with 12% (w/v) trichloroacetic acid and radioactivity was measured.

Trang 9

finding is in accordance with a previous report showing

that the de novo biosynthetic pathway of fatty acids in

P falciparum involved C10:0 to C14:0, some of them

hydroxylated [34] Fraction II migrated very near Frac-tion I and was shown to be a mixture of ceramides and monohexosyl ceramides, not very well resolved The latter were mainly monohexosylceramides of d18:0 and d20:0 acylated with C10h:0 and C12h:0 (Fig 4B, Table 2) Spectrum C (Fig 4) showed that the predominant com-ponent of Fraction IV is a trihexosylceramide (m/z 1036.5) with a possible sphingoid-fatty acid combination d18:0-C14h:0 (or d20:0-C12h:0) On the other hand, Fraction V (Fig 4, spectrum D) showed less intense signals than the others However, it was very interesting

to detect a component of m/z 1185.0 corresponding to a tetrahexosylceramide bearing an N-acetylhexosamine resi-due This result agrees with the fact that incorporation of tritiated glucosamine led to the preferential detection of GSLs migrating as highly glycosylated species [10] Biosynthesis of GSLs in P falciparum pointed to the presence of an active glucosylceramide transferase When the enzyme activity was searched in parasite lysates using UDP-[14C]glucose as marker as well as using fluorescent ceramides, activity was found only when the dihydrocera-mide was used as substrate This is in good agreement with the result described above and would explain earlier reports showing that the parasites were not competent to the formation of glucosylceramide when using unsaturated ceramides [28]

GCS from different eukaryotic kingdoms have been cloned; remarkably their sequences present only a few conserved amino acids and the overall similarity between the enzymes from species with remote evolutionary rela-tionship is rather low [31] In particular for P falciparum,

we were unable to find any sequences related to GCS This is not rare as it has been suggested that enzymes are more difficult to identify in P falciparum by sequence similarity methods The difficulty has been attributed either to the great evolutionary distance between P falciparum and other well studied organisms or to the high A + T content

of the genome [35] Nevertheless, we detected a potential gene for GCS (GenBankTM, accession number NP_701286) with conserved domains for glycosyltransferases [36] However, in an attempt to detect the presence of the plasmodial enzyme in a parasite lysate, immunoprecipita-tion with polyclonal antibodies against the human GCS was tried A band of molecular mass near 48 kDa was recognized in the three stages of the parasite This band

Fig 6 Inhibition of GSL synthesis by threo-PPMP treatment GSLs

were purified from threo-PPMP treated and nontreated parasites and

were further analysed by TLC in chloroform/methanol/water

(65 : 25 : 3, v/v/v) (A) Comparison of the radioactivity recovered in

the GSL fractions obtained from treated (unfilled bars) and nontreated

(filled bars) [14C]glucose incorporated parasites R, ring forms;

T, trophozoites; S, schizonts; C, control uninfected erythrocytes (B)

Lane 1, control [ 14 C]glucose labeled ring stage (4.2 · 10 8 parasites);

lane 2, threo-PPMP-treated [ 14 C]glucose labeled ring stage (5.5 · 10 8

parasites) (C) Comparison of the radioactivity recovered in the GSL

fractions obtained from treated (unfilled bars) and nontreated (filled

bars) [ 14 C]palmitic acid incorporated parasites R, ring forms;

T, trophozoites; S, schizonts; C, control uninfected erythrocytes (D)

Lane 1, control [14C]palmitic acid labeled schizonts (0.13 · 10 8

para-sites); lane 2, threo-PPMP-treated [ 14 C]palmitic acid labeled schizonts

(0.16 · 10 8

parasites) In all cases, at each stage, a similar number of

parasites was compared.

Table 3 Effect of 5 l M threo-PPMP on parasite development Parasite cultures were incubated with 5 l M DL -threo-PPMP After 24 h of treatment, parasites were labeled with [ 14 C]palmitic acid or [ 14 C]glu-cose for 18 h in the presence of the drug Control cultures without the inhibitor were labeled under the same conditions The effect on para-site development was monitored by microscopy of Giemsa-stained blood smears in two independent experiments R, ring forms;

T, trophozoites; S, schizonts.

[ 14 C]glucose [ 14 C]palmitic acid

Control Treated Control Treated

Trang 10

was absent in the control erythrocytes The apparent Mr

resembles the predicted molecular mass of the human and

rat GCS polypeptides although the empirical molecular

mass described is 38 kDa [20]

In mammalian cells, low concentrations (1–5 lM) ofD,L

-threo-PPMP have no effect on sphingomyelin synthase but

can inhibit the synthesis of glucosylceramides In P

falcipa-rum, PPMP has been described as a potent inhibitor of the

intraerythrocytic maturation leading to an arrest of the

parasites at ring stage Rings formed in the presence of

the drug contain no tubular structures On the contrary,

mature trophozoites and schizonts that contain a fully

extended tubular networkwere not affected by the drug

[26,27,29,30] When we tried the action of PPMP in vitro on

the GCS, using UDP-[14C]glucose as marker, the enzyme

activity which resulted was clearly reduced (Fig 5D) In

another experiment, when the inhibitor was added in parasite

cultures, we observed an arrest on parasite development

Parasites collected at the ring stage had been treated with

PPMP at the trophozoite stage ( 40 h before), and resulted

unaffected (Table 3) On the contrary, parasites collected at

the schizont stage that had received the inhibitor at the ring

stage, were not able to evolve and died When the [14

C]glu-cose labeled GSL fraction purified from PPMP treated and

from control parasites collected at the ring stage were

compared by TLC, disappearance of GSLs was shown

(Fig 6B) This fact indicates that although parasites are able

to evolve to the ring stage, no new GSLs are biosynthesized

Using [14C]palmitic acid as precursor, the analysis could

also be performed with the schizont stage Likewise,

para-sites treated with PPMP showed disappearance of GSLs

(Fig 6D) In this case two hypotheses may be postulated:

PPMP is also acting on the glucosyltransferase and although

there is de novo synthesis of ceramides, the glycosylating step

is blocked; or, parasites that overcome treatment are so

stressed that the tubovesicular membrane networkis not able

to import the lipidic precursor Anyway, the possibility of

both events taking place simultaneously must be considered

In conclusion, we have isolated and characterized the

major GSL structures present in the intraerythrocytic forms

of P falciparum by UV-MALDI-TOF mass spectrometry

A glucosylceramide synthase activity with specificity for

saturated ceramides which can be inhibited by low

concen-trations of PPMP was identified for the first time The

inhibitor, used in cultures, arrests parasite development with

a concomitant depletion of GSLs The special feature

presented by the plasmodial GCS, joined to the expanding

number of cellular functions that may be glycosphingolipid

dependent, makes this enzyme a promising target for

antimalarial drug development Studies are underway for

the characterization of the enzyme and its intracellular

location in P falciparum

Acknowledgements

This workwas supported by grants from: CONICET, Universidad de

Buenos Aires and Agencia Nacional de Promocio´n Cientı´fica y

Tecnolo´gica (Pict 06-06545), Argentina FAPESP, CNPq, PRONEX,

Brazil, UNDP/World Bank/WHO (TDR) A S C and R E.-B are

members of Research Council CONICET (Argentina) and C C.,

ANPCyT fellow Mass spectrometry was performed as part of the

Academic Agreement between R E.-B and H N with the facilities of

the High Resolution Liquid Chromatography-integrated Mass Spec-trometer System Laboratory of the United Graduate School of Agricultural Sciences (Ehime University, Japan) and partially suppor-ted by Heiwa Nakajima Foundation.

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