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In this context, we have previously reported [2] that exogenous sphingomyelinase EC 3.1.4.12 treatment brought about alterations in brain ethanolamine Etn plasmalogen metabolism.. Taking

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Signaling events mediating activation of brain ethanolamine

plasmalogen hydrolysis by ceramide

Eduardo Latorre1, M Pilar Collado1, Inmaculada Ferna´ndez1, M Dolores Aragone´s1

and R Edgardo Catala´n2

1

Departamento de Bioquı´mica y Biologı´a Molecular I, Facultad de Quı´micas, Universidad Complutense de Madrid, Madrid, Spain;

2

Departamento de Biologı´a Molecular, Centro de Biologı´a Molecular ‘Severo Ochoa’, Universidad Auto´noma de Madrid,

Madrid, Spain

Ceramide is a lipid second messenger that acts on

mul-tiple-target enzymes, some of which are involved in other

signal-transduction systems We have previously

demon-strated that endogenous ceramide modifies the metabolism

of brain ethanolamine plasmalogens The mechanism

involved was studied On the basis of measurements of

breakdown products, specific inhibitor effects, and

previ-ous findings, we suggest that a plasmalogen-selective

phospholipase A2 is the ceramide target

Arachidonate-rich pools of the diacylphosphatidylethanolamine subclass

were also affected by ceramide, but the most affected

were plasmalogens Concomitantly with production of

free arachidonate, increased 1-O-arachidonoyl ceramide

formation was observed Quinacrine (phospholipase A2

inhibitor) and

1-O-octadecyl-2-O-methyl-rac-glycerol-3-phosphocholine (CoA-independent transacylase inhibitor)

prevented all of these ceramide-elicited effects Therefore,

phospholipase and transacylase activities are tightly cou-pled Okadaic acid (phosphatase 2A inhibitor) and

PD 98059 (mitogen-activated protein kinase inhibitor) modified basal levels of ceramide and sphingomyelinase-induced accumulation of ceramide, respectively Therefore, they provided no evidence to determine whether there is a sensitive enzyme downstream of ceramide The evidence shows that there are serine-dependent and thiol-dependent enzymes downstream of ceramide generation Further-more, experiments with Ac-DEVD-CMK (caspase-3

speci-fic inhibitor) have led us to conclude that caspase-3 is downstream of ceramide in activating the brain plasmalo-gen-selective phospholipase A2

Keywords: brain ethanolamine plasmalogens; caspase-3; ceramide; phospholipase A2; plasmalogen-selective phos-pholipase A2

It is well known that messengers derived from sphingolipid

and glycerolipid, and their target enzymes, establish

mul-tiple relationships leading to the formation of complicated

networks for the effective transduction of signals In the last

few years, the regulatory role of ceramide (Cer) generated by

the sphingomyelin cycle has received increasing attention It

is known to activate multiple serine/threonine protein

kinases and protein phosphatases [1], leading to the

tissue-specific downstream regulation of several target enzymes,

some of which are involved in other lipid signaling

pathways In this context, we have previously reported [2]

that exogenous sphingomyelinase (EC 3.1.4.12) treatment brought about alterations in brain ethanolamine (Etn) plasmalogen metabolism

The role of plasmalogens as a source of second messengers in lipid signal-transduction systems [3–5] and

as ubiquitous endogenous antioxidants [6] has been investigated Plasmalogens are phospholipids characterized

by the presence of a vinyl ether substituent at the sn-1 position of the glycerol backbone They are especially abundant in electrically active tissues, such as brain, where most of them are Etn-phosphoglycerides The latter have the propensity to facilitate membrane fusion, strongly suggesting their involvement in synaptic transmission [3]

In addition, Etn plasmalogens have been reported to be involved in the vulnerability to oxidative stress associated with aging and pathological conditions [6] Evidence is accumulating on age-related changes in the quantities [7] and fatty acid profile of these phospholipids [7,8] On the other hand, significant and selective deficiencies in brain Etn plasmalogens have been reported at the site of neurodegeneration in Alzheimer’s disease [9], brain peroxisomal disorders [8] and Down’s syndrome [10] In some instances, the decreased Etn plasmalogen levels are accompanied by a marked increase in the concentration of the degradation metabolites or their derivatives, such as PEtn [11] or prostaglandins [3] Therefore, the evidence suggests that several phospholipase types may be involved

Correspondence to R E Catala´n, Departamento de Biologı´a

Molecular, Centro de Biologı´a Molecular ‘Severo Ochoa’,

Universidad Auto´noma de Madrid, E-28049 Madrid, Spain.

Fax: + 34 91 3974870, Tel.: + 34 91 3974869,

E-mail: ecatalan@cbm.uam.es

Abbreviations: BSS, balanced salt solution; Cer, ceramide; C 2 -Cer,

N-acetylsphingosine; Etn, ethanolamine; ET-18-OCH 3 ,

1-O-octa-decyl-2-O-methyl-rac-glycerol-3-phosphocholine; MAPK,

mitogen-activated protein kinase; PLA 2 , phospholipase A 2 ;

PtdEth, phosphatidylethanolamine.

Enzymes: phospholipase A 2 (EC 3.1.1.4); sphingomyelinase

(EC 3.1.4.12); CoA-independent transacylase (EC 2.3.1.147).

(Received 2 August 2002, revised 16 October 2002,

accepted 7 November 2002)

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in the metabolism of brain Etn plasmalogens in

physio-pathological states The existence of a

plasmalogen-select-ive phospholipase A2(PLA2, EC 3.1.1.4) which selectively,

but not exclusively, acts on

1-alk-1¢-enyl-2-acyl-sn-glycero-3-PEtn has been reported [4,5,12] This enzyme has been

purified from bovine brain and shown to be specific for

neural tissues and distinct from other non-neuronal

plasmalogen-specific PLA2 enzymes and brain PLA2

enzymes [3,4] There is evidence that Etn plasmalogen

degradation by a PLA2 plays an important role in

neutrophil activation by agonists [13] From the latter

study and others [14–16], the idea has emerged that Etn

plasmalogen hydrolysis may be coupled with the formation

of acyl Cers, eicosanoids and/or platelet-activating factor

Thus, PLA2in the presence of a suitable acceptor molecule

possesses a dual enzymatic function, i.e PLA2 and

CoA-independent transacylase, generating: (a) free arachidonate,

which can be converted into eicosanoids [13,15], and (b) an

acyl derivative, mainly the arachidonoyl derivative [16] On

the other hand, Etn plasmalogens can be resynthesized

from the lyso-plasmenylEtn released by a

CoA-independ-ent transacylase from 1-radyl-2-arachidonoylGroPCho,

generating lyso-platelet-activating factor derivatives, which

can lead to formation of platelet-activating factor by

transacetylation [15,16] We would like to emphasize that

all this experimental evidence has been obtained in

non-neural cell-free systems or isolated cells

Taking into account that the following have been

reported, (a) a brain plasmalogen-selective PLA2[4,5], (b)

a brain PLA2acting on Etn phosphoglyceride with

trans-acylase activity [16], and (c) a Cer-elicited decrease in brain

Etn plasmalogen levels concomitant with 1-O-acylCer

formation [2], the aim of this study was to clarify the

mechanism by which Cer regulates brain Etn plasmalogen

metabolism First, we investigated the type of enzymatic

activities involved and, secondly, the involvement of

poten-tial downstream Cer target enzyme(s)

Materials and methods

Materials

Staphylococcus aureus sphingomyelinase [180 UÆ(mg

pro-tein))1], N-acetylsphingosine (C2-Cer), Cer type III (from

brain sphingomyelin containing primarily stearic and

ner-vonic acids), phenylmethanesulfonyl fluoride, quinacrine

hydrochloride, ganglioside type II (from bovine brain

containing 15% N-acetylneuraminic acid) were purchased

from Sigma, St Louis, MO, USA Bromoenol lactone was

from Alexis Biochemicals, La¨ufelfingen, Switzerland

[1-14C]Arachidonic acid (55 mCiÆmmol)1) was from

American Radiolabeled Chemicals Inc., St Louis, MO,

USA [c-32P]ATP (3000 CiÆmmol)1) was from Nuclear

Iberica, Madrid, Spain [2-14C]Ethan-1-ol-2-amine

hydro-chloride (55 mCiÆmmol)1) was purchased from Amersham

Escherichia coli diacylglycerol kinase (EC 2.7.1.107),

2¢-amino-3¢-methoxyflavone (PD 98059), okadaic acid,

Ac-DEVD-chloromethylketone (Ac-DEVD-CMK) and

a-iodocetamide were from Calbiochem, San Diego,

CA, USA 1-O-Octadecyl-2-O-methyl-rac-glycerol-3-PCho

(ET-18-OCH3) was from Bachem AG, Budendorf,

Switzer-land High-performance TLC plates were obtained from

Merck, Darmstadt, Germany All other reagents were of the highest analytical grade available 1-O-AcylCer standard was synthesized as described previously [2]

Tissue preparation and incubation of slices Experiments were carried out with male Wistar rats (180–

200 g) The animals were maintained at 22–24C and given free access to standard laboratory diet and water

ad libitum Rat care, handling and all the experimental procedures were in accordance with internationally accep-ted principles concerning the care and use of laboratory animals The rats were killed [2], and their brains were removed Pial vessels and white matter were carefully discarded, and cerebral cortex was obtained Slices (dimensions: 350· 350 lm) were prepared with a MacIl-wain tissue chopper, as previously reported [17] They were equilibrated in a balanced salt solution (BSS): 135 mM NaCl, 4.5 mM KCl, 1.5 mM CaCl2, 0.5 mM MgCl2, 5.6 mM glucose, 10 mM Hepes, pH 7.4, equilibrated with 95% O2/5% CO2 for 1 h Aliquots (300 lL) of gravity-packed slices were transferred to glass tubes containing BSS and then sphingomyelinase (unless otherwise indica-ted, the final concentration was 0.38 UÆmL)1, as described previously [2]) dissolved in 50 mM phosphate buffer,

pH 7.4, with 50% (v/v) glycerol, or C2-Cer dissolved in dimethyl sulfoxide (10–100 lM), or diluents alone were added and the mixture incubated for 30 min at 37C [2]

In one set of experiments, slices were treated with 0.1 lM endothelin-1 for 30 min [18] In experiments in which different inhibitors were tested, the slices were preincubated

in their absence or presence before the addition of sphingomyelinase or C2-Cer When the inhibitors used were dissolved in dimethyl sulfoxide or ethanol, the final concentration of diluent was never higher than 1% The incubation mixtures were continuously gassed with 95%

O2/5% CO2 The incubations were stopped by removal of the medium and replacement with 0.38 mL BSS containing

10 mMEDTA and 1 mL chloroform/methanol/13MHCl (100 : 100 : 1, v/v/v) Lipids were immediately extracted as described below

As we used an inhibitor (ET-18-OCH3) with low diffusion through slices, some experiments with homogen-ates were performed Homogenhomogen-ates of cerebral cortex were prepared in BSS equilibrated as described above Previous comparative experiments showed that cerebral slices and homogenates exhibited the same responsiveness to the sphingomyelinase treatment [2]

Experiments with labeled precursors

In some experiments, labeled precursors were used Slices from 8–10 brains were preincubated in the presence of labeled precursors: 4 lCi (0.2 lCiÆmL)1) [1-14C]arachidonic acid [2] or 50 lCi (2 lCiÆmL)1) [2-14C]ethan-1-ol-2-amine hydrochloride [19] at 37C in BSS for 120 or 30 min, respectively The preincubations were continuously gassed with 95% O2/5% CO2 Then, the incubation medium was removed, and the slices were washed three times with cold BSS Aliquots of slices were taken for incubation with sphingomyelinase or C2-Cer; incubations were stopped as described above

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Extraction of total lipids; separation of sphingolipids

Lipids were extracted as described previously [20] The

organic phases were dried under a N2atmosphere, and total

lipids were weighed and dissolved in chloroform/methanol

(2 : 1, v/v) Lipids were separated by TLC 1-O-AcylCer

was resolved by sequential 1D TLC in: (a) ethyl ether; (b)

chloroform/methanol/acetic acid/water (25 : 15 : 4 : 1.5,

v/v/v/v), and (c) chloroform/methanol/acetic acid

(65 : 2.5 : 4, v/v/v) The first solvent system was developed

through the plate, the second reached 7 cm from the bottom

of the plate, whereas the third reached 13 cm from the

bottom This sequential TLC also resolves the nonesterified

fatty acid fraction and the Etn phospholipid subclasses, as

stated below To determine Cer levels, one aliquot of total

lipid was subjected to alkaline hydrolysis in 0.1M

metha-nolic KOH at 37C for 1 h to remove glycerolipids, as

previously described [2] Cer was resolved by sequential 1D

TLC using solvent systems (a) and (c) described above, but

the former reached 3 cm from the top of the plate, whereas

the latter was developed through the plate, as described

previously [2] Lipid standards were cochromatographed

with samples Lipids were visualized with iodine vapor, and

the bands of 1-O-acylCer and nonesterified fatty acids were

scraped from the plates to quantitate the radioactivity

incorporated by liquid scintillation The bands

correspond-ing to Cers were scraped from the plates and extracted with

chloroform/methanol (4 : 5, v/v) and dried under a N2

atmosphere for subsequent quantitation

Analysis of the subclasses of Etn phospholipids

Etn plasmalogen levels were determined as described

previously [2]

In some experiments with [14C]arachidonic acid as

precursor, three further subclasses of Etn phospholipids

were separated, as previously described [21] First, total Etn

phospholipids were obtained from the total lipids by

sequential 1D TLC as described above After extraction

with chloroform/methanol (2 : 1, v/v), the dry residue was

incubated with 40 U phospholipase C per sample for 16 h

The resulting diacylglycerols were extracted three times with

ether/hexane (1 : 1, v/v) Once the extracts had been dried,

acetylated derivatives were prepared by incubation for 3 h

in pyridine/acetic anhydride (1 : 5, v/v) The solution was

dried and extracted twice with ether/hexane (1 : 1, v/v) The

final dried residue was fractionated by TLC using

sequen-tially: (a) hexane/ether/methanol/acetic acid (90 : 20 : 3 : 2,

v/v/v/v), and (b) toluene as solvents [22] Phospholipids were

visualized with iodine vapor and identified from the

respective standards and reported Rfvalues Once scraped

from the plate, the radioactivity in each fraction was

measured by liquid scintillation

Radioenzymatic determination of Cer levels

Extracted Cer was phosphorylated in the presence of

diacylglycerol kinase, as described previously [2] Cers were

solubilized and phosphorylated in the presence of 5 lg of

the enzyme and 10 mM [c-32P]ATP for 10 min After

incubation, phosphorylated derivatives of Cer were

extrac-ted, fractionated by TLC, visualized by autoradiography

using Kodak X-Omat film and quantitated by liquid-scintillation counting Calibration curves were constructed using known amounts of Cer

Radioenzymatic determination of diacylglycerol mass Aliquots of total lipids were phosphorylated in the presence

of diacylglycerol kinase, as described previously [23] Aliquots of total lipids were evaporated under N2and the dried lipids were solubilized and phosphorylated in the presence of 5 lg enzyme and 10 mM[c-32P]ATP for 30 min Then, samples were spotted on silica gel TLC plates and developed with chloroform/methanol/acetic acid/acetone/ water (40 : 13 : 12 : 15 : 8, v/v/v/v) Spots corresponding to phosphatidic acid were visualized by autoradiography using Kodak X-Omat film and quantitated by liquid-scintillation counting Calibration curves were constructed using known quantities of 1-stearoyl-2-arachidonoylglycerol

Analysis of water-soluble products of hydrolysis

of Etn phospholipids

In experiments with [14C]Etn, the upper phases from the lipid extraction (see above) containing the water-soluble metabolites were analyzed by TLC [24] The upper phases were lyophilized and the residue was then dissolved in 50% ethanol, and Etn, PEtn and CDP-Etn tracers were added as carriers Water-soluble products were separated by TLC using methanol/0.5% NaCl/NH4OH (50 : 50 : 5, v/v/v) as solvent Bands were detected with 1% ninhydrin in ethanol Spots were scraped from the plate and analyzed for radioactivity counting

Determination of 1-O-alkenyl-2-lysoGroPEtn radioactivity

Aliquots of total lipids from experiments performed with [14C]Etn were subjected to alkaline hydrolysis and separated using TLC The system used was chloroform/methanol/ acetic acid (65 : 25 : 4, v/v/v) After development, spots were visualized with ninhydrin and identified from respect-ive standards Spots were scraped from the plates, and their mass determined by measurement of phosphorus content [25] The radioactivity incorporated was quantitated by liquid-scintillation counting

Statistical analysis Student’s t test was used for paired observations P < 0.05 was considered to be significant

Results

Sphingomyelinase and C2-Cer affect brain Etn plasmalogen metabolism

We have previously reported that Etn plasmalogen meta-bolism is specifically affected by sphingomyelinase treatment [2] Here we first studied the effect of different concentrations

of sphingomyelinase on Etn plasmalogen and Cer levels (Fig 1) At a concentration of 0.38 UÆmL)1, sphingomye-linase significantly (P < 0.05) decreased Etn plasmalogens

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to 65% (Fig 1A) Concomitantly, a significant (P < 0.05) increase in Cer levels was observed (100% over control value), in agreement with our previous data [2] Higher sphingomyelinase concentrations further increased Cer levels, but had no further effect on Etn plasmalogen levels (Fig 1A) A concentration of 0.19 UÆmL)1 sphingo-myelinase had a slight, but not significant, effect (data not shown)

As many effects evoked by sphingomyelinase treatment are mimicked by short-chain cell-permeable Cer analogs, we also tested the effect of C2-Cer on Etn plasmalogen levels The concentration range of C2-Cer was chosen on the basis

of previous evidence [26,27] A concentration of 50 lMwas the lowest capable of decreasing Etn plasmalogen levels by 55% of the control value (P < 0.05) (Fig 1B) Higher concentrations did not produce further variation in Etn plasmalogen levels

To determine the mechanism by which sphingomyelinase and C2-Cer affect Etn phosphoglyceride metabolism, we carried out labeling studies with [1-14C]arachidonic acid and [1-14C]Etn (Table 1) Sphingomyelinase and C2-Cer both significantly (P < 0.05) reduced labeling in the plasmalo-gen fraction but scarcely affected that in the acid-resistant fraction Interestingly, the most noticeable result was the low radioactivity from [1-14C]arachidonic found in the plasmalogen fraction ( 10% of the control value) when slices were treated with sphingomyelinase

Experiments to separate the Etn phosphoglycerides into their three subclasses, i.e diacyl, alkylacyl and plasmalo-gens, were also performed In the light of the above data (Table 1), we used [14C]arachidonate as the labeled precur-sor These results are presented in Table 2 Both diacyl and plasmalogen fractions exhibited significantly (P < 0.05) reduced radioactivity ( 30% of the control value) after treatment with sphingomyelinase or C2-Cer

Lipids were extracted [20] from one aliquot of incuba-tion medium, and 14C radioactivity was determined This provides a measure of activation of secretory PLA2 Results

in Table 3 show that extracellular [1-14C]arachidonate release was not affected, but the cell-associated 14C

Fig 1 Dose–response relationship of sphingomyelinase-induced and

C 2 -ceramide-induced changes in brain Etn plasmalogen levels (A)

Cerebral cortex slices were exposed to sphingomyelinase (SMase) for

30 min, and levels of Etn plasmalogens (PlsEtn; left axis; filled bars)

and ceramide (right axis; striped bars) were measured (B) Cerebral

cortex slices were exposed to C 2 -ceramide for 30 min, and Etn

plas-malogens were measured Data represent mean ± SE and are from

two experiments performed in triplicate Values significantly different

from their respective controls are indicated: *P < 0.05.

Table 1 Variations in [ 14 C]arachidonic acid-labeled and [ 14 C]Etn-labeled Etn phospholipids evoked by sphingomyelinase and C 2 -Cer Slices were labeled with 0.2 lCiÆmL)1[14C]arachidonic acid for 120 min or 2 lCiÆmL)1[14C]Etn for 30 min After removal of the labeled precursor, slices were exposed to 0.38 UÆmL)1sphingomyelinase or two different C 2 -Cer concentrations for 30 min Total lipids were split into two aliquots: one was untreated, and the other was exposed to HCl fumes Radioactivity in plasmalogen was obtained by subtracting the acid-resistant fraction from that obtained in the total Etn phospholipids Data are expressed as the percentage of radioactivity incorporated in each fraction with respect to that incorporated in total lipid They represent mean ± SD from one representative experiment of two experiments performed in quintuplicate.

ND, Not determined.

Treatment

Radioactivity incorporated into Etn phospholipids

Acid-resistant fraction

Plasmalogen fraction

Acid-resistant fraction

Plasmalogen fraction

C 2 -Cer

* P < 0.05 compared with respective control.

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radioactivity had increased by nearly 30% after treatment

with sphingomyelinase or C2-Cer

Identification of the phospholipase type involved

in the Cer-elicited decrease in Etn plasmalogen levels

To determine the type of enzymatic activity involved, we

next measured the levels of the breakdown products

released by phospholipase type D or C, i.e Etn, PEtn,

and diacylglycerol In addition, an intermediary of their

biosynthesis, CDP-Etn, was measured (Fig 2A,B) It is

evident that no significant alterations were elicited by

sphingomyelinase treatment The involvement of a PLA2

was tested by examining potential alterations in levels and

[14C]Etn radioactivity in the lyso form of Etn plasmalogens

evoked by sphingomyelinase or C2-Cer (Fig 2C,D,

respect-ively) Surprisingly, no significant changes were found in the

presence of 0.38 UÆmL)1sphingomyelinase (Fig 2C) How-ever, in a dose–response study with C2-Cer as agonist, a significant increase in the level of and radioactivity in lyso-Etn plasmalogens could only be observed in the presence of 100 lMC2-Cer (Fig 2D)

Before definitely establishing whether a PLA2was the Cer target, we next examined the effect of the widely used nonspecific PLA2 inhibitor quinacrine [4] on the sphingo-myelinase-elicited effect (Fig 3) Quinacrine alone (25 lM) did not alter the14C radioactivity from [1-14C]arachidonic acid in the Etn plasmalogens, but, in the presence of sphingomyelinase, it not only prevented the decrease caused

by sphingomyelinase, but also evoked a significant (P < 0.05) increase in the14C radioactivity found in Etn plasmalogens

This led us to hypothesize that the target enzyme for Cer action may be the 39 kDa plasmalogen-selective PLA2 described and characterized previously [3,5,12] The enzyme

is specifically and markedly inhibited by sialic acid, glucos-aminoglucans, gangliosides and sialoglycoproteins [3,5] In contrast, the brain 110 kDa cytosolic PLA2, acting prefer-entially on PtdEtn, has been reported to be much less sensitive to these inhibitory effects [3,5,12] These differences

in behavior prompted us to test the effect of sphingomye-linase on slices pretreated with a brain ganglioside mixture The ganglioside mixture did not itself evoke significant variation in either14C radioactivity or levels of PtdEtn, but did prevent the decrease in radioactivity in, and levels of, Etn plasmalogens caused by sphingomyelinase (Fig 3A,B)

We also tested the effect of bromoenol lactone, a specific and potent inhibitor of myocardial Ca2+-independent plasmalogen-specific PLA2 [28] devoid of effect on the brain plasmalogen-selective PLA2[4,9] Pretreatment with bromoenol lactone did not block the effect of sphingo-myelinase on Etn plasmalogen levels (Fig 3B) Therefore, our results are in agreement with those reported for the brain enzyme [4,9]

A first attempt was made to establish whether the sphingomyelinase-sensitive PLA2 acting on Etn plasmalo-gens also shows CoA-independent transacylase activity For this, we used ET-18-OCH3, a specific inhibitor [29] ET-18-OCH3(25 lM) itself did not modify either14C radioactivity

in, or levels of, Etn plasmalogens (Fig 3A,B, respectively) However, when ET-18-OCH3was added before sphingo-myelinase, the effect of sphingomyelinase on the Etn plasmalogens was prevented (Fig 3A,B) In agreement with our previous report [2], we first observed a significant (P < 0.05) sphingomyelinase-elicited increased production

of 1-O-[1-14C]acylCer (Table 4), which can be used as an index of transacylase activity [16] It is also evident that an increase in the level of14C radioactivity in the nonesterified fatty acid fraction was concomitantly evoked by sphingo-myelinase Interestingly, the ganglioside mixture (0.26 gÆL)1) and ET-18-OCH3(25 lM) both completely prevented both these sphingomyelinase-evoked effects

Mechanism by which Cer decreases Etn plasmalogens levels

Cer has been reported to activate okadaic acid-sensitive protein phosphatase 2A To test whether this protein phosphatase is involved in the Cer effect, we treated brain

Table 2 Variations in [14C]arachidonic acid-labeled Etn phospholipid

subclasses evoked by sphingomyelinase and C 2 -Cer Slices were labeled

with 0.2 lCiÆmL)1[ 14 C]arachidonic acid for 120 min After removal of

the labeled precursor, slices were exposed to 0.38 UÆmL)1

sphingo-myelinase or 100 l M C 2 -Cer for 30 min Total Etn phospholipids were

hydrolyzed with phospholipase C The resulting diacylglycerols were

extracted and the acetylated derivatives were prepared After their

fractionation by TLC, the radioactivity in each was measured Data

are expressed as radioactivity incorporated (d.p.m.) in each subclass

per mg of total lipids They represent mean ± SD from one

repre-sentative experiment of two experiments performed in triplicate.

Treatment

Radioactivity incorporated into Etn phospholipid subclasses Alkenylacyl Alkylacyl Diacyl

Sphingomyelinase 38.8 ± 3.2* 43.8 ± 3.6 39.4 ± 5.1*

* P < 0.05 compared with their respective controls.

Table 3 Variations in the extracellular and cell-associated radioactivity

from [ 14 C]arachidonic acid evoked by sphingomyelinase and C 2 -Cer.

Slices were labeled with 0.2 lCiÆmL)1[14C]arachidonic acid (AA) for

120 min After removal of the labeled precursor, slices were exposed to

0.38 UÆmL)1sphingomyelinase or 100 l M C 2 -Cer for 30 min Aliquots

(50 lL) from the incubation medium were taken for radioactivity

measurement Tissue total lipids were extracted, dissolved, and

aliqu-ots (10 lL) were taken for radioactivity measurement Extracellular

arachidonic acid is expressed as d.p.m per aliquot and cell-associated

arachidonic acid as d.p.m per mg total lipids Data represent

mean ± SD from one representative experiment of two experiments

performed in triplicate.

Treatment

Radioactivity incorporated Extracellular AA Cell-associated AA

* P < 0.05 compared with their respective controls.

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slices with okadaic acid (2.5 and 25 nM) before

sphingo-myelinase treatment (Fig 4) Okadaic acid alone produced

no change in Etn plasmalogen levels (Fig 4A) but did

prevent the effect of sphingomyelinase treatment on Etn

plasmalogen levels

Data on Cer levels are shown in Fig 4B

Sphingomye-linase increased the level of endogenous Cer by nearly 100%

(P < 0.05) Okadaic acid by itself did not alter Cer levels

However, when slices were pretreated with okadaic acid, the

sphingomyelinase-elicited increase was prevented

There-fore, okadaic acid was acting as a modulator of Cer

metabolism but not of the Cer-evoked effect

On the other hand, Cer has been reported to induce

mitogen-activated protein kinase (MAPK) activity, which in

turn phosphorylates and activates cytosolic PLA2 [1]

PD 98059 has been widely used as a specific inhibitor to

study whether p42/p44 MAPK is downstream of Cer

generation Experiments with PD 98059 were therefore

performed (Fig 5) At concentrations ranging from 10–

100 lM, PD 98059 significantly (P < 0.05) increased Cer

levels in a dose-dependent manner (Fig 5B)

Concomit-antly, Etn plasmalogen levels decreased by about 75%,

in a dose-independent manner (Fig 5A) Unexpectedly,

PD 98059 was able to prevent the sphingomyelinase-elicited

increase in Cer levels (Fig 5B) and partially reverse the

Cer-evoked reduction in Etn plasmalogen levels (Fig 5A)

It has been shown that specific protease activation is a

pivotal element in Cer-regulated processes Thus, Cer acts

downstream of caspase-8 but upstream of caspase-3 [27] In

addition, a serine proteolytic enzyme is also a Cer target

[30] Therefore, iodoacetamide (as a thiol-specific inhibitor) and phenylmethanesulfonyl fluoride (as a blocking agent of serine enzymes) were tested (Fig 6) Neither iodoacetamide nor phenylmethanesulfonyl fluoride by themselves affected basal Cer (Fig 6B) or Etn plasmalogen (Fig 6A) levels However, both inhibitors were able to prevent the Cer effect

on Etn plasmalogen levels (Fig 6A) without modifying the enhanced Cer levels (Fig 6B)

In view of these results, we next explored whether caspase-3 is involved in the regulation of plasmalogen-selective PLA2 Experiments with the cell-permeable caspase-3-specific tetrapeptide inhibitor Ac-DEVD-CMK were performed The Ac-DEVD-CMK concentration used has been shown to inhibit apoptosis induced by 30 lM

C2-Cer and caspase-3 activity [27] The results obtained are shown in Table 5 The caspase-3 inhibitor by itself did not produce any effect, but partially prevented the sphingo-myelinase-elicited decrease in Etn plasmalogen levels with-out affecting Cer levels

Etn plasmalogen hydrolysis can also be elicited

by endogenous agonists

We have previously reported that the neuropeptide endo-thelin-1 is able to evoke Cer production in cerebral cortex [18] Therefore, we next hypothesized that Etn plasmalogen hydrolysis may occur concomitantly with endogenous Cer production evoked by a natural agonist Table 6 shows that treatment with 0.1 lMendothelin-1 for 30 min (conditions under which maximum Cer production is evoked by

Fig 2 Variations in breakdown products of Etn phospholipid evoked by sphingomyelinase (SMase) and C 2 -ceramide (A) Sphingomyelinase-evoked variations in [14C]Etn-labeled water-soluble metabolites; (B) sphingomyelinase-evoked variations in levels of total diacylglycerols; (C) sphingo-myelinase-evoked variations in [ 14 C]Etn radioactivity (left axis; open bars) and in levels of 1-O-alkenyl-2-lyso-GroPEtn (right axis; filled bars); (D)

C 2 -ceramide-evoked variation in [ 14 C]Etn radioactivity (left axis; open bars) and in levels of 1-O-alkenyl-2-lyso- GroPEtn (right axis; filled bars) Cerebral cortex slices were prelabeled with 2 lCiÆmL)1[14C]Etn for 30 min (A, C and D) and then exposed to either 0.38 UÆmL)1sphingomyelinase

or different C 2 -ceramide concentrations for 30 min Levels of, and the radioactivity in, the metabolites were determined Data represent mean ± SE from two separate experiments performed in quintuplicate Values significantly different from their respective controls are indicated: *P < 0.05.

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endothelin-1) resulted in a significant (P < 0.05) decrease

( 35%) in Etn plasmalogen levels, concomitantly with an

increase of 60% in the Cer level

Discussion

Involvement of the brain plasmalogen-selective PLA2

in the Cer-elicited decrease in Etn plasmalogen levels

We have previously shown that sphingomyelinase decreases

the levels of brain Etn plasmalogens [2] To rule out the

possibility that Etn plasmalogens are directly hydrolyzed by sphingomyelinase, experiments with C2-Cer were per-formed The effect of sphingomyelinase on Etn plasmalogen levels was mimicked by C2-Cer Although several differen-tial effects of sphingomyelinase and Cer analogs have been described [26], we conclude that the decrease in Etn plasmalogen levels is a response, at least in part, to endogenous Cer accumulation

The complete prevention of the Cer effect caused by quinacrine and gangliosides, combined with the lack of effect exhibited by bromoenol lactone, led us to think that the enzyme involved is the 39 kDa plasmalogen-selective PLA2 [4,9,12] That other Etn phospholipids besides plasmalogens are affected is consistent with the specificity shown by the brain 39 kDa plasmalogen-selective PLA2 [12] In addition, we also observed that there was no loss of sphingomyelinase-elicited extracellular arachidonate or its derivatives, which precludes the involvement of a secretory PLA2

Two additional findings are noteworthy First, the arachi-donate-rich pool of Etn plasmalogens is appreciably affected

by Cer (Tables 1 and 2) The docosahexaenoate-rich pool of Etn plasmalogens is the other major pool of brain Etn plasmalogens [7] and therefore it would be interesting to study

it further Secondly, plasmalogen hydrolysis by PLA2 is coupled with CoA-independent transacylase activity, as these processes are blocked in parallel by inhibitors of each (gangliosides and ET-18-OCH3) This coupling has also been observed in other PLA2enzymes acting on alkenylacylglyc-erophospholipids of Etn, such as the 14 kDa PLA2present in monocytes [31], or on diacylglycerophospholipids of Etn, such as the 40 kDa PLA2 of brain [16] In fact, the latter enzyme is a single polypeptide chain with a molecular mass of

 40 kDa, similar to that of the plasmalogen-selective PLA2 described previously [12]

An interesting picture begins to emerge from the present evidence However, it is necessary to consider several facts

Fig 3 Effect of quinacrine (Q), ganglioside mixture (G), ET-18-OCH 3

(E) and bromoenol lactone (B) on sphingomyelinase (SMase)-induced

alterations in brain Etn plasmalogens (PlsEtn) (A) Radioactivity from

[ 14 C]arachidonic acid in Etn plasmalogens is expressed as the

per-centage of radioactivity incorporated into these phospholipids with

respect to that incorporated into total lipids (B) Levels of Etn

plas-malogens Cerebral cortex slices were labeled with 0.2 lCiÆmL)1

[14C]arachidonic acid for 120 min After removal of the labeled

pre-cursor, slices were incubated with 250 l M quinacrine for 25 min,

0.26 gÆL)1ganglioside mixture for 2 min, or 10 l M bromoenol lactone

for 10 min Cerebral cortex homogenates were labeled as described

above and exposed to 25 l M ET-18-OCH 3 for 2 min They were then

treated with 0.38 UÆmL)1sphingomyelinase for 30 min Respective

controls were performed by incubating slices or homogenates in the

presence of the respective solvents Radioactivity and/or levels of Etn

plasmalogens were measured Data represent mean ± SE and are

from two experiments performed in quintuplicate Values significantly

different from the control are indicated: *P < 0.05.

Table 4 Effect of plasmalogen-selective PLA 2 and CoA-independent transacylase inhibitors on the formation of 1-O-acylCer and release of free arachidonic acid evoked by sphingomyelinase Slices were labeled with 0.2 lCiÆmL)1[14C]arachidonic acid for 120 min After removal of the labeled precursor, slices were incubated with 0.26 gÆL)1ganglioside mixture (G) for 2 min or with 25 l M ET-18-OCH 3 (E) for 2 min Then, 0.38 UÆmL)1sphingomyelinase was added for 30 min Total lipids were fractionated by TLC, and the radioactivity in 1-O-acylCer and nonesterified fatty acid fractions was measured Data are expressed as radioactivity incorporated (d.p.m.) in each fraction per

mg total lipids Data represent mean ± SD from one representative experiment of two experiments performed in quintuplicate.

Treatment

Radioactivity incorporated 1-O-AcylCer Nonesterified fatty acid

G + sphingomyelinase 383 ± 200 6354 ± 503

* P < 0.05 compared with their respective controls.

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First, in the plasma membrane of eukaryotic cells, the

Etn-containing phospholipids reside in the inner leaflet

whereas sphingomyelin is located in the outer leaflet There

is evidence that, during the early stages of apoptosis, this

asymmetric distribution is lost, resulting in exposure of

PtdEtn on the cell surface [32] Thus, the potential activation

of a membrane-associated neutral sphingomyelinase by

apoptosis inducers would generate Cers that initiate a

cascade of events, including the hydrolysis of Etn

plasma-logens, which may be suitably positioned by a previous

traslocation event

Secondly, Cer has been shown to be involved in oxidative

stress through the production of mitochondrial oxygen-free

radicals [33] On the other hand, Etn plasmalogens are

antioxidant molecules that protect cells from oxidative stress

[6] Cer-elicited hydrolysis of Etn plasmalogens could

produce an increase in susceptibility to oxidative agents,

leading to apoptosis Thus, our data may indicate a new role

for Cer in apoptosis

Caspase-3 is involved in the Cer-elicited decrease

of Etn plasmalogen levels

It has been previously reported that Cer does not affect purified plasmalogen-selective PLA2 [12] Therefore, our next experiments were designed to identify enzyme(s) downstream of Cer capable of regulating plasmalogen PLA2

We unexpectedly found that okadaic acid and PD 98059, used as inhibitors of protein phosphatase and MAPK, respectively, were able to modify the sphingomyelinase-enhanced or basal endogenous Cer levels Consistent with this, complex modulation of Cer levels evoked by okadaic acid has been reported [34] Although we cannot rule out the possibility that okadaic acid itself affects sphingomyelinase,

it is very likely that the metabolic fate of Cer is affected As the okadaic acid effect is evoked by concentrations as low as 2.5 nM, a protein phosphatase 2A may regulate Cer metabolism

Fig 4 Effect of okadaic acid on basal and sphingomyelinase-altered Etn

plasmalogen levels and ceramide accumulation (A) Etn plasmalogen

(PlsEtn) levels; (B) ceramide levels Cerebral cortex slices were

incu-bated in the absence or presence of 2.5 or 25 n M okadaic acid (OKA)

for 10 min and then exposed to 0.38 UÆmL)1 sphingomyelinase

(SMase) for 30 min Etn plasmalogen and ceramide levels were

obtained from the same tissue sample Data represent mean ± SE

from three experiments performed in quintuplicate Values

signifi-cantly different from the control are indicated: *P < 0.05.

Fig 5 PD 98059-evoked effect on basal and sphingomyelinase-altered Etn plasmalogens levels and ceramide accumulation (A) Etn plasma-logen (PlsEtn) levels; (B) ceramide levels Cerebral cortical slices were incubated in the absence or presence of several concentrations of

PD 98059 for 30 min and then exposed to 0.38 UÆmL)1 sphingo-myelinase (SMase) for 30 min Etn plasmalogen and ceramide levels were obtained from the same tissue sample Data represent mean ± SE from three experiments performed in quintuplicate Val-ues significantly different from control are indicated: *P < 0.05.

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The effects of PD 98059 are even more complex, as the

inhibitor increased the basal levels of Cer but prevented the

sphingomyelinase-induced increase This may be explained

in terms of some MAPK members being upstream and/or

downstream of Cer generation As the decrease in Etn

plasmalogens was still observed in its presence, it is likely

that a Cer metabolite is also regulating the

plasmalogen-selective PLA2 Studies are currently being carried out to

clarify this point

Despite the complex mechanism of action of PD 98059, it

is clear that the Cer-elicited activation of

plasmalogen-selective PLA2is not mediated by the activation of p42/p44

MAPK This is a characteristic that is not shared by the

cytosolic PLA2from many cell types, including that from

rat cerebral cortex [35] Nevertheless, the possibility of the

involvement of p38 MAPK remains open, as it is also a Cer

target [1], and the regulation of cytosolic PLA by this

MAPK subfamily has been reported in other biological systems [36]

The potential involvement of some proteolytic step in the regulation of plasmalogen PLA2 by Cer was also tested First, we studied the action of thiol protease and serine protease inhibitors Neither class of inhibitors was able to modify both basal and sphingomyelinase-enhanced Cer levels, but they did prevent the Cer-elicited lowering effect

on Etn plasmalogen levels One possible explanation is that there are proteases (or other enzymes) that contain serine or cysteine in their active center downstream of Cer that mediate the activation of the plasmalogen PLA2 This hypothesis is supported by evidence on the regulation of other types of PLA2 by proteolytic cleavage phenomena [37] Alternatively, it is possible that serine and cysteine residues are functionally important and/or are present in the catalytic site of the plasmalogen-selective PLA2 Consistent with this, it is well known that many esterases, including PLA2, are sensitive to the action of iodoacetate and phenylmethanesulfonyl fluoride Furthermore, in studies with the purified plasmalogen-selective PLA2, preliminary evidence on its sensitivity to iodoacetate has been reported [3] In contrast, it has been reported that any serine residue is essential for the transacylase reaction of the 40 kDa brain

Fig 6 Preventive effect of iodoacetamide and phenylmethanesulfonyl

fluoride on the sphingomyelinase-elicited alterations in Etn plasmalogen

levels and ceramide accumulation (A) Etn plasmalogen (PlsEtn) levels;

(B) ceramide levels Cerebral cortex slices were pretreated with 10 m M

iodoacetamide (I) or 2 m M phenylmethanesulfonyl fluoride (PMSF)

for 60 min and then exposed to 0.38 UÆmL)1 sphingomyelinase

(SMase) for 30 min Etn plasmalogen and ceramide levels were

obtained from the same sample Data represent mean ± SE from two

experiments performed in quintuplicate Values significantly different

from the control are indicated: *P < 0.05.

Table 5 Effect of caspase-3 inhibitor on Etn plasmalogen hydrolysis and Cer accumulation evoked by sphingomyelinase Cerebral cortex slices were preincubated with 50 l M Ac-DEVD-CMK for 60 min, then treated with 0.38 UÆmL)1sphingomyelinase for 30 min Total lipids were split into three aliquots: one was untreated, another was exposed

to HCl fumes, and the other was hydrolyzed by alkali They were fractionated by TLC, and the levels of Etn plasmalogens and Cer were measured Data are expressed as nmol each fraction per mg total lipids They represent mean ± SD from one representative experiment of two experiments performed in quintuplicate.

Treatment

Lipid fraction level

Ac-DEVD-CMK+

sphingomyelinase

* P < 0.05 compared with their respective controls.

Table 6 Effect of endothelin-1 (ET-1) on brain Etn plasmalogen and Cer levels Slices were incubated with 0.1 l M endothelin-1 for 30 min After total lipid extraction, Etn plasmalogen and Cer levels were measured Data are expressed as nmol per mg total lipids They are mean ± SD from one experiment performed in triplicate.

Treatment

Lipid fraction level

* Significantly different (P < 0.05) from the control value.

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transacylase These contradictory observations remain to be

clarified

Of more interest was the fact that the caspase-3-like

protease-specific inhibitor Ac-DEVD-CMK could partially

abolish Cer-elicited Etn plasmalogen hydrolysis without

altering sphingomyelinase-elicited Cer accumulation

Sev-eral PLA2 enzymes are substrates for caspase-3, but,

depending on the type of PLA2, this cleavage leads to their

inactivation (in the case of the cytosolic PLA2, type IV,

without arachidonate-phospholipid remodeling activity) or

activation (in the case of the Ca2+-independent PLA2, type

VI, with arachidonate-phospholipid remodeling activity)

[37] As the brain plasmalogen-selective PLA2 is Ca2+

-independent [4], and has been shown here to have

CoA-independent transacylase activity, its potential activation by

caspase-3 is consistent with the available evidence

Further-more, it has been suggested that activation of hydrolysis of

Etn phospholipids by PLA2 results from the covalent

modification of the enzyme [38]

In an attempt to establish the potential

pathophysiolo-gical significance of the present findings, we made a

preliminary study to determine whether a natural

Cer-generating agonist, such as the neuropeptide

endothe-lin-1 [18], can modify Etn plasmalogen levels in brain

tissue The positive evidence obtained suggests that the

present findings can be extrapolated to in vivo conditions

Further studies in this field are currently being performed

in our laboratory

We may tentatively conclude that the findings reported

here are relevant to the knowledge of some processes in

Alzheimer’s disease and cerebral ischemia, despite the fact

that some aspects still remain unclear

Activated caspase-3 has been in situ-immunodetected in

only a small subpopulation of hippocampal neurons, but

not in the cortex in patients with Alzheimer’s disease [39] In

addition, amyloid beta peptide can activate caspase-3 and

induce neuronal apoptosis in vitro [39] Furthermore, it has

been suggested [9] that stimulation of the Ca2+-independent

plasmalogen-selective PLA2may account for the decreased

levels of Etn plasmalogens found in the affected regions of

the brain in Alzheimer’s disease, such as the cerebral cortex

It is feasible that an early and reversible activation of

caspase-3 by endogenous Cer may be sufficient to produce

an irreversible loss of Etn plasmalogens

The picture in other pathological states is clearer Brain

sections from patients with neuropathological evidence of

apoptosis secondary to stroke, seizure or trauma exhibit

neuronal-activated caspase-3 immunoreactivity [39]

Acti-vation of a brain Ca2+-independent PLA2acting on PtdEtn

[38] and a decrease in Etn plasmalogen levels [6,40] have

been reported to occur in ischemia

It is noteworthy that other Ca2+-independent PLA2

enzymes acting on plasmalogens from non-neural sources

have been described [3] Whether these are also regulated by

Cer remains an open question; the answer may provide

evidence for cross-talk phenomena between the

sphingo-myelin cycle and PLA2-mediated arachidonate metabolism

[30]

In summary, our data show, for the first time, that brain

Ethanolamine plasmalogen hydrolysis is regulated by the

endogenous level of Cer, and a caspase-3-like protease is a

downstream Cer effector

Acknowledgements

We are indebted to Mrs M V Mora Gil and Mrs Belinda Benhamu´ This work was funded by grants from the DGICYT and the Fundacio´n

‘Ramo´n Areces’.

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