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Indeed, in intact cells J774 macrophages, HeLa cells and AG1518 fibroblasts the lysosomotropic detergent O-methyl-serine dodecylamide hydrochloride MSDH causes lysosomal rupture, enhanced

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Lysosomal enzymes promote mitochondrial oxidant production,

Ming Zhao1, Fernando Antunes1,2, John W Eaton1,3and Ulf T Brunk1

1

Division of Pathology II, Faculty of Health Sciences, Linko¨ping University, Sweden;2Grupo de Bioquı´mica e Biologia Teo´ricas – Instituto Bento da Rocha Cabral and Department of Chemistry and Biochemistry, Faculty of Sciences, University of Lisbon, Portugal;

3

James Graham Brown Cancer Center, University of Louisville, Louisville, KY, USA

Exposure of mammalian cells to oxidant stress causes early

(iron catalysed) lysosomal rupture followed by apoptosis

or necrosis Enhanced intracellular production of reactive

oxygen species (ROS), presumably of mitochondrial origin,

is also observed when cells are exposed to nonoxidant

pro-apoptotic agonists of cell death We hypothesized that ROS

generation in this latter case might promote the apoptotic

cascade and could arise from effects of released lysosomal

materials on mitochondria Indeed, in intact cells (J774

macrophages, HeLa cells and AG1518 fibroblasts) the

lysosomotropic detergent O-methyl-serine dodecylamide

hydrochloride (MSDH) causes lysosomal rupture, enhanced

intracellular ROSproduction, and apoptosis Furthermore,

in mixtures of rat liver lysosomes and mitochondria, selective

rupture of lysosomes by MSDH promotes mitochondrial

ROSproduction and cytochrome c release, whereas MSDH

has no direct effect on ROSgeneration by purifed

mito-chondria Intracellular lysosomal rupture is associated with the release of (among other constituents) cathepsins and activation of phospholipase A2 (PLA2) We find that addi-tion of purified cathepsins B or D, or of PLA2, causes substantial increases in ROSgeneration by purified mito-chondria Furthermore, PLA2) but not cathepsins B or

D) causes rupture of semipurified lysosomes, suggesting an amplification mechanism Thus, initiation of the apoptotic cascade by nonoxidant agonists may involve early release of lysosomal constituents (such as cathepsins B and D) and activation of PLA2, leading to enhanced mitochondrial oxidant production, further lysosomal rupture and, finally, mitochondrial cytochrome c release Nonoxidant agonists

of apoptosis may, thus, act through oxidant mechanisms Keywords: apoptosis; cathepsins; lysosomes; lysosomotropic detergents; oxidative stress

In the last two decades, the phenomenon of apoptosis has

attracted great interest and many intricate molecular events

underlying the process have been elucidated [1–8] Several

crucial steps are thought to involve mitochondrial release

of pro-apoptotic factors, although the exact mechanisms

involved in this release are less well understood

In this regard, there is substantial evidence that, at least

in some circumstances, the discharge into the cytosol of

lysosomal constituents may be an early and, perhaps,

initiating event in apoptosis, and that mitochondrial release

of pro-apoptotic factors might be a consequence of earlier

lysosomal destabilization [9–18] In further, albeit indirect,

support of this, it was recently found that activation of the

pro-apoptotic tumour supressor protein, p53, also results

in early lysosomal rupture, although through still unknown mechanisms [14]

In the case of simple oxidant-induced apoptosis, lyso-somal rupture occurs in two sequential phases [19,20], where the second one includes activation of phospholipase A2 (PLA2) with production of free arachidonic acid (AA) [21,22] Theoretically, released lysosomal enzymes, PLA2, and AA all might be capable of destabilizing mitochondrial membranes Interestingly, over-expression of the anti-apoptotic protein, Bcl-2, abrogates the secondary phase of lysosomal rupture, the activation of PLA2, and the mitochondrial release of cytochrome c [19,21,22] However, the precise mechanisms through which Bcl-2 mediates these effects are presently unknown

Remarkably, in apoptosis caused by a number of nonoxidative agents, there appears to be increased intracel-lular generation of reactive oxygen species (ROS), probably

of mitochondrial origin [23–30] Although the mechanisms responsible for enhanced mitochondrial ROSproduction during the process of apoptosis remain unknown, this phenomenon raises the possibility that internally generated ROS, like exogenously added oxidants, may act through a common pathway–lysosomal destabilization

The present investigations were aimed at identifying intracellular events that might connect exposure of cells to nonoxidative agonists of apoptosis and intracellular ROS production As mentioned above, there is abundant evi-dence that) at least in some circumstances ) lysosomal rupture might be an early, perhaps even initiating, event in

Correspondence to M Zhao, Division of Pathology II,

Faculty of Health Sciences, Linko¨ping University,

SE-581 85 Linko¨ping, Sweden.

Fax: +46 13 22 15 29, Tel.: +46 13 22 15 15,

E-mail: ming.zhao@inr.liu.se

Abbreviations: AA, arachidonic acid; DHE, dihydroethidium; HRP,

horseradish peroxidase; LE, lysosomal enzymes; LEF,

lysosome-mitochondria enriched fraction; MSDH, O-methyl-serine

dodecylamide hydrochloride; PLA2, phospholipase A2;

ROS, reactive oxygen species.

(Received 28 April 2003, revised 11 July 2003,

accepted 24 July 2003)

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the apoptotic cascade Therefore, in the present

investiga-tions we have used a synthetic lysosomotropic detergent,

O-methyl-serine dodecylamide hydrochloride (MSDH) to

specifically induce lysosomal rupture and ensuing apoptosis

[12,31,32] This was done in order to determine whether

internal oxidative stress of mitochondrial origin might arise

as a consequence of lysosomal rupture and act as an

amplifying loop causing further lysosomal breach Here, we

present evidence that released lysosomal enzymes) both

directly and through activation of PLA2) may trigger

enhanced mitochondrial production of superoxide and

hydrogen peroxide, and cause the release of cytochrome c

Materials and methods

Materials

Chemicals were from Sigma unless stated otherwise RPMI

1640 medium, Hepes, foetal bovine serum, glutamine,

penicillin, and streptomycin were from Gibco BODIPY

FL phallacidin and dihydroethidium (DHE) were from

Molecular Probes Monoclonal anti-cytochrome c Igs were

from Pharmingen, and horseradish peroxidase

(HRP)-conjugated goat anti-mouse Igs were from DAKO Percoll

was from Amersham Pharmacia Biotech

Cell cultures

Human foreskin fibroblasts (AG-1518, passages 14–20;

Coriell Institute, Camden, NJ, USA), J774 cells (a murine

histiocytic lymphoma cell line), and human epithelial cells

(HeLa) were cultured at 37C in humidified air with 5%

CO2 in RPMI 1640 medium supplemented with 2 mM

glutamine, 50 IUÆmL)1 penicillin-G, 50 lgÆmL)1

strepto-mycin, and 10% foetal bovine serum Cells were

subcul-tured once a week Twenty-four hours before experiments,

cells were trypsinized and seeded into 35-mm Petri dishes or

96-well plates (Costar, Cambridge, MA, USA) at a density

of 10 000 cells per cm2

Apoptosis assays

DNA fragmentation was assessed using propidium iodide

staining of nuclear DNA, essentially as described by

Nicoletti et al [33] Briefly, cell pellets from individual

wells were gently resuspended in 1.5 mL of a hypotonic

and membrane-disrupting solution of propidium iodide

(50 lgÆmL)1 in 0.1% sodium citrate with 0.1% Triton

X-100) in 12· 75 mm polypropylene tubes The tubes were

kept overnight in the dark at 4C before flow-cytometric

analyses The propidium iodide-induced red fluorescence of

suspended individual nuclei was measured by flow

cyto-fluorometry, using the FL3 channel Nuclei with partly

degraded DNA were counted, and their frequency was

expressed as a percentage of the total number of nuclei

analysed in at least 10 000 cells

Actin staining

AG1518 fibroblasts were seeded in 35-mm Petri dishes and

cultured for 24 h before being exposed to 30 lMMSDH in

ordinary medium for 3 h Cellular actin was stained with

BODIPY FL phallacidin Cells were fixed for 10 min in 4% formaldehyde in NaCl/Pi, permeabilized for 10 min in 0.3% Triton X-100 in phosphate-buffered saline (NaCl/Pi), and stained for 30 min with BODIPY FL phallacidin (final concentration 0.6 lgÆmL)1) at 37C After staining, cells were washed twice in NaCl/Pi, and visualized and documented (kEX 495 nm; kEM 520 nm) using a Nikon microphot-SA fluorescence microscope with a Hamamatsu ORCA-100 color digital camera and Adobe PHOTOSHOP software

Evaluation of oxidative stress AG1518 fibroblasts, J774 and HeLa cells were seeded in 96-well plates and cultured for 24 h under standard conditions before being exposed to 30 lM MSDH and

10 lMDHE (in complete medium) Fluorescence intensity, indicating oxidation of DHE was assayed at various periods

of time after addition of MSDH and DHE on a VICTOR

1420 (Wallac Sverige AB, Upplands Va¨sby, Sweden) fluorescent plate-reader (kEX 485 nm; kEM 620 nm) In some experiments, cells were observed and documented under green light excitation (kEX 546 nm; kEM 590 nm) using fluorescence microscopy as described above

Preparation of rat liver lysosome-mitochondria enriched fraction

Livers were removed from 160–200-g female Sprague– Dawley rats (starved overnight), homogenized in 0.3M sucrose (1 : 9, w/v) and centrifuged at 500 g for 10 min The supernatants were again centrifuged at 3500 g for

10 min, the pellets discarded, and the lysosome/mitochon-dria-containing supernatants centrifuged at 10 000 g for

10 min The pellets were washed, suspended and re-centri-fuged at 10 000 g for 10 min and finally resuspended in the sucrose solution to a protein concentration of

 1.5 mgÆmL)1 The resultant lysosome/mitochondria enriched fraction (LEF) was found to be stable (no release

of lysosomal enzymes) for up to 4 h in the homogenization solution at 4C, while some release of lysosomal enzymes occurred within 2 h at 37C

Preparation of a purified mitochondria fraction Mitochondria were purified from rat liver using a combi-nation of differential and Percoll gradient centrifugation [34,35] All procedures were carried out at 4C Briefly, fresh liver was minced and homogenized in M-SHE buffer (0.21M mannitol, 0.07M sucrose, 10 mM Hepes pH 7.4,

1 mM EDTA, 1 mM EGTA, 0.15 mMspermine, 0.75 mM spermidine) Unbroken cells and nuclei were pelleted at

500 g for 10 min The supernatant was centrifuged at

10 000 g to pellet mitochondria and lysosomes which were resuspended and washed twice with M-SHE buffer A 2-mL suspension was then layered onto 37.5 mL of Percoll solution (50% Percoll, 50% 2· M-SHE) and centrifuged for 1 h at 50 000 g in a Ti-60 rotor The brown mitochondrial band was collected, either by fractionating the gradient or by direct syringe aspiration The purified mitochondria were pooled, diluted 10-fold with M-SHE buffer, again pelleted by centrifugation and, finally,

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resuspended in M-SHE buffer to a protein concentration

of 1.5 mgÆmL)1 The degree of lysosomal contamination

of the purified mitochondria fraction was estimated by

assaying b-galactosidase/protein and compared to that

of LEF

Enzymatic detection of lysosomal integrity

and estimation of fraction purity

The integrity of lysosomes in the LEF preparation was

assessed by assaying released b-galactosidase LEF

(200 lL) was incubated for 3 h at 37C with either

PLA2 (0.2 UÆmL)1), 30 lM MSDH, 2.5 lgÆmL)1

cathep-sin B, or 2.5 lgÆmL)1cathepsin D and then centrifuged at

14 000 g for 10 min Stock solutions of the cathepsins were

made up in NaCl/PipH 6.0, whereas MSDH and PLA2

were in NaCl/PipH 7.4 The supernatants were removed,

and 1 mL distilled water with Triton X-100 (final

concen-tration 0.1%) was added to the pellets to cause complete

lysis of remaining intact lysosomes Activities of

b-galac-tosidase were measured as described previously [22] on the

ruptured lysosomal pellet and on the supernatant The

results were expressed as percentage released over total

b-galactosidase

Mitochondrial generation of H2O2 Mitochondrial production of H2O2was assayed essentially

as described elsewhere [36] Briefly, 1.33 UÆmL)1 HRP, 0.066 mgÆmL)1 q-hydroxyphenylacetate, 0.013 mgÆmL)1 superoxide dismutase, and 1 mg mitochondrial protein were added to 2.4 mL respiratory buffer (0.07M sucrose, 0.23M mannitol, 30 mM Tris/HCl, 4 mM MgCl2, 5 mM

KH2PO4, 1 mMEDTA, 0.5% BSA, pH 7.4) in a spectro-fluorophotometer cuvette at 37C Succinate (final concen-tration 6.67 mM) and antimycin A (final concentration 0.83 lgÆmL)1) were added, and H2O2-induced fluorescence recorded (kEX320 nm; kEM400 nm) during the first 10 min after mixing

Western blotting for cytochromec Two-hundred microlitres LEF, or purified mitochondria, were incubated for 3 h at 37C with either 30 lM MSDH, PLA2 (0.2 UÆmL)1), 2.5 lgÆmL)1cathepsin B, or 2.5 lgÆmL)1cathepsin D Stock solutions of the cathepsins were made up in NaCl/PipH 6.0, while MSDH and PLA2 were in NaCl/Pi pH 7.4 Following centrifugation at

14 000 g for 10 min, the supernatants were separated by

Fig 1 MSDH induces apoptosis and stress fibre formation in fibroblasts (A) Cells were seeded into 35-mm Petri dishes at a density of

10 000 cellsÆcm)2 After 24 h, 30 l M MSDH was added to complete culture medium (2 mL), and cells were incubated for another

10 h under standard culture conditions The Nicoletti technique for apoptotic nuclei was applied One representative experiment out of three is shown (B) Cells were seeded in 35-mm Petri dishes and kept for 24 h before being exposed to 30 l M MSDH for 3 h Actin staining was then performed as described in Materials and methods.

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SDS/PAGE (12% acrylamide) and transferred onto

Immo-bilon membranes (2 h; 200 mA) Membranes then were

incubated at room temperature for 1 h in blocking buffer

[5% low-fat milk powder in Tris-buffered saline (TBS)] and

for another 2 h in dilution buffer (0.5% low-fat milk

powder in TBS) containing a 1 : 400 dilution of a

mono-clonal anti-cytochrome c Ig After washing in TBSwith

0.06% Tween 20, Immobilon membranes were incubated

for 1 h at room temperature in a buffer containing a

1 : 1500 dilution of peroxidase-conjugated secondary

antibodies After washing, peroxidase-dependent chemilu-minescence was detected by using enhanced chemilumines-cence Western blotting reagents and hyperfilm according to the manufacturer’s instructions (Amersham Pharmacia Biotech)

Statistical analysis All experiments were repeated at least three times Values are given as arithmetic mean ± SD Significance

Fig 2 MSDH induces intracellular ROS production Cells were seeded into 96-well plates at a density of 10 000 cellsÆcm)2 After 24 h, cells were exposed simultaneously to 30 l M MSDH and 10 l M DHE under otherwise standard culture conditions while control cells were exposed to DHE only (A) Fluorescence intensity arising from oxidized dihydroethidium in J774, HeLa and AG1518 cells was measured at indicated time points (B) J774 cells were visualized and photographed after 3 h exposure to MSDH (n ¼ 3) Very similar results were obtained with HeLa and AG1518 cells under the same conditions although detectable oxidant generation occurred earlier.

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of differences between groups was determined using

Student’s two-tailed t-test *P£ 0.05; **P£ 0.01;

***P£ 0.001

Results

Cultured cells exposed to the synthetic lysosomotropic

detergent, MSDH, undergo lysosomal rupture and ensuing

apoptosis or necrosis depending upon the extent of

lysosomal destabilization [12] In the present experiments,

we induced apoptosis in fibroblasts, J774 cells, and HeLa cells by exposing them to 30 lM MSDH After 8 h of MSDH exposure, nuclear propidium iodide staining and flow cytometry (to detect DNA fragmentation) revealed apoptotic nuclei appearing as a broad, hypodiploid DNA smear in front of a narrow peak of diploid DNA (Fig 1A shows results in fibroblasts) At an early stage in this process, well before the appearance of frank apoptosis, fibroblasts showed significantly increased numbers of stress fibres (Fig 1B)

Fig 3 MSDH induces mitochondrial ROS production by rupturing lysosomes Purified mitochondria (1.0 mg proteinÆmL)1) or a lysosome/mito-chondria-enriched fraction (1.0 mg proteinÆmL)1) were incubated with either of MSDH (30 l M ), PLA2 (0.2 UÆmL)1), or cathepsin B or D (12.5 lgÆmL)1; pH 6.0) for 3 h (A) H 2 O 2 production, (B) cytochrome c release, and (C) lysosomal stability were assayed as described in Materials and methods (n ¼ 3).

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Because oxidative stress has been reported to induce

stress fibre formation [37], we suspected that the MSDH

exposure might be causing increased intracellular generation

of ROS This latter was monitored by following changes in

DHE-induced fluorescence When oxidized, this compound

intercalates into DNA and RNA, resulting in an increase in

quantum yield Fluorescence intensity was measured

kineti-cally at indicated time points Increased ROSproduction

occurred after 1 h of MSDH-exposure in fibroblasts

(AG1518) and epithelial cells (HeLa), but was significant

only after 3 h in macrophages (J774) (Fig 2A) Note that in

fibroblasts and HeLa cells, and also in J774 cells (results not

shown), the oxidation of DHE eventually reached a steady

state consistent with only a transient production of ROS

Figure 2B shows DHE-exposed J774 cells after 3 h

expo-sure to MSDH, when there were still no morphological

signs of apoptosis

Theoretically, the increased oxidant generation might

arise from effects of released lysosomal enzymes (directly or

by activation of PLA2) on mitochondrial ROSproduction

or, alternatively, from direct effects of MSDH on the

mitochondria To discriminate between these possibilities,

we added MSDH to purified rat liver mitochondria

(4.5-fold purified from lysosomal contamination as compared to

the LEF preparation, results not shown) Under these

conditions, no changes in mitochondrial production of

H2O2(Fig 3A) or release of cytochrome c (Fig 3B) took

place Because we previously observed that lysosomal

contents cause activation of PLA2 in J774 cells [22], we

also exposed mitochondria to that enzyme and found it to

enhance mitochondrial production of ROS(Fig 3A) and to

release cytochrome c as well (Fig 3B) These findings strongly suggest that MSDH affects mitochondria by first destabilizing lysosomes and causing the release of hydrolytic enzymes which, in turn, attack mitochondria or activate PLA2 Activated PLA2 may further promote this cascade

of events, attacking both mitochondrial and lysosomal membranes and causing further lysosomal rupture This supposed sequence of events was confirmed by adding MSDH to a lysosome/mitochondria-enriched rat liver fraction, where it was found to induce enhanced mito-chondrial production of H2O2 (Fig 3A), release of cyto-chrome c (Fig 3B), and lysosomal rupture (Fig 3C)

To test further the idea that released lysosomal hydrolases might enhance mitochondrial ROSproduction, release of cytochrome c, and activation of PLA2 (all of which may promote the apoptotic cascade), we tested the effects of two lysosomal cathepsins (cathepsin B, a cysteine protease, and cathepsin D, an aspartic protease)

on purified mitochondria Both proteases caused substan-tial increases in mitochondrial production of H2O2 (Fig 3A) and release of cytochrome c (Fig 3B) However, neither cathepsin B nor D caused detectable lysosomal rupture in LEF preparations (Fig 3C), although, as expected, both MSDH and PLA2 did induce lysosomal rupture (Fig 3C)

Thus, cathepsins B and D do not directly cause rupture of lysosomes in an LEF preparation However, the possibility remains that the intracellular release of other lysosomal hydrolases may do so, or that lysosomal proteases might secondarily destabilize lysosomes through, for example, enhanced oxidative stress or activation of PLA2 following

Fig 4 The lysosomal/mitochondrial axis theory of apoptosis Both the internal and external pathways may involve lysosomal rupture Released lysosomal enzymes (LE) may: (a) attack mitochondria directly, inducing oxidative stress and release of cytochrome c (this study and [12,20–22,49– 52]); (b) activate lytic pro-enzymes, such as PLA2, which may attack both mitochondria or lysosomes (this study and [22]); (c) activate Bid [53]; (d) directly activate caspases [15,16,54,55] It is also possible that released lysosomal enzymes backfire on still intact lysosomes, causing further rupture Caspase 8 may somehow induce lysosomal rupture [56,57] or the activation of death receptors may cause production of sphingosine [58], which is a lysosomotropic detergent [59]; while p53 causes lysosomal labilization by unknown mechanisms [14] Other mechanisms may also be involved in lysosomal labilization in relation to apoptosis.

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mitochondrial attack by cathepsins and PLA2 Indeed, low,

steady-state oxidative stress has been shown to destabilize

lysosomes [20] and relocation of lysosomal enzymes to the

cytosol was earlier shown to activate PLA2 [22]

Discussion

We previously suggested that oxidative stress-induced

apoptosis might be initiated by iron-catalysed lysosomal

rupture [9,10] It has since been found that early release to

the cytosol of lytic lysosomal enzymes may be characteristic

of apoptosis caused by a variety of stimuli [10,12–14,

19,21,22,38–40] In these latter circumstances, it appears

that relocation of lysosomal enzymes to the cytosol may, as

in the case of oxidant-induced apoptosis, precede changes

of mitochondrial membrane potential, release of

cyto-chrome c, and all the morphological signs of apoptosis

These considerations raised the question of whether there

might be some ROS-dependent mechanisms common to

apoptosis caused by oxidants and that caused by

nonoxi-dant agents

In most cells, the predominant source of intracellular

ROSgeneration is the mitochondrial electron transport

chain which, even under normal conditions, may leak

1–2% of all electrons as ROS[41–43] (although there is

controversy regarding this estimate and the absolute

percentage may well be lower [44]) Not only will exogenous

oxidants, such as H2O2, directly induce apoptosis, but

enhanced intracellular production of ROSoccurs when cells

are exposed to a number of pro-apoptotic agents including

tumour necrosis factor-a [23], ceramide [24], growth factor

withdrawal, HIV infection, and lipopolysaccharide [25–30]

In these cases it is unclear whether such oxidative stress is

the cause or an effect of apoptosis

We hypothesized that released lysosomal enzymes or

PLA2 directly or indirectly activated by such enzymes [22]

might attack mitochondria and induce not only release

of cytochrome c, but also enhanced formation of ROS

Released arachidonic acid may further exaggerate this

process [45] These ROSof mitochondrial origin could

promote further lysosomal rupture but could also have the

secondary effect of maintaining any cytochrome c released

by the mitochondria in the oxidized form (although we

should note that the cellular cytoplasm contains an

abun-dance of reducing agents which could counteract this)

Cytochrome c is involved in the activation of caspase-9

[7,46] and is considered a key component of the apoptotic

cascade Ordinarily, any cytochrome c released from

mitochondria in oxidized form would rapidly be reduced

by the reductive cytosolic milieu However, it has been

proposed that cytochrome c needs to remain oxidized

in order to promote apoptosis [46], and the oxidizing

equivalents generated by mitochondria may have precisely

this effect

MSDH is a lysosomotropic detergent that rapidly induces

specific lysosomal rupture and therefore is a very useful tool

for detailed kinetic studies of the consequences of lysosomal

rupture The pKa of MSDH is 5.8–5.9 [31,32], allowing it to

accumulate in charged form intralysosomally (pH 4.5)

due to proton trapping [47], while its accumulation in the

cytosol (pH 7.2) is negligible In protonated, charged

form MSDH acts as a much stronger detergent than when

uncharged, further targeting the action of this agent to the lysosomal compartment [31]

We previously reported that released lysosomal enzymes activate PLA2 causing further lysosomal fragmentation [22] The new data presented here confirm and extend those findings and show that relocated lysosomal enzymes work

in concert with activated PLA2, causing the release of cytochrome c, enhanced mitochondrial formation of ROS, and promoting further lysosomal degradation With regard

to the mechanisms involved in enhanced mitochondrial ROSproduction, one particularly likely possibility is that of generation of free fatty acids At least in pancreatic beta cell mitochondria, free fatty acids have been shown to increase ROSgeneration, perhaps through electron leak involving complex I of the respiratory chain [48] Whether the progressive lysosomal destabilization is dependent exclu-sively on upstream actions of cathepsins B and D, or whether other lysosomal constituents might similarly desta-bilize mitochondria and lysosomes is not yet clear Our present understanding concerning the involvement

of lysosomes in apoptosis is summarized in Fig 4 As shown, the initiation of apoptosis by exogenous oxidants, and by at least some other agonists, may involve early lysosomal rupture The release of lysosomal enzymes (LE) into the cell cytoplasm may set off a cascade of intracellular degradative events These LE may: (a) attack mitochondria directly, inducing release of cytochrome c; (b) directly and/

or indirectly cause enhanced formation of mitochondrial ROS(and further oxidant-induced lysosomal destabiliza-tion); (c) activate lytic pro-enzymes, such as PLA2, which in turn would attack both mitochondria and lysosomes; (d) activate Bid and/or other pro-apoptotic proteins; and (e) directly activate pro-caspases Notably, this sequence of early events (except for cytochrome c release) may be relatively independent of the classical apoptotic cascade involving caspase activation In many circumstances, this

lysosomal-mitochondrial axis apoptotic pathway, invol-ving combined effects of caspases, lysosomal hydrolases and mitochondrial ROSgeneration, may be of central import-ance in the final execution of the apoptotic cascade wherein

a lysosomal/mitochondrial cross-talk may constitute an amplifying loop

Acknowledgements

We thank G Dubowchik (Bristol-Myers Squibb; Pharmaceutical Research Institute) for the kind gift of MSDH This study was supported by a grant from the Swedish Cancer Foundation (grant no 4296) JWE was the recipient of a visiting professorship from the Linko¨ping University Hospital and is supported by The Common-wealth of Kentucky Research Challenge Trust Fund.

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