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In an oxa1D mutant, cellular and mitochondrial levels of phosphatidylethanolamine were lowered similar to a mutant with PSD1 deleted, and the rate of phosphatidylethanolamine synthesis b

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is affected by the mitochondrial components Oxa1p and Yme1p

Ruth Nebauer1, Irmgard Schuiki1, Birgit Kulterer2, Zlatko Trajanoski2and Gu¨nther Daum1

1 Institute of Biochemistry, Graz University of Technology, Austria

2 Institute for Genomics and Bioinformatics and Christian-Doppler Laboratory for Genomics and Bioinformatics,

Graz University of Technology, Austria

Phosphatidylserine decarboxylases (PSDs) catalyze the

formation of phosphatidylethanolamine (PtdEtn) from

phosphatidylserine (PtdSer) These enzymes play a key

role in phospholipid metabolism from bacteria to

humans In the yeast Saccharomyces cerevisiae there

are two different PSDs, Psd1p, which is associated

with the inner mitochondrial membrane (IMM) [1],

and Psd2p, which is a component of the Golgi [2]

Unlike bacteria, yeast and other eukaryotes can also

synthesize PtdEtn via a pathway that is independent

of PSDs and uses cytidine diphosphate-ethanolamine

and diacylglycerol as substrates [3,4]

PtdEtn is an essential component of yeast mitochon-drial membranes Depletion of PtdEtn in mitochondria leads to dysfunctions in respiration, defects in the assembly of mitochondrial protein complexes and loss

of mitochondrial DNA [5–7] Deletion of the major PtdEtn-synthesizing enzyme, Psd1p, causes a substan-tial decrease in PtdEtn in cellular and mitochondrial membranes, thereby conferring a petite phenotype characterized by a loss of respiratory capacity [5] The link between cell respiration and PtdEtn homeostasis

in mitochondria tempted us to speculate that: (a) other defects resulting in the depletion of mitochondrial

Keywords

mitochondria; Oxa1p;

phosphatidylethanolamine;

phosphatidylserine decarboxylase; yeast

Correspondence

G Daum, Institute of Biochemistry, Graz

A-8010 Graz, Austria

Fax: +43 316 873 6952

Tel: +43 316 873 6462

E-mail: guenther.daum@tugraz.at

(Received 27 August 2007, revised 10

Octo-ber 2007, accepted 11 OctoOcto-ber 2007)

doi:10.1111/j.1742-4658.2007.06138.x

The majority of phosphatidylethanolamine, an essential component of yeast mitochondria, is synthesized by phosphatidylserine decarboxylase 1 (Psd1p), a component of the inner mitochondrial membrane Here, we report that deletion of OXA1 encoding an inner mitochondrial membrane protein translocase markedly affects the mitochondrial phosphatidyletha-nolamine level In an oxa1D mutant, cellular and mitochondrial levels of phosphatidylethanolamine were lowered similar to a mutant with PSD1 deleted, and the rate of phosphatidylethanolamine synthesis by decarboxyl-ation of phosphatidylserine in vivo and in vitro was decreased This was due to a lower PSD1 transcription rate in the oxa1D mutant compared with wild-type and compromised assembly of Psd1p into the inner mito-chondrial membrane Lack of Mba1p, another component involved in the assembly of mitochondrial proteins into the inner mitochondrial mem-brane, did not affect the amount of phosphatidylethanolamine or the assembly of Psd1p Deletion of the inner membrane protease Yme1p enhanced Psd1p stability suggesting that Yme1p contributed substantially

to the proteolytic turnover of Psd1p in wild-type In summary, our results demonstrate a link between the mitochondrial protein import machinery, assembly and stability of Psd1p, and phosphatidylethanolamine homeo-stasis in yeast mitochondria

Abbreviations

IMM, inner mitochondrial membrane; PSD, phosphatidylserine decarboxylase; PtdCho, phosphatidylcholine; PtdEtn,

phosphatidylethanolamine; PtdIns, phosphatidylinositol; PtdSer, phosphatidylserine.

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PtdEtn may also cause the petite phenotype, and⁄ or

(b) petite mutations may generally affect the formation

of mitochondrial PtdEtn Based on this hypothesis, we

screened a yeast petite mutant collection [8] for strains

with abnormal phospholipid patterns Among the

candidate strains identified (R Nebauer, unpublished

results), a mutant with OXA1 deleted exhibited marked

PtdEtn depletion

Oxa1p is a polypeptide involved in the insertion of

mitochondrially encoded proteins into the IMM, but it

also mediates the assembly of nuclear-encoded proteins

into this submitochondrial fraction [9] The import of

proteins synthesized on cytoplasmic ribosomes into

mitochondria starts with translocation across the outer

mitochondrial membrane, mediated by a general

import machinery, the translocase of the outer

mem-brane complex Assembly of polypeptides into the

IMM requires an energized IMM and another

trans-location machinery, the translocase of the inner

membrane complex [9–12] IMM proteins are targeted

to mitochondria by N-terminal targeting signals,

imported into the mitochondrial matrix and sorted to

the IMM via a specific export pathway [9,13–15]

Proteins with their N-termini protruding into the

inter-membrane space attain their inter-membrane orientation by

physical interaction with Oxa1p [16], although the

function of this protein is not limited to proteins that

undergo N-terminal tail export Recently, Mba1p was

identified as a protein that interacts with the Oxa1p

insertion machinery of the IMM [17] Mba1p binds to

the large subunit of mitochondrial ribosomes and

thereby cooperates with the C-terminal

ribosome-bind-ing domain of Oxa1p to ensure proper insertion of

proteins into the IMM

Like the majority of mitochondrial proteins, the

mitochondrial PtdSer decarboxylase Psd1p is encoded

by a nuclear gene, synthesized as a larger precursor on

cytoplasmic ribosomes and imported

post-translation-ally into mitochondria [18] As indicated in the

Uni-Prot knowledge base (http://www.uniprot.org⁄ ), the

yeast Psd1p proenzyme has one potential

mitochon-drial targeting sequence and an a-chain and b-chain

linked by a defined cleavage site [19] According to

von Heijne [20] or applications available at ExPASy

(http://www.expasy.org/) [21], Psd1p localized to the

IMM [1] has at least one transmembrane domain The

N-terminus of Psd1p contains motifs for protein

tar-geting to mitochondria and specifically to the IMM⁄

intermembrane space [18,22]

In this study, we analyzed the roles of Oxa1p,

Mba1p and the IMM protease Yme1p in the

forma-tion of PtdEtn by Psd1p We demonstrate that in an

oxa1D mutant inefficient assembly of Psd1p into the

IMM leads to decreased PtdEtn levels in yeast mito-chondria No such effect could be observed in an mba1D strain Moreover, we show that lack of the IMM protease Yme1p prevents degradation of Psd1p resulting in partial protection of its enzymatic activity Thus, specific components of the mitochondrial bio-synthetic machinery indirectly affect phospholipid homeostasis in this organelle

Results

The oxa1D mutant has an abnormal phospholipid composition

Screening of a set of petite (respiratory-deficient) yeast strains [8,23] for defects in the PtdEtn and phosphati-dylcholine (PtdCho) biosynthetic pathways revealed a number of candidate genes whose deletion caused changes in the amounts of at least one of the major phospholipids PtdCho, PtdEtn, and⁄ or phosphatidyl-inositol (PtdIns) in the cell homogenate and⁄ or mito-chondria (R Nebauer, unpublished data) One of these strains exhibiting decreased cellular PtdEtn levels com-pared with wild-type was the oxa1D mutant (Table 1), which is known to bear a defect in protein transloca-tion from the mitochondrial matrix to the IMM (see above) Fluorescence microscopic inspection employing DAPI staining revealed mitochondrial DNA in wild-type and oxa1D The amount of mitochondrial DNA appeared to be lower in oxa1D than in wild-type Thus, the petite phenotype of the mutant was not caused by a rho-mutation

The decrease in cellular PtdEtn in oxa1D was com-pensated by increased amounts of PtdIns, and also of lysophospholipids, phosphatidic acid and, to a lesser extent, PtdCho (Table 1) The decrease and compensa-tion in oxa1D were similar to psd1D, which lacks the major enzyme of cellular PtdEtn formation, mitochon-drial Psd1p In oxa1D mitochondria, the effect of PtdEtn depletion was even more pronounced than in total cell extracts Depletion of mitochondrial PtdEtn

in oxa1D was mainly compensated by an increase in PtdIns and, to a lesser extent, PtdCho Although the decrease in PtdEtn in oxa1D mitochondria was compa-rable with that in psd1D, there was a difference in the amount of mitochondrial PtdSer in these two strains

In psd1D, PtdSer imported into mitochondria from the endoplasmic reticulum [3] was not further converted to PtdEtn and accumulated in this organelle to some extent, whereas no such accumulation was observed with oxa1D Lack of such an accumulation appears to

be due to residual Psd1p activity in oxa1D mitochon-dria, as shown below

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During our studies of oxa1D-dependent PtdEtn depletion in yeast mitochondria we also investigated the effect on PtdEtn homeostasis of other yeast gene products that are related or linked to the Oxa1p-dependent protein translocation machinery These strains were mutants of MBA1, which encodes a com-ponent involved in the Oxa1p-dependent export of mitochondrially encoded proteins into the IMM [17], and YME1, which encodes an intermembrane space-located ATP-dependent AAA protease (ATPase associ-ated with various cellular activities) [24] The mba1D deletion strain exhibited only a slight decrease in total cellular PtdEtn and essentially the same mitochondrial phospholipid pattern as wild-type (Table 1) The yme1D and yme1D oxa1D mutants contained cellular and mitochondrial amounts of PtdEtn (Table 1) that exceeded wild-type levels

Deletion of OXA1 affects the rate of PtdEtn synthesis by Psd1p in vivo

Because mitochondrial Psd1p is the major producer of cellular PtdEtn, we hypothesized that the decrease in total cellular and mitochondrial PtdEtn levels in the oxa1D mutant were due to reduced activity of this enzyme To test this hypothesis, we performed in vivo experiments labeling PtdSer with [3H]serine and fol-lowed its conversion to PtdEtn and PtdCho in a time-dependent manner (see Experimental procedures) All strains tested showed a linear increase in the formation

of the three aminoglycerophospholipids within the selected timeframe, which enabled us to determine the rate of formation, i.e the incorporation of radiolabel per period, for each phospholipid The formation rates for PtdSer, PtdEtn and PtdCho in wild-type cells were set at 100%, and the corresponding rates for mutant strains were calculated accordingly As can be seen from Fig 1, deletion of OXA1 decreased the rate of formation of all aminoglycerophospholipids The rate

of PtdSer synthesis decreased to 80%, the rate of PtdEtn formation to 70% and that of PtdCho synthesis

to 60% of wild-type Because Oxa1p was assumed to compromise only the mitochondrial PtdEtn-synthesiz-ing Psd1p, leavPtdEtn-synthesiz-ing the Golgi-located Psd2p unaffected, the decrease in the rate of PtdEtn synthesis in oxa1D confirmed a defect in Psd1p-dependent PtdEtn forma-tion Under these circumstances, the decreased rate of PtdCho formation seemed to be due to the lowered rate

of PtdEtn formation, whereas reduced PtdSer forma-tion might reflect a response to a feedback regulatory mechanism It should be noted that the steady-state lev-els of individual phospholipids do not necessarily reflect the rates of synthesis of the components

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In contrast to psd1D, however, the oxa1D mutation

led to a smaller reduction of PtdEtn synthesis That

the psd1D strain and the oxa1D psd1D double mutant

had comparable rates of PtdEtn formation suggests

that Oxa1p acted upstream of Psd1p Not

unexpect-edly, rates of PtdEtn formation in the oxa1D psd2D

mutant were lower than in the psd2D mutant indicating

an additive effect of these two mutations acting on

two different pathways Taken together, deletion of

OXA1 affected synthesis of PtdEtn by Psd1p, but did

not completely abolish the activity of this enzyme

Activity of Psd1p in vitro is impaired in oxa1D

PtdEtn depletion in mitochondria and the decreased

rate of Psd1p-dependent PtdEtn formation in oxa1D

suggested a functional impairment of mitochondrial

Psd1p To address the question of Psd1p enzyme

activ-ity we subjected subcellular fractions of an oxa1D

mutant to enzymatic analyses As can be seen from

Fig 2, the in vitro activity of Psd1p with oxa1D

chondria was only 60% that of wild-type In

mito-chondria from the psd1D mutant there was no

measurable Psd1p activity (data not shown) Psd1p

activity in mitochondria from mba1D was not

decreased, in line with the unchanged mitochondrial

level of PtdEtn in this strain (Table 1)

Studies on the stability of subunits of the

mitochon-drial membrane complexes Cox and ATPase revealed

that these proteins are degraded in the absence of

Oxa1p [25] When functional Oxa1p is missing the

membrane subunits of these complexes cannot be

assembled and are cleaved by the intermembrane space

(i)-AAA protease Yme1p and⁄ or by the matrix

(m)-AAA protease Afg3p⁄ Yta12p To test whether Psd1p

stability was also affected by the presence or absence

of these mitochondrial hydrolases, we analyzed Psd1p activity in the respective single mutants or in double mutants in combination with oxa1D Deletion of YME1encoding the i-AAA protease led to a consider-able increase in Psd1p activity (Fig 2), which is in line with the increased PtdEtn level in a deletion mutant compared with wild-type (Table 1) This observation was surprising because overexpression of the PtdEtn biosynthetic pathway enzymes phosphatidylserine syn-thase 1 (Pss1p) and⁄ or Psd1p did not change the PtdEtn level (R Birner-Gruenberger, unpublished results) The yme1D oxa1D double mutant showed an intermediate value for the Psd1p activities from the single mutants Yme1p appears to contribute markedly

Fig 1 Deletion of OXA1 causes a

decreased rate of PtdEtn synthesis in vivo.

Wild-type and mutant strains were labeled

Incorporation of label into PtdSer, PtdEtn

and PtdCho was determined by

liquid-scintil-lation counting after separation of

phospho-lipids by TLC (see Experimental procedures).

The formation rate of PtdSer, PtdEtn and

PtdCho of wild-type (black bars) was set at

100% Values are means from three

inde-pendent experiments with mean deviations

as indicated by the error bars.

Fig 2 The oxa1D mutation affects Psd1p activity in vitro Enzy-matic assays were performed with isolated mitochondrial fractions from wild-type BY4742, mba1D, oxa1D, yme1D and yme1D oxa1D Values are expressed relative to wild-type which was set at 100% and are means from three independent experiments with mean deviations as indicated by the error bars.

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to the proteolytic turnover of Psd1p Deletions of

either subunit of the m-AAA protease yta12D and

afg3D, respectively, seemed to have no effects on

Psd1p turnover (data not shown) and were not

investi-gated further

A decreased transcription rate for PSD1 and a

defect in Psd1p maturation are the molecular

basis of the decreased rate of PtdSer

decarboxylation in oxa1D mitochondria

One obvious explanation for the decreased amount of

Psd1p in oxa1D mitochondria was a possible reduction

in the PSD1 transcription rate in the mutant To

address this question we performed RT-PCR analyses

of PSD1 mRNA with wild-type and oxa1D (see

Exper-imental procedures) These analyses revealed a

reduc-tion in PSD1 mRNA in the mutant The transcripreduc-tion

rate for PSD1 was repressed in oxa1D to  50% that

of wild-type Thus, downregulation of PSD1

expres-sion at the transcriptional level appears to be one

reason for the decreased Psd1p activity in oxa1D

Because Oxa1p had been shown to facilitate

mem-brane assembly in several mitochondrial proteins (see

above), it was tempting to speculate that it was also

necessary for correct insertion of Psd1p into the IMM

To test this hypothesis, we performed import

experi-ments of radioactively labeled Psd1p into isolated

mitochondria These in vitro assays (see Experimental

procedures) allowed analysis of protein assembly into

mitochondrial membranes independent of the

tran-scriptional level of a respective gene The full-length

precursor form of Psd1p was synthesized by a coupled

transcription⁄ translation reaction and incubated with

wild-type and oxa1D mitochondria Complete

process-ing of Psd1p occurred in three proteolytic steps

(Fig 3A) The primary translation product of 57 kDa

was cleaved to a first intermediate of 52 kDa, most

likely during or immediately after the import process

This cleavage step is in agreement with the finding that

a positively charged amino acid stretch at the

N-termi-nus of Psd1p serves as a mitochondrial targeting

sequence Processing of Psd1p was continued by

cleav-age of a 2 kDa fragment representing the

inter-membrane space sorting signal, yielding the second

intermediate of 50 kDa Assembly of Psd1p into the

IMM was completed by (autocatalytic) cleavage of the

50 kDa intermediate to one a-chain and one b-chain

(4 and 46 kDa mature forms) The 4 kDa a-subunit

was not detected in electrophoretic analysis

In oxa1D (Fig 3B), import and processing of Psd1p

occurred more slowly than in wild-type resulting in

a lower ratio of mature form to precursors Thus,

deletion of OXA1 decreased both the transcription rate

of PSD1 and the Psd1p assembly rate into the IMM Both effects appear to result in a reduced amount of enzymatically active Psd1p in the IMM and thus in a decreased capacity to form PtdEtn

Discussion

The biosynthetic scheme shown in Fig 4 summarizes the possible ways in which the mitochondrial level of PtdEtn can be affected First, is the supply of PtdSer

to the mitochondria as a precursor for PtdEtn forma-tion by Psd1p This process includes synthesis of Ptd-Ser in the endoplasmic reticulum by the PtdPtd-Ser synthase Pss1p and translocation of PtdSer to the site

of Psd1p-catalyzed decarboxylation in the IMM Sec-ond, mitochondrial factors may, directly or indirectly,

A

B

Fig 3 Proteolytic processing of Psd1p Maturation of Psd1p was measured in wild-type BY4742 (A) and oxa1D (B) The primary translation product of 57 kDa (not shown in the diagram) was cleaved to a 52 kDa intermediate (d), which was further processed

to yield a 50 kDa polypeptide (h) The final processing step leads

to the formation of the mature 46 kDa b-subunit of Psd1p (*) For each time point, the amount of every single processing intermedi-ate was expressed as percent of the sum of all intermediintermedi-ates Values are means from three independent experiments with mean deviations as indicated by the error bars.

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affect the activity of Psd1p, thereby decreasing or

increasing the efficiency of mitochondrial PtdEtn

for-mation Third, import and export of PtdEtn may

con-tribute to a balance in the level of this phospholipid in

mitochondria Finally, although not addressed

specifi-cally in this scheme, transcriptional⁄ translational

regu-lation of PSD1 expression has to be taken into

account

Similar to plants [26], increased levels of yeast

mito-chondrial Psd1p are not necessarily accompanied by

an increase in the amount of mitochondrial PtdEtn In

strains overexpressing Pss1p and⁄ or Psd1p neither the

PtdSer nor the PtdEtn level was markedly changed

compared with wild-type (R Birner-Gruenberger,

unpublished data) These findings imply that the

amount of mitochondrial PtdEtn is tightly controlled

by one of the above-mentioned regulatory mechanisms

Alternatively, the wild-type level of Psd1p may already

represent an excess of activity which cannot be

enhanced further by increasing the amount of protein

A search for components affecting mitochondrial

PtdEtn levels led to the identification of mitochondrial

components interacting directly with

mitochon-drial Psd1p One example of such a component is the

mitochondrial prohibitin, Phb1p⁄ Phb2p Recent studies

in our laboratory demonstrated the synthetic lethality

of a psd1D phb1⁄ 2D double mutant [7] It was

specu-lated that the decreased PtdEtn level in mitochondria

caused by psd1D might be harmful in the phb1⁄ 2D

back-ground, which by itself causes an increase in

mitochon-drial PtdEtn In view of the results of this study, this

hypothesis appears to be wrong, because depletion of

the mitochondrial PtdEtn level by oxa1D to an amount

comparable with that in psd1D did not lead to synthetic

lethality with phb1⁄ 2D (R Nebauer, unpublished

results) Thus, it is the direct interaction of Psd1p

and Phb1⁄ 2p or even a more complex effect through

combination of the two gene products that may be important for mitochondrial function

In this study, we demonstrate another mode of action that affects Psd1p activity in yeast mitochon-dria, namely disturbance of the import and assembly

of this polypeptide into mitochondrial membranes We show that Oxa1p facilitates the import of Psd1p to its proper destination in the IMM Oxa1p has been char-acterized previously as a helper protein for the assembly of a number of other IMM proteins [9]

In wild-type yeast cells, import into mitochondria, pro-cessing and assembly into mitochondrial membranes of Psd1p is accomplished by a three-step mechanism simi-lar to Chinese hamster ovary cells [27] According to Boeckmann et al [28], Psd1p contains all the features

of a typical IMM⁄ intermembrane space protein, namely a positively charged N-terminal sequence fol-lowed by a hydrophobic stretch The three cleavage steps are accomplished by the mitochondrial-process-ing peptidase (MPP), the intermembrane space prote-ase Imp1p and autocatalysis

In oxa1D, the Psd1p processing rate was decreased (Fig 3) This resulted in slower utilization of the pre-cursor polypeptide in the mutant than in wild-type, delayed formation of intermediates and finally a decreased appearance of the mature form Although only one intermediate step in the process, namely translocation of the 50 kDa intermediate to the IMM, appears to be directly affected by Oxa1p, the whole process of Psd1p assembly into the IMM occurs more slowly in the mutant than in the wild-type The resid-ual Psd1p activity in oxa1D appears to be due to alter-native import pathways

In addition to the reduced rate of Psd1p import into mitochondria, the decreased transcription rate of PSD1 in oxa1D seems to play a role in imbalanced PtdEtn formation of the mutant We can only

specu-Fig 4 Factors affecting the PtdEtn level in mitochondria Pss1p (phosphatidylserine synthase 1), Psd1p (phosphatidylserine decarboxylase 1), Psd2p (phosphatidylserine decarboxylase 2), Dpl1p (dihydrosphingosine 1-phosphate lyase 1), import of PtdSer into mitochondria (x), export of PtdEtn from mitochondria (y), import of PtdEtn into mitochondria (z) and factors (F) affecting level and activity of Psd1p in mito-chondria may contribute to the mitomito-chondrial PtdEtn.

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late at present that a negative feedback control caused

by unassembled Psd1p precursor or intermediate

pro-teins might trigger this transcriptional regulation

However, the additive effects of reduced PSD1

tran-scription and Psd1p assembly are sufficient to cause a

limitation of active Psd1p being present in

mitochon-dria of oxa1D

Another component that affects the mitochondrial

level of Psd1p activity is the intermembrane space

protease Yme1p In a yme1D strain, Psd1p activity

exceeded the wild-type level, and in the oxa1D

back-ground, yme1D restored Psd1p activity to a higher

level than wild-type Under the latter conditions,

Oxa1p-independent insertion of Psd1p seems to be

suf-ficient to ensure assembly of a functional enzyme

exhibiting activity higher than wild-type We assume

from these results that Yme1p contributes to Psd1p

degradation and turnover In a yme1D strain, an excess

of Psd1p appears to accumulate in the IMM leading

to the observed effects of enhanced enzyme activity

and increased PtdEtn levels

In summary, our results demonstrate a link between

the mitochondrial machinery of protein assembly and

PtdEtn homeostasis in mitochondria and the whole

cell We have to keep in mind, however, that depletion

of mitochondrial PtdEtn by the various possible effects

described appears to negatively affect proteins involved

in mitochondrial function or membrane properties and

may thus contribute to a petite phenotype (respiratory

defect) By contrast, it should be noted that not all

respiratory defects of mitochondria need to be linked

to lipid defects in mitochondrial membranes as

docu-mented by a recent screening of petite strains in our

laboratory (R Nebauer, unpublished results) Rather

it appears that Psd1p-dependent PtdEtn formation is

affected by a distinct set of mitochondrial proteins,

e.g Oxa1p, which are involved in the correct assembly

of Psd1p into the IMM

Experimental procedures

Strains and culture conditions The yeast strains used in this study are listed in Table 2 Yeast mutants exhibiting a petite phenotype as described by Dimmer et al [8] were obtained from the Euroscarf strain collection (Frankfurt, Germany) S cerevisiae strains were grown under aerobic conditions at 30C on YPD medium containing 1% yeast extract, 2% peptone, and 2% glucose

as the carbon source For large scale cultivation, inocula-tions to a D600 of 0.1 in fresh medium were made by diluting precultures grown to the stationary phase For auxotrophy tests, yeast strains were cultivated on solid syn-thetic medium [29]

Plasmid and strain constructions Primers used in this study are listed in Table 3 The yeast deletion mutants oxa1D::His3MX6 and psd1D::His3MX6 were constructed as described by Longtine et al [30] Prim-ers OXA1-F1 and OXA1-R1 or PSD1-F1 and PSD2-F2, respectively, were used to amplify the His3MX6 disruption cassette The cassette was introduced into the respective strain by lithium acetate transformation [31] Correct inser-tion of the cassette was tested by growing strains on selec-tive media without the respecselec-tive amino acid and by colony PCR with the appropriate primers Double-deletion mutants were constructed by mating the corresponding single-deletion mutants, sporulation of zygotes, and tetrad dissection using standard methods Identity of strains was confirmed by marker-dependent growth and colony PCR

Fluorescence microscopy Visualization of mitochondrial DNA in living cells was per-formed using the fluorescent dye DAPI In brief, cells were grown in YPD medium over night at 30C An inoculation

to a D600of 0.3 in fresh medium was made by diluting of

Table 2 Yeast strains used in this study.

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an overnight culture and cells were harvested in the mid-log

phase DNA was stained with 2.5 lgÆmL)1 of DAPI

dis-solved in NaCl⁄ Piat 30C for 30 min After staining, cells

were rinsed once with NaCl⁄ Pi and then resuspended in

NaCl⁄ Pi Suspensions were placed on a glass slide and

cov-ered with a cover slip Cells were then visualized using a

fluorescence microscope (Axiovert 35, Carl Zeiss, Jena,

Germany) with the appropriate filter set for the

blue-emit-ting fluorochrome DAPI and a 100-fold oil immersion

objective Mitochondrial DNA was visualized as smaller

spots distinct from larger nuclear DNA At least 100 cells

from all strains to be tested were inspected

Labeling of aminoglycerophospholipids in vivo

Labeling of aminoglycerophospholipids in vivo was

deter-mined by following the incorporation of [3H]serine into

PtdSer, PtdEtn and PtdCho as described by Birner et al

[7] For each time point, an equivalent of 10 D600 from

an overnight culture ( 1 mL, corresponding to

1.45· 108cells) was harvested, washed once, suspended in

500 lL YPD and incubated for 30 min at 30C Cells were

labeled with 10 lCi [3H]serine (27 CiÆmmol)1, Perkin–

Elmer, Boston, MA) per time point Samples were taken at

0, 15, 30 and 60 min, put on ice and harvested by

centrifu-gation Chloroform⁄ methanol (2 : 1, v ⁄ v) and glass beads,

3 mL each, were added to the cell pellets For

disintegra-tion of cells samples were shock frozen in liquid nitrogen

and shaken vigorously on an IKA Vibrax VXR for

15 min at 4C Then, lipids were extracted for 30 min by

the method of Folch et al [32] Individual phospholipids

were separated by TLC on Silica gel 60 plates (Merck,

Darmstadt, Germany) with chloroform⁄ methanol ⁄ 25%

ammonia (50 : 25 : 6, v⁄ v ⁄ v) as a developing solvent Spots

on TLC plates were stained with iodine vapor, scraped off

and suspended in 8 mL scintillation cocktail (Packard

Bio-Science, Groningen, the Netherlands) containing 5% water

Radioactivity was determined by liquid scintillation

count-ing using a Packard TriCarb Liquid Scintillation

Analyzer

Preparation of subcellular fractions, protein analysis, and enzymatic analysis

Mitochondria were prepared from spheroplasts by pub-lished procedures [1,33] Relative enrichment of markers and cross-contamination of subcellular fractions were assessed as described by Zinser and Daum [34] Protein was quantified by the method of Lowry et al [35] by using BSA

as a standard SDS–PAGE was carried out as published by Laemmli [36] Western blot analysis of proteins from subcellular fractions prepared as described above was performed as described by Haid and Suissa [37] Immunore-active bands were visualized by enzyme-linked immunosor-bent assay using a peroxidase-linked secondary antibody (Sigma-Aldrich, St Louis, MO) following the manufac-turer’s instructions

PtdSer decarboxylase activity was measured in isolated mitochondria from yeast cells grown in YPD to the loga-rithmic growth phase as reported by Kuchler et al [38] with minor modifications: 100 nmol [3H]PtdSer (specific activity

of 28 900 dpmÆnmol)1) was used as the substrate, and the assay was performed in 0.1 m Tris⁄ HCl, pH 7.2, containing

10 mm EDTA

Import of Psd1p into mitochondria in vitro Import, processing and assembly of Psd1p into mitochon-dria in vitro were assayed following the protocol of Ryan

et al [39] The precursor Psd1p was synthesized in the presence of [35S]methionine (15 mCiÆmL)1; Amersham Bio-sciences, Chalfont, UK) by coupled transcription⁄ translation in a reticulocyte lysate (Promega, Madison, WI) following the manufacturer’s instructions The T7 RNA polymerase system with a PCR-generated DNA fragment

as a template was employed Primers T1 and PSD1-U1 (see Table 2) were used to amplify PSD1 from genomic DNA Yeast mitochondria were isolated as described above and aliquoted at 10 mgÆmL)1 in SEM buffer containing

250 mm sucrose, 1 mm EDTA, 10 mm Mops-KOH, pH 7.2, and stored at )70 C The import assay involved

sequences are homologous to the His3MX6 disruption cassette (OXA1-F1, OXA1-R1, PSD1-F1, PSD1-R1) or to the PSD1 ORF (PSD1-T1) Primer PSD1-U1 is complementary to the region spanning the stop codon of the PSD1 ORF.

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tion of the radiolabeled Psd1p precursor with isolated

mito-chondria in the presence of NADH (1.8 mm) and ATP

(1.8 mm) in a buffer containing 3% (w⁄ v) fatty acid-free

BSA, 250 mm sucrose, 80 mm KCl, 5 mm MgCl2, 2 mm

KH2PO4, 5 mm methionine, 10 mm Mops-KOH, pH 7.2

[39] After 2, 5, 10, and 15 min samples were withdrawn

and put on ice in the presence of valinomycin (final

concen-tration 0.5 lm) to stop the import reaction Supernatants

were removed by centrifugation at 12 000 g and 4C for

5 min Pellets were washed once in SEM buffer, recovered

by centrifugation and suspended in SDS⁄ PAGE loading

buffer [36] prior to heating at 95C for 5 min Analysis of

radioactively labeled translation products, intermediates

and mature polypeptides was performed employing

stan-dard methods of SDS–PAGE, autoradiography and

densi-tometric scanning

Phospholipid quantification

For the analysis of total cellular phospholipids yeast cells

harvested from a 500 mL culture grown to the late

logarith-mic phase were disintegrated by shaking with glass beads in

a Merckenschlager homogenizer under CO2 cooling in the

presence of 10 mm Tris⁄ HCl, pH 7.2, and 1 mm

phenyl-methylsulfonyl fluoride (Calbiochem, La Jolla, CA) After

removal of the beads by centrifugation the supernatant

rep-resenting the total cell homogenate was aliquoted and

stored at )70 C Lipids from samples containing 3 mg

protein were extracted by the procedure of Folch et al [32]

using 4 mL chloroform⁄ methanol (2 : 1, v ⁄ v) Isolated

mitochondria (2 mg protein) were subjected to lipid

extrac-tion by the same method

Individual phospholipids were separated by 2D TLC

using chloroform⁄ methanol ⁄ 25% ammonia (70 : 35 : 5,

v⁄ v ⁄ v) as first, and chloroform ⁄ acetone ⁄ methanol ⁄ acetic

acid⁄ water (55 : 20 : 10 : 10 : 5, v⁄ v ⁄ v ⁄ v ⁄ v) as second

developing solvent Phospholipids were visualized on TLC

plates by staining with iodine vapor, scraped off and

quan-tified by the method of Broekhuyse [40]

RNA preparation and real-time PCR

Total RNA was isolated using phenol⁄ chloroform

extrac-tion as described previously [29] and further purified by

RQ1 RNase-free DNase (Promega) treatment according to

the manufacturer’s instructions and subsequent ethanol

pre-cipitation Integrity of RNA was tested by agarose gel

elec-trophoresis and determination of the 260 to 280 nm ratio

of the absorbencies RNA concentration was determined by

measurement of the absorbance at 260 nm

Total RNA was subjected to reverse transcription using

the SuperScriptTMII First Strand Synthesis System

(Invi-trogen, Carlsbad, CA) for real-time PCR (RT-PCR)

Possible traces of contaminating genomic DNA were

removed by DNAse I digestion In detail, 2.5 lg of RNA

with a concentration of 500 ngÆlL)1 were incubated with 10· DNAse I buffer, DNAse I amplification grade and

4 U RNaseOutTM ribonuclease for 15 min at room tem-perature (all reagents from Invitrogen) DNA digestion was stopped by adding 1 lL of EDTA (25 mm) and 2 lL

of H2O, incubating for 5 min at room temperature and further 5 min at 70C The DNase I treated RNA was mixed with 0.5 lg of oligo-dT2-18, 3 lg of random primers, and 4 U of RNaseOutTM ribonuclease, heated for 5 min

at 70C and left at room temperature for another 5 min The RNA sample was mixed with the cDNA synthesis mix, consisting of 5· RT buffer, dithiothreitol (0.1 m), dNTP (10 mm), 4 U RNaseOutTM and 200 U Super-ScriptTMII reverse transcriptase (all reagents from Invitro-gen), and heated to 45C for 1 h The reaction was stopped by heating to 95C for 5 min RT-PCR assays were performed using the Platinum SYBR Green Su-perMix-UDG (Invitrogen) following the manufacturer’s recommendations Primers for RT-PCR were designed using the software tool primer expressTM (ABI) For a

25 lL RT-PCR reaction, 1 lL of primer pair (800 nm), 0.5 lL of diluted cDNA (10 ngÆlL)1) and 12.5 lL of Plati-num SYBR Green SuperMix-UDG were applied In addition, no template controls (NTC) and no RT reaction (No RT) controls were performed The cycling conditions

on an ABI Prism 7000 were set for 2 min at 50C,

10 min at 95C and 40 cycles of 15 s at 95 C and 1 min

at 60C Data were analyzed using the ABI Prism 7000 sds software

Acknowledgements

The authors wish to thank A Hermetter for providing access to the fluorescence microscope (FWF instru-ment) This work was financially supported by the FWF (Fonds zur Fo¨rderung der wissenschaftlichen Forschung in O¨sterreich) projects 14468 and 17321 to GD

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