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Interestingly, decylQ, an analogue unable to support growth on glycerol, is not toxic, but antagonizes growth of DCoQ yeast in the pres-ence of exogenous CoQ2.. Recently we have shown th

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coenzyme Q analogues requires the isoprenoid side chain Andrew M James1, Helena M Cocheme´1,2, Masatoshi Murai3, Hideto Miyoshi3

and Michael P Murphy1

1 Medical Research Council Mitochondrial Biology Unit, Wellcome Trust⁄ MRC Building, Cambridge, UK

2 Institute of Healthy Ageing and GEE, University College London, Darwin Building, London, UK

3 Division of Applied Life Sciences, Graduate School of Agriculture, Kyoto University, Sakyo-ku, Kyoto, Japan

Introduction

CoenzymeQ (CoQ) is composed of a head group that

cycles between reduced ubiquinol and oxidized

ubiqui-none forms and a hydrophobic isoprenoid tail that

keeps the redox activity of the head group located

within the lipid bilayer The length of the isoprenoid tail

varies between species, with Saccharomyces cerevisiae, rats and humans predominantly synthesizing forms of CoQ containing six (CoQ6), nine (CoQ9) and 10 (CoQ10) isoprenoid units, respectively CoQ is synthe-sized endogenously by a series of enzymes localized to

Keywords

coenzyme Q; diauxic shift; mitochondria;

ubiquinone; yeast

Correspondence

A M James, Medical Research Council

Mitochondrial Biology Unit, Wellcome

CB2 0XY, UK

Fax: +44 1223 252905

Tel: +44 1223 252903

E-mail: aj@mrc-mbu.cam.ac.uk

Website: http://www.mrc-mbu.cam.ac.uk

(Received 18 December 2009, revised

2 February 2010, accepted 22 February

2010)

doi:10.1111/j.1742-4658.2010.07622.x

The ubiquinone coenzyme Q (CoQ) is synthesized in mitochondria with a large, hydrophobic isoprenoid side chain It functions in mitochondrial res-piration as well as protecting membranes from oxidative damage Yeast that cannot synthesize CoQ (DCoQ) are viable, but cannot grow on nonfer-mentable carbon sources, unless supplied with ubiquinone Previously we demonstrated that the isoprenoid side chain of the exogenous ubiquinone was important for growth of a DCoQ strain on the nonfermentable sub-strate glycerol [James AM et al (2005) J Biol Chem 280, 21295–21312] In the present study we investigated the structural requirements of exoge-nously supplied CoQ2for growth on glycerol and found that the first dou-ble bond of the initial isoprenoid unit is essential for utilization of respiratory substrates As CoQ2analogues that did not complement growth

on glycerol supported respiration in isolated mitochondria, discrimination does not occur via the respiratory chain complexes The endogenous form

of CoQ in yeast (CoQ6) is extremely hydrophobic and transported to mito-chondria via the endocytic pathway when supplied exogenously We found that CoQ2 does not require this pathway when supplied exogenously and the pathway is unlikely to be responsible for the structural discrimination observed Interestingly, decylQ, an analogue unable to support growth on glycerol, is not toxic, but antagonizes growth of DCoQ yeast in the pres-ence of exogenous CoQ2 Using a DCoQ double-knockout library we iden-tified a number of genes that decrease the ability of yeast to grow on exogenous CoQ Here we suggest that CoQ or its redox state may be a sig-nal for growth during the shift to respiration

Abbreviations

hexadecanoic acid; YPD, yeast extract, peptone, glucose; YPG, yeast extract, peptone, glycerol; YPGG, yeast extract, peptone, glycerol with glucose.

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the mitochondrial inner membrane, yet is a component

of lipid bilayers throughout the cell [1] In addition to

its function as an electron carrier in the mitochondrial

inner membrane, the reduced ubiquinol form of CoQ

acts as a recyclable antioxidant that protects biological

membranes from oxidative damage [2] CoQ10levels in

humans decrease in many pathological situations For

this reason it has been used as a therapy in diseases

where oxidative damage is thought to be important, for

example very high doses of CoQ10have been used with

some beneficial effect in Parkinson’s disease [3] As the

requirement for high doses probably results from the

extreme hydrophobicity of CoQ10and consequently its

low bioavailability, efforts have been made to improve

its water solubility by developing analogues with shorter

and more hydrophilic hydrocarbon tails [4,5]

In the yeast S cerevisiae, CoQ is not essential for

via-bility, as strains lacking the ability to synthesize

ubiqui-none (DCoQ) grow by fermentation on glucose

However, they do not grow on nonfermentable

sub-strates unless they are supplemented with exogenous

CoQ, in which case mitochondrial respiration is restored

[6] It has generally been thought that the redox active

ubiquinone head group is the important moiety and that

the hydrophobic tail merely anchors this activity in the

membrane Recently we have shown that when decylQ

and idebenone, two artificial analogues of CoQ

contain-ing a saturated 10-carbon alkane tail, are supplied

exogenously they are unable to restore growth on

non-fermentable substrates in DCoQ yeast [6] As more

hydrophilic (CoQ2) or hydrophobic (CoQ4or CoQ6)

iso-prenoid analogues could restore growth on

nonferment-able substrates [6], it would appear that the inability of

decylQ to do this does not result from differences in

pas-sive diffusion to mitochondria in yeast Instead, it

proba-bly arises from a selective protein interaction that can

differentiate between an isoprenoid and a saturated

alkane tail This is important, as shorter alkyl

ubiquinon-es, such as idebenone and decylQ, are easier to synthesize

and have been used therapeutically [4,5] Therefore, we

set out to ascertain the structural requirements for the

utilization of exogenous ubiquinone in yeast and to

iden-tify proteins that might be involved in discriminating

between alkane and isoprenoid ubiquinones

Results

Complementation of cell growth in CoQ-deficient

yeast by short-chain CoQ analogues shows a

dramatic dependence on the isoprenoid side chain

Yeast strains that cannot synthesize CoQ

endoge-nously (DCoQ) are unable to grow on nonfermentable

substrates, such as glycerol However, when CoQ is supplied exogenously in the growth medium, the abil-ity to grow on nonfermentable substrates is restored The utilization of glycerol as an energy source for growth requires the presence of CoQ in the mitochon-drial inner membrane to support respiration That DCoQ strains grow when supplemented with exoge-nous CoQ indicates that some of the externally sup-plied CoQ is reaching the mitochondrial inner membrane within yeast

Previous work showed that CoQ2 and CoQ6 sup-ported growth when supplied exogenously to DCoQ yeast [6] However, not all short-chain ubiquinone ana-logues could do this, as decylQ and idebenone, both of which contain a 10-carbon saturated side chain, failed

to support growth on nonfermentable substrates once glucose was depleted (Fig 1A) This suggested that the structure of the side chain was important for determin-ing whether DCoQ strains were able to grow on exoge-nously supplied ubiquinone Each isoprenoid unit contains a double bond and a methyl group that could explain the differential reactivity To identify the struc-tural basis of this interaction we utilized a series of ubiquinone analogues with varying similarities to CoQ2 (shown in Fig 1B) [7,8] The analogues differed from CoQ2 in a systematic way, with either the removal of a double bond, the deletion of a methyl group or the addition of a carbon atom When we investigated whether each analogue supported growth

in a DCoQ strain on the nonfermentable substrate glycerol, a consensus structural pattern emerged (Fig 1C) Analogues in which the first (A1-Q2) or sec-ond (A2-Q2) methyl group or second double bond (A3-Q2) were removed exhibited normal growth in glygerol-containing media, suggesting that none of these is required for growth (Fig 1C) This was in stark contrast to the analogues in which the first dou-ble bond was removed (A4-Q2 and A6-Q2), as these failed to promote any growth on nonfermentable sub-strates in the DCoQ strain (Fig 1C) The position of the double bond was also important, as inserting an extra carbon between the head group and the first dou-ble bond (A5-Q2) abolished aerobic growth (Fig 1C) Therefore, the presence of a double bond between C2 and C3 in the first isoprenoid unit of exogenously sup-plied CoQ is of critical importance for restoration of growth in DCoQ yeast strains

In summary, the high degree of selectivity for ubiq-uinones containing a double bond between C2 and C3

in the first isoprenoid unit suggests the presence of a specific interaction, presumably with a protein that is able to recognize subtle structural differences in the

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side chain of CoQ Therefore, we set out to try and

understand the nature of this interaction better

All CoQ2analogues restore respiration in isolated

mitochondria from DCoQ yeast

Growth on glycerol requires CoQ to pass electrons

from glycerol-3-phosphate dehydrogenase to complex

III of the mitochondrial respiratory chain Therefore,

we first considered the possibility that the different side

chains affected the way in which the ubiquinone

ana-logues interacted with the mitochondrial respiratory

chain To assess this, we tested their ability to restore

respiration on glycerol-3-phosphate in isolated

mito-chondria (Fig 2) All the analogues could do this with

equal efficacy (Fig 2), suggesting that the inability of

decylQ and the analogues A4-Q2, A5-Q2and A6-Q2 to

support growth on YPGG (yeast extract, peptone,

glycerol with glucose; Fig 1C) does not result from a

failure of their mitochondria to pass electrons from glycerol-3-phosphate to O2

Short-chain ubiquinone analogues do not appear

to require an intracellular transport pathway That DCoQ strains can grow on YPG (yeast extract, peptone, glycerol) when supplemented with exogenous CoQ indicates that a sufficient quantity of the supplied CoQ is reaching the mitochondrial inner membrane

As all of the analogues tested so far probably have hydrophobicities similar to that of CoQ2 and support respiration to a comparable degree in isolated mito-chondria (Fig 2), it is perhaps surprising that they do not all restore growth in intact cells (Fig 1C) One simple explanation would be that decylQ does not reach the mitochondrial inner membrane within cells

in sufficient quantities to facilitate the passage of elec-trons from glycerol to O2 This could arise via an

Time (h)

A600

0

2

4

6

8

10

12

14

Wild-type

Dcoq2 + 50 µM CoQ2

Dcoq2 + 50 µM decylQ

Dcoq2

0

2

4

6

8

10

A600

2

DecylQ A1-Q

2

A4-Q 2 A5-Q 2 A6-Q 2 A3-Q

2 A2-Q 2

O

O MeO

O

O MeO

O

O MeO

O

O MeO

O

O MeO

O

O MeO

O

O MeO

O

O MeO

O

O MeO

C

with growth above this requiring utilization of glycerol Values are the mean ± range of two independent experiments each carried out in duplicate.

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endogenous uptake pathway that recognizes the

iso-prenoid structure of CoQ2, but cannot interact with

the alkane tail of decylQ or any of the CoQ2analogues

lacking the first double bond That a CoQ transport

pathway exists for endogenous CoQ6in yeast has been

considered probable for some time, as the enzymes for

CoQ6 synthesis are located in the mitochondrial inner

membrane, but CoQ6 is found throughout the cell

[1,9] Spontaneous diffusion through the cytosol

appeared unlikely for this dispersion, as CoQ6 is very

hydrophobic with a predicted octanol⁄ water partition

coefficient in the region of 1014 [10] Recently it has

been shown that DCoQ yeast strains require at least four genes (tlg2, erg2, pep12 and vps45) from the endo-cytic pathway to grow on exogenously supplied CoQ6 [11] To test whether the endocytic uptake pathway was also required for the uptake of short-chain ana-logues such as decylQ and CoQ2 in our experiments,

we created two double-knockout strains and tested their ability to grow on CoQ2 Both Dcoq2Dtlg2 and Dcoq2Derg2 strains grew in the presence of CoQ2 (Fig 3A) as well as the even more hydrophobic ana-logue CoQ4(data not shown) The growth characteris-tics were similar to those seen in the Dcoq2 strain

C

G3P

+

FCCP

2 min

myxo

15 10

20

–1 ·mg

–1 ·mg

0 50 100 150 200 250

0 50 100 150 200 250

0 50 100 150 200 250

0

50

100

150

200

250

0 50 100 150 200 250

0 50 100 150 200 250

0 50 100 150 200 250

0

50

100

150

200

250

indicated by the arrowheads That the oxygen consumption was mitochondrial in origin was confirmed by addition of the inhibitor

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(Fig 3D), suggesting that the endocytic pathway is not

required for the uptake of CoQ2 to the mitochondria

and, therefore, this pathway is unlikely to be

responsi-ble for discriminating between decylQ and CoQ2 That

a vesicle-based mechanism is not required for the

uptake is reasonable as the octanol⁄ PBS partition

coef-ficients of CoQ2( 104.5) and decylQ ( 105.5) are

sev-eral orders of magnitude lower than that of CoQ6

[6,10]

This suggests that CoQ2 is sufficiently hydrophilic

that it can passively equilibrate quite effectively within

cells over the 48 h timeframe of the growth

experi-ments To test this we measured whether yeast

sup-plied with ubiquinone exogenously contained a

significantly lower concentration of decylQ than that

of CoQ2 or whether the accumulation of decylQ was

slower than for CoQ2 To limit complications due to

growth, a DCoQ culture was maintained in YPGG for

24 h, at which point they had consumed the available

glucose and their growth had arrested In the

contin-ued absence of exogenous CoQ, the absorbance of this

culture remained stable, with no evidence of growth

for several days (Figs 1A and 3B) Upon the addition

of a mixture of CoQ2 and decylQ it took 3–6 h for

growth to restart (Fig 3B) To determine if there were

differences in the accumulation of CoQ2 and decylQ

we measured the amount of the two ubiquinone

ana-logues in the cell pellets From 30 min up to 48 h

after the addition of a mixture of 10 lm CoQ2 and

10 lm decylQ to a yeast suspension, the ratio of

decylQ to CoQ2 in the pellet remained very similar

(Fig 3C) Although a difference was observed at a

very early 2 min time point, the equilibration rate of

both was relatively rapid in the context of the 48 h

experiments where decylQ failed to restore growth

This suggests that the association of CoQ2and decylQ

with yeast cells is similar, despite decylQ not being

able to complement nonfermentative growth in DCoQ

yeast

If more subtle differences in diffusion of decylQ to

mitochondria within cells were responsible, it might be

possible to restore growth with decylQ by adding a

large excess of it to DCoQ yeast Therefore we

mea-sured the ability of DCoQ yeast to grow on glycerol

with concentrations of decylQ up to 100 lm No

non-fermentative growth was observed with decylQ, even

when its concentration was up to 50- and 250-fold

higher than that required to observe such growth with

CoQ2 and CoQ4, respectively (Fig 3D) However,

there was a small, but significant, increase in the

absorbance of the culture when concentrations of

decylQ as low as 2 lm were added relative to the

dim-ethylsulfoxide carrier alone, suggesting decylQ induced

some change in the culture The small increase was also observed with 10 lm of the ineffective CoQ2 analogues A4-Q2, A5-Q2 and A6-Q2, as well as with CoQ1, but it was not observed with 10 lm of CoQ9 or CoQ10 (data not shown) The lack of any concentra-tion dependence when an excess of decylQ was sup-plied exogenously suggests that its failure to complement nonfermentative growth in DCoQ yeast does not result from subtle differences in diffusion, particularly given that the hydrophobicity of decylQ is intermediate between CoQ2and CoQ4

To demonstrate that the physiochemical properties

of decylQ and CoQ2 are grossly similar, we measured their ability to diffuse between noncontiguous mem-branes by mixing two populations of vesicles Both populations of vesicles contained equal concentrations

of the very hydrophobic fluorophore, 1-pyrene hexa-decanoic acid (Pyr16), but only the second population contained ubiquinone Ubiquinone collisionally quenches pyrene fluorescence if both are present in the same membrane system and are capable of physical interaction [12] There is a linear relationship between ubiquinone concentration and I0⁄ I – 1, where I0is the initial fluorescence and I is the fluorescence after the addition of ubiquinone, and for a typical ubiquinone

in our hands the slope of this line is 24 mm)1 [12] Using this value we would expect a decrease in relative fluorescence (I⁄ I0) from 1 to 0.83 upon the addition of ubiquinone-containing vesicles because the initial unquenched vesicle population (2 mL) is being diluted with a highly quenched second vesicle population con-taining ubiquinone (500 lL) This drop does not occur

if the added vesicle population does not contain ubi-quinone (data not shown) We would then expect a further decrease in relative fluorescence to 0.51 if the ubiquinone is able to equilibrate between bilayers because 40 lm ubiquinone in 100% of vesicles will quench total fluorescence more effectively than 200 lm ubiquinone in 20% of vesicles The exchange of ubi-quinone between the two populations of vesicles can

be seen most easily using CoQ4, as there is a decrease

in relative fluorescence from 0.77 to 0.57 over the course of the experiment (Fig 3E) When the second population of vesicles was reconstituted with the rela-tively more hydrophilic analogues, CoQ2 or decylQ, quenching was largely complete within seconds and after approximately 10 min relative fluorescence was

 0.57 for both analogues, suggesting they are free to exchange between the phospholipid bilayers of the two vesicle populations (Fig 3E) If the experiment was repeated with the very hydrophobic analogue, CoQ9, there was no evidence of movement between the two populations during the course of the 10 min

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incubation, as relative fluorescence remained stable at

0.82 (Fig 3E) Therefore, both CoQ2 and decylQ

dif-fuse rapidly between the noncontiguous membranes of

the two vesicle populations, taking only a few seconds

to equilibrate This is consistent with the rapid

equili-bration of both CoQ2 and decylQ within cells

(Fig 3C)

Finally, we sought to confirm that decylQ and CoQ2

both reach mitochondria in intact cells directly

DecylQ and CoQ2 participate effectively in respiration

by isolated mitochondria in the absence of endogenous CoQ6 (Fig 2) Therefore, if they can migrate to mito-chondria their redox state should be sensitive to com-pounds that manipulate the mitochondrial respiratory chain To test this we incubated intact yeast cells with either decylQ or CoQ2for 3 h, after which we exposed them to the either cyanide (KCN) or carbonylcyanide-p-trifluoromethoxy-phenylhydrazone (FCCP) and ex-tracted the ubiquinone KCN inhibits complex IV and leads to a reduced ubiquinone pool as electrons can no

Ubiquinone concentration (µ M )

0 1 2 3 4 5

Ubiquinone concentration (µ M )

0 1 2 3 4 5

Time (h)

A600

0 5 10 15 20 25

decylQ

A600

Time (h)

Δcoq2Δerg2 Δcoq2Δtlg2

0 1 2 3 4 5 6 7 8

decylQ

A600

A600

0 5 10 15 20 25

0 1 2 3 4 5 6

(h)

A600

E

CoQ2/decylQ

Ubiquinone-loaded vesicles

I/ 0

0 10 20 30 40 50 60 70 80

Ubiquinone

FCCP KCN

Time (min)

F

0.4 0.5 0.6 0.7 0.8 0.9 1 1.1

CoQ2

CoQ4 CoQ 9

decylQ

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longer flow to O2, whereas FCCP uncouples

mitochon-dria, increasing respiration and oxidizing the

ubiqui-none pool as electrons rapidly flow from ubiquiubiqui-none

to O2 in the absence of a membrane potential [6]

Although CoQ2 responded as expected, surprisingly

decylQ was not significantly reduced in the presence of

KCN (Fig 3F) In the absence of FCCP or KCN, the

ratio of reduced to oxidized ubiquinone was similar to

that in the presence of FCCP (data not shown)

In summary, the presence of a sufficient

concentra-tion of ubiquinone in the mitochondrial membrane is

required for growth on glycerol The above results

sug-gest that CoQ2and decylQ are both sufficiently

hydro-philic that they can rapidly equilibrate between

noncontiguous membranes through the aqueous phase

without this movement being mediated by a

hydropho-bic phase such as a vesicle This makes it unlikely that

the difference between CoQ2 and decylQ is due to

dif-ferences in their ability to diffuse to mitochondria

within cells Paradoxically, decylQ was largely

insensi-tive to a mitochondrial inhibitor that should have led

to a reduction in decylQ in the presence of a substrate

The reason for this discrepancy remains unclear and

will be discussed later

DecylQ is unlikely to be exported by yeast cells

Above we dealt with the possibility that CoQ2is

selec-tively transported to mitochondria within cells The

converse could equally have been true and decylQ could have been selectively excluded by yeast cells This is because yeast can remove foreign molecules, primarily by using ATP binding cassette (ABC) trans-porters, as a mechanism for protecting themselves from toxins [13] Selective exclusion of decylQ from the cell appears unlikely, as the amount of decylQ associated with the yeast cell pellet is similar to that of CoQ2 (Fig 3C), the putative export machinery cannot

be overwhelmed by high concentrations of decylQ (Fig 3D) and the structures that fail to restore growth are less similar than the ones that do restore growth, implying selective recognition of CoQ2 rather than of decylQ (Fig 1B)

DecylQ is not toxic, but does confound growth complementation by CoQ2

The previous experiments suggest that decylQ can migrate to mitochondria within cells Therefore, it is possible that decylQ is in some way toxic to yeast cells and thereby prevents nonfermentative cell growth, despite complementing respiration (Fig 2) To deter-mine if this was the case, we added increasing equimo-lar amounts of CoQ2 and decylQ to DCoQ cultures and measured their growth in YPGG over 48 h (Fig 4A) The addition of equimolar decylQ to CoQ2 had no significant detrimental effect on growth com-pared with CoQ2 alone Therefore, decylQ appears to

ubiquinone was subtracted to give the growth achieved on glycerol (B) Time lag in the growth response of stationary phase yeast to

hexane from both the supernatant and the pellet They were separated by HPLC and quantitated using electrochemical detection The values

Data are the mean ± standard error of the mean of three independent experiments (D) Even a 50-fold excess of decylQ above that required

The growth of the BY4743Dcoq2 strain in the absence of ubiquinone was subtracted to give the growth achieved on glycerol (E) DecylQ

expressed as the relative amount of fluorescence at a given point in time (I) to initial fluorescence just before the addition of

of three independent experiments.

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be nontoxic We next reasoned that because decylQ

should diffuse to mitochondria (Fig 3) and once there

function effectively in oxidative phosphorylation

(Fig 1D), that decylQ might be unable to restore

growth because CoQ2 is required in trace amounts for

some secondary function To determine if this was the

case, we added increasing amounts of decylQ

(0–100 lm) in combination with 5 lm CoQ2 to DCoQ

cultures in YPGG and measured their growth over

48 h This showed that trace amounts of CoQ2did not allow decylQ to complement growth (Fig 4B) In fact, rather surprisingly, at higher concentrations decylQ largely inhibited the growth that would have been observed with 5 lm CoQ2 alone (Fig 4B) Interest-ingly, there was a strong correlation between the frac-tion of the total added ubiquinone present as CoQ2 and growth (Fig 4C; r2= 0.99) Thus, it would appear that decylQ in some way prevents CoQ2 inter-acting with a protein that is required for nonfermenta-tive growth This might occur via decylQ binding directly to the protein and preventing CoQ2interacting

or growth could require an interaction with the reduced or the oxidized form of CoQ2 and decylQ dis-turbs the normal CoQ2H2⁄ CoQ2ratio

In summary, decylQ is not toxic, but can antagonize the stimulation of growth by CoQ2, especially when its concentration markedly exceeds that of CoQ2 This suggests that there may be a protein that regulates growth that can bind to CoQ2and that decylQ antago-nizes this interaction in some way

Screen for proteins influencing CoQ-dependent growth in yeast

The data so far suggest that there is a protein that can distinguish between CoQ2 and decylQ that is essential for growth on nonfermentable substrates The nature

of this CoQ–protein interaction is unclear, as we have been unable to identify an obvious secondary role for CoQ in growth on nonfermentable substrates from the literature To investigate this interaction further we set

up a screen to identify potential ORFs that might be involved in CoQ-dependent growth in yeast The yeast

0

1

2

3

4

5

6

CoQ2 CoQ2 and equimolar decylQ

A600

0

1

2

3

4

5

6

X µ M decylQ + X µ M CoQ 2

X µ M decylQ + 5 µ M CoQ2

X µ M decylQ

Ubiquinone concentration (X µM)

A

B

0

0.5

1

1.5

2

2.5

([CoQ2] / [CoQ2+decylQ])

C

A600

A600

Fig 4 DecylQ is not toxic, but does interfere with growth

stimulated nor inhibited by the presence of equimolar decylQ The BY4743Dcoq2 strain was grown in YPGG supplemented with either

Data are the mean ± standard error of the mean of three indepen-dent experiments The growth of the BY4743Dcoq2 strain in the absence of ubiquinone was subtracted to give the growth achieved

BY4743Dcoq2 strain was grown in YPGG with either decylQ alone

growth of the BY4743Dcoq2 strain in the absence of ubiquinone was subtracted to give the growth achieved on glycerol (C) Growth

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S cerevisiae contains  6000 ORFs, of which  5000

can be deleted with the strain still viable To do this

we first created a double-knockout library unable to

synthesize CoQ by crossing a strain in which a gene

required for the endogenous synthesis of CoQ6 (coq2)

was deleted with a commercial library containing

 4800 strains each harbouring a deletion in a single

nonessential gene To generate a stable haploid

double-knockout library we utilized an approach

developed by Tong and coworkers [14] The stability

arises because BY4743Dcoq2 contains a histidine

synthesis gene under the control of a mating-type ‘a’

promoter (MFA1pr-HIS3) inserted into the middle of

an arginine permease gene (CAN1) This allows

mating-type ‘a’ progeny to be selected without cloning

For details on how the double-knockout library was

created, see the Materials and Methods section

After its creation, the Dcoq2DORF strains in the

double-knockout library were screened for their ability

to grow in liquid YPGG when supplemented

exoge-nously with 100 lm CoQ2 This identified 379

double-knockout strains that could not grow on YPGG

containing CoQ2(Fig 5A) Of these, 324 were not

con-sidered further for three reasons First, theDcoq2DORF

strain had grown in liquid YPGG in the absence of

CoQ2, suggesting they may not be DCoQ (Fig 5B)

Second, the corresponding DORF strain failed to grow

on solid YPG, indicating that the ORF deleted in the

strain was a gene essential for nonfermentative growth, e.g a nuclear-encoded respiratory chain complex sub-unit (Fig 5C) Finally, they did not produce viable progeny after mating and sporulation on the final selective plate Therefore, there was no Dcoq2DORF strain to test for dependence on exogenous CoQ (Fig 5D) The remaining 55 strains potentially har-boured a deletion that removes a gene that influences growth on exogenous CoQ Consequently, these strains were cloned and analysed further to confirm whether they respond abnormally to exogenous CoQ

Confirmation of double-knockout strains with poor growth on exogenously supplied CoQ

An inoculation from each of the 55 Dcoq2DORF strains identified from the screen were grown in 3 mL YPGG and 10 lm CoQ4 In addition, nine control Dcoq2DORF strains were selected to control for the mating and sporulation (see Materials and Methods) After 48 h their growth was measured and the 24 strains with decreased growth relative to the BY4743Dcoq2 parental strain and the nine Dcoq2-DORF control strains were cloned, as were their Dcoq2-DORF counterparts from the original commercial library All

of these Dcoq2DORF strains grew by fermentation in YPD (yeast extract, peptone, glucose) to an A600 of

 10 (data not shown) and in YPGG without CoQ4to

Fig 5 Creation of the double-knockout

library (A, B) Double-knockout Dcoq2DORF

strains grown in liquid glycerol media (YPG)

supplemented with canavanine, G418 and

strains grown on solid YPG (D)

Double-dele-tion Dcoq2DORF strains grown in solid

glucose-based synthetic media lacking

histi-dine and arginine and supplemented with

canavanine, G418 and cloneNAT The red

squares indicate strains that failed to give

information about whether the deleted ORF

is or is not involved in CoQ-dependent

growth Unmarked strains have exogenous

CoQ-dependent growth and are

uninterest-ing, whereas black squares indicate the

desired combination of phenotypes and

these were analysed further The top left

black square is Srb8 and the bottom right is

Kcs1.

Trang 10

an A600 of  0.8 (data not shown) Therefore, their

utilization of glucose for growth was grossly normal

and they were unable to make the transition to the

uti-lization of glycerol as a carbon source in the absence

of CoQ This can also be seen in the slightly positive

values obtained for growth in YPGG containing

10 lm CoQ4, as a failure to grow on glucose would

lead to negative values (Table 1)

These 24 Dcoq2DORF clones were grown in glass

tubes with 3 mL YPGG and 10 lm CoQ4 for 48 h at

30C The growth of 21 of these fell below the range

of the nine Dcoq2DORF control strains and the

BY4743Dcoq2 parental strain To ensure the defect in

growth was related to an inability to utilize exogenous

CoQ and not related to a general defect in

mitochon-drial metabolism, the corresponding DORF strains

were grown in glass tubes with 3 mL YPGG for 48 h

at 30C For five of the remaining ORFs, poor

growth of the Dcoq2DORF strain could be sufficiently

explained by similar poor growth of their DORF

strain This left 16 ORFs where the Dcoq2DORF strain

could not grow as well when supplemented with

exoge-nously supplied CoQ4 as any of the nine control

Dcoq2DORF strains yet contained mitochondria that

were at least partially functional in their DORF strain

(Table 1) Unlike the control Dcoq2DORF strains, for

many of the potentially interesting Dcoq2DORF strains

the counterpart DORF strain had reduced growth on

glycerol (Table 1) However, when the growth of the

Dcoq2DORF strain is expressed as a percentage of that

of its corresponding single knockout, it is apparent

that any decrease in growth on glycerol in the DORF

strain is compounded by the introduction of a Dcoq2

deletion In summary, we have identified 16 ORFs, the

deletion of which dramatically decreased the ability of

exogenous CoQ to support growth on glycerol in the

absence of an ability to synthesize endogenous CoQ

Discussion

Yeast lacking the ability to synthesize CoQ

endoge-nously require exogenous CoQ to grow on

nonfer-mentable substrates Utilizing several very similar

analogues of CoQ2 we have shown that exogenous

supplementation of decylQ and all analogues lacking

the first double bond of the side chain failed to

sup-port the growth of DCoQ yeast on glycerol This

sug-gests that there is very selective structural recognition

of elements within the side chain, presumably by a

protein, and that this interaction is required to

com-plete the diauxic shift to nonfermentable substrates

(Fig 1) The most obvious level at which this

struc-tural recognition could occur is mitochondrial

respira-tion However, isolated mitochondria lacking CoQ respired normally on glycerol-3-phosphate when sup-plemented with all CoQ2 analogues tested (Fig 2) This suggests that the selectivity does not arise from the respiratory chain complexes

The next possibility is that the protein of interest is involved in the transport of exogenous CoQ from the extracellular medium into the cell and on to mitochon-dria, and that decylQ and the analogues A4-Q2, A5-Q2 and A6-Q2are not taken up correctly by this pathway Deletion of two endocytic proteins shown to be involved in the transport of exogenous CoQ6 to mito-chondria [11] failed to prevent growth of DCoQ yeast

on glycerol (Fig 3A), suggesting that this pathway is not an absolute requirement for the uptake of more hydrophilic CoQ analogues Consistent with this, CoQ2 and decylQ reached apparent equilibrium within minutes of being added to a culture of DCoQ yeast (Fig 3C) Even though 2 lm CoQ2 led to appreciable growth, 100 lm decylQ did not, suggesting that uptake

of decylQ to mitochondria is not the factor limiting growth Furthermore, mixing populations of vesicles containing fluorescent pyrenes with vesicles containing decylQ, CoQ2 and CoQ4 indicated rapid redistribution

of decylQ and CoQ2 over a timeframe of seconds and CoQ4 within minutes (Fig 3E) CoQ2and decylQ were also rapidly lost into the bulk aqueous phase during cell subfractionation or cell pellet washing steps (unpublished observations) Together this suggests that even though decylQ and CoQ2 are hydrophobic, they are not hydrophobic enough to be retained within a membrane system over the 48 h course of the growth experiments and presumably equilibrate throughout all the lipid bilayers of the cell

The experiments outlined above suggest that enough CoQ2 diffuses around blocks in the endocytic pathway used to transport CoQ6 to support growth on nonfer-mentable substrates (Fig 3A) However, even though

it appears possible for CoQ2 to diffuse to mitochon-dria, growth on glycerol with exogenously supplied CoQ2 is significantly slower than that observed on endogenous CoQ6 (Fig 1) One reason for the dimin-ished growth on exogenous CoQ2 relative to endoge-nous CoQ6 could be because the concentration of CoQ

in mitochondrial membranes appears significantly higher than that of other cellular membranes [11] Because CoQ2 could redistribute between bilayers (Fig 3E) there may be no way of maintaining an ele-vated mitochondrial CoQ concentration, thereby resulting in suboptimal growth Alternatively, it could arise from a decrease in substrate concentration for an enzyme because CoQ2 cannot be accumulated and retained in a membrane system or via a decrease in

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