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In an attempt to clarify this point, we examined the process in Saccharomyces cerevisiae cells synthesizing cytochrome P450scc as its native precursor pCYP11A1 or versions in which its N

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Effects of various N-terminal addressing signals on sorting

and folding of mammalian CYP11A1 in yeast mitochondria

Irina E Kovaleva, Lyudmila A Novikova, Pavel A Nazarov, Sergei I Grivennikov

and Valentin N Luzikov

Belozersky Institute of Physico-Chemical Biology, Lomonosov Moscow State University, Moscow, Russia

Topogenesis of cytochrome P450scc, a resident protein of the

inner membrane of adrenocortical mitochondria, is still

obscure In particular, little is known about the cause of its

tissue specificity In an attempt to clarify this point, we

examined the process in Saccharomyces cerevisiae cells

synthesizing cytochrome P450scc as its native precursor

(pCYP11A1) or versions in which its N-terminal addressing

presequence had been replaced with those of yeast

mitoch-ondrial proteins: CoxIV(1–25) and Su9(1–112) We found

the pCYP11A1 and CoxIV(1–25)-mCYP11A1 versions to

be effectively imported into yeast mitochondria and

subjec-ted to proteolytic processing However, only minor portions

of the imported proteins were incorporated into

mito-chondrial membranes, whereas their bulk accumulated as

aggregates insoluble in 1% Triton X-100 Along with

pre-viously published data, this suggests that a distinguishing

feature of the import of the CYP11A1 precursors into yeast

mitochondria is their easy translocation into the matrix

where the foreign proteins mainly undergo proteolysis or aggregation The fraction of CYP11A1 that happens to be inserted into the inner mitochondrial membrane is effectively converted into the catalytically active holoenzyme Experi-ments with the Su9(1–112)-mCYP11A1 construct bearing a re-export signal revealed that, after translocation of the fused protein into the matrix and its processing, the Su9(67–112) segment ensures association of the mCYP11A1 body with the inner membrane, but proper folding of the latter does not take place Thus it can be said that the most specific stage of CYP11A1 topogenesis in adrenocortical chondria is its confinement and folding in the inner mito-chondrial membrane In yeast mitochondria, only an insignificant portion of the imported CYP11A1 follows this mechanism

Keywords: yeast mitochondria; import; sorting; folding; aggregation

Cytochrome P450sccis a resident protein of adrenocortical

mitochondria In co-operation with adrenodoxin and

adrenodoxin reductase it carries out conversion of

choles-terol into pregnenolone Cytochrome P450sccis synthesized

in the cytoplasm as a precursor [1] that is imported into

mitochondria, where it becomes an integral protein of the

inner membrane [2] At least two peculiarities of its

topogenesis are still unclear First, this protein cannot be

extracted from the membrane by carbonate treatment,

although it does not contain any distinct transmembrane

domains Second, the import of the cytochrome P450scc

precursor into mitochondria is tissue-specific In fact,

pCYP11A1 is imported into adrenocortical and liver

mito-chondria but not into heart mitomito-chondria [3,4] On the other

hand, the import of various versions of pCYP11A1 has been

demonstrated with mitochondria of COS-1 [5] and yeast [6] cells Moreover, even plant mitochondria can import pCYP11A1 [7] Mitochondria of transformed yeast cells exhibit side-chain cleavage activity in reconstituted systems, the activity being detected in the inner membrane fraction [6,8] These data seem to suggest similar topogenesis of CYP11A1 in various organisms However, no details of its import into heterologous mitochondria have been studied in the above publications Efforts in this direction were undertaken in the experiments with isolated yeast mitochon-dria and in vitro synthesized bovine pCYP11A1 [9] It turned out that upon import into mitochondria the protein accu-mulates mainly in the matrix in precursor and mature forms Only a trace amount of the protein was found in the inner membrane fraction This was in discord with the earlier data [10] according to which pCYP11A1 does not leave the inner membrane upon its import into isolated adrenocortical mitochondria and subsequent maturation Thus, the crucial distinction of the import of pCYP11A1 into yeast mito-chondria is that, for unknown reasons, the protein is rapidly translocated into the mitochondrial matrix The imported protein largely undergoes proteolysis by matrix protease Pim1p, which additionally testifies to such translocation [9] Proteolysis competes with aggregation dominating in mito-chondria with mutant forms of Pim1p or mtHsp70 In contrast to pCYP11A1, the Su9(67–112)-CYP11A1(75–481) version was detected in a considerable amount in the inner membrane fraction where it was directed by the re-export

Correspondence to I E Kovaleva, Belozersky Institute of Physical

and Chemical Biology, Moscow State University,

119899 Moscow, Russia Fax: + 7 095 939 3181,

E-mail: kovaleva@genebee.msu.su

Abbreviations: pCYP11A1, precursor form of CYP11A1;

mCYP11A1, mature form of CYP11A1; SMP,

submito-chondrial particles.

Enzymes: CYP11A1 (EC 1.14.15.6); adrenodoxin reductase

(EC 1.14.15.4).

(Received 26 March 2002, revised 6 November 2002,

accepted 20 November 2002)

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mechanism [11] using the N-terminal transmembrane

domain of the Su9 protein The Su9(1–112) segment is

known to ensure the initial stage of the import of Su9 into the

matrix followed by two-stage processing and reinsertion of

its N-terminus into the inner membrane in such a way that

the transmembrane domain spans the membrane while the

14-mer extension becomes exposed into the intermembrane

space The Su9(1–112) sequence fused to N-termini of some

foreign proteins is capable of carrying out the same transport

function [12] The membrane-bound

Su9(67–112)-CYP11A1(75–493) form was degraded by the membrane

Yta10p-12p proteolytic complex [9], which testifies to its

improper folding Unfortunately, the in vitro experiments

could not provide information about conversion of

CYP11A1 into a catalytically active holoenzyme Using

Saccharomyces cerevisiae yeast expressing CYP11A1

ver-sions (CYP11A1s) with different topogenic signals, in this

work we planned to answer the following questions: (a) how

do various topogenic signals influence the sorting of

CYP11A1s in yeast mitochondria; (b) what is the relation

between the contents of membrane-bound and aggregated

forms of the imported CYP11A1 and (c) how effective is the

conversion of membrane-bound CYP11A1 into the

cataly-tically active form? Such information revealing specific

features of CYP11A1 import into foreign mitochondria

might be helpful both for understanding the topogenesis of

this protein in nature and for assessing its possibilities in

transgenic organisms

Materials and methods

Media for cultivation of yeast and bacteria were from Difco

Laboratories (USA) 22(R)-hydroxycholesterol and

d-ami-nolevulinic acid were from Sigma (USA) Restriction

endo-nucleases, Klenow fragment, and T4 DNA ligase were from

Fermentas (Lithuania) Plasmids were maintained and

am-plified in Escherichia coli JM-109 (Promega, USA) cDNAs

encoding recombinant proteins were used for transformation

of Saccharomyces cerevisiae 2805 (aMATpep4::HIS3 prb1-d

can 1 GAL2 His3 ura3–5) deficient in vacuolar proteases

Rabbit polyclonal anticytochrome P450sccIgG,

adrenocor-tical mitochondria, purified bovine cytochrome P450scc,

adrenodoxin, and adrenodoxin reductase were generous gifts

from V M Shkumatov (State University of Belarus, Minsk)

DNAs and plasmids

Yeast expressing the shuttle vector pYeDP1-8/2 (pYeDP)

[13] was used for expression of cDNAs encoding

recombin-ant proteins This vector includes a galactose-inducible

chimeric promoter GAL10-CYC1, a terminator of yeast

phosphoglycerate kinase, and an auxotrophic marker

URA3 The pYeDP/Cox(1–25)-mCYP11A1 with the

pre-sequence of subunit IV of yeast cytochrome oxidase has

been prepared earlier in this laboratory [6] The pYeDP/

pCYP11A1 encoding CYP11A1 with its own N-terminal

addressing presequence was constructed by inserting cDNA

for human CYP11A1, a gift from W L Miller (University

of California, San Francisco, USA), into pYeDP at the

EcoRI and KpnI sites cDNA for a fusion protein composed

of mCYP11A1 preceded by the N-terminal fragment of

subunit 9 (Su9) of yeast F-ATPase, including its addressing

presequence and the N-proximal transmembrane segment, was constructed on the basis of pGEM4/Su9(1–112)-CYP11A1(75–493) [9] Full-size cDNA for mCYP11A1 was obtained by substituting the NcoI-BglII fragment of pTrc99A/mCYP11A1 [14] for the BamHI-BglI fragment in pGEM4/Su9(1–112)-CYP11A1(75–493) Prior to ligation, the BamHI end of the plasmid and the NcoI end of the excised cDNA were treated with Klenow fragment Hybrid cDNA encoding the Su9(1–112)-mCYP11A1 fusion protein was excised from pGEM4/Su9(1–112)-mCYP11A1 at EcoRI and KpnI sites and inserted into pYeDP pretreated with BamHI and KpnI The BamHI and EcoRI ends were treated with Klenow fragment prior to ligation The resulting pYeDP/Su9(1–112)-mCYP11A1 plasmid was used

to transform yeast cells To achieve low-copy expression of CoxIV(1–25)-mCYP11A1, the corresponding cDNA was inserted into pINT2 (an integrative vector with phospho-glycerate kinase promoter and URA3 marker; kindly pro-vided by D G Kozlov, Institute of Genetics and Selection

of Industrial Microorganisms, Moscow, Russia) by excising this cDNA from pYeDP/CoxIV(1–25)-mCYP11A1 with EcoRI and XbaI and subcloning it sequentially in pUC19 and pUC18 to obtain appropriate ends for insertion into pINT2 at BamHI and EcoRI sites Final ligation gave the pINT2/CoxIV(1–25)-mCYP11A1 construct

Transformation of yeast cells with pYeDP/pCYP11A1, pYeDP/Cox(1–25)-mCYP11A1, and pYeDP/Su9(1–112)-mCYP11A1 was carried out as described earlier [15] The yeast cells were grown in SD medium (0.67% yeast nitrogen bases, 1% ammonium sulfate and 2% glucose) for 24 h, and then for 14 h in a selective medium with 2% galactose added for promoter induction After this, the growth medium was also supplemented with 200 lMd-aminolevulinic acid

Isolation and fractionation of yeast mitochondria Yeast mitochondria were isolated as described in [16] and fractionated according to [4] with minor modifications Mito-chondria were suspended in 5 mMTris/HCl, pH 7.4, incu-bated for 20 min at 4°C to disrupt the outer membranes, and subjected to sonic disintegration The mitochondrial suspen-sion was then centrifuged for 15 min at 12 000 g The resulting suspension was centrifuged for 1 h at 106 000 g to obtain the submitochondrial particles (SMP) fraction

Assessment of aggregation of recombinant proteins

in yeast mitochondria Mitochondria (0.5 mg protein mL)1) were incubated in

20 mM Hepes/KOH, pH 7.4, with 150 mM NaCl, 1 mM

phenylmethyl sulphonyl fluoride, and 1% Triton X-100 for

30 min on ice The suspension was then centrifuged for

15 min at 25 000 g The supernatant proteins were precipi-tated with 10% (v/v) trichloroacetic acid, and the pellets were dissolved in SDS/PAGE buffer The content of CYP11A1 was estimated in Triton X-100 soluble and insoluble fractions by Western blot analysis

Western blot analysis

To estimate the contents of recombinant CYP11A1 ver-sions in mitochondria and 12 000 g supernatants from

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mitochondrial homogenates, we used cytochrome P450scc

-calibrated immunoblotting For this purpose, SDS/PAGE

was carried out with fixed amounts of isolated cytochrome

P450sccin 2.5–20 or 25–200 ng ranges, with the contents of

the protein in adjacent lanes differing by a factor of two

Then routine procedures were used for transferring the

proteins to nitrocellulose membranes [17] and their

detec-tion with rabbit anticytochrome P450sccIgG and conjugate

of anti-rabbit IgG with peroxidase This approach allowed

one to estimate the content of various CYP11A1 versions

with an accuracy of ± 50%

Assays of cholesterol side-chain cleaving activity

of various CYP11A1 versions

Mitochondria isolated from yeast cells expressing CYP11A1

versions were subjected to hypotonic shock and sonication

as indicated above The assays were carried out with

supernatants after centrifugation of mitochondrial

homo-genates at 12 000 g, which removed large mitochondrial

fragments and protein aggregates The reaction mixture

contained 200–500 lg of mitochondrial protein, purified

bovine adrenodoxin (0.8 nmole), adrenodoxin reductase

(0.2 nmole), and 25 nmoles of 22(R)-hydroxycholesterol in

0.5 mL of 30 mMphosphate buffer, pH 7.5, supplemented

with 0.05% (v/v) Tween 20 The samples were preincubated

for 20 min at 25°C The reaction was started by addition of

an NADPH-generating system including 0.1 mMNADPH,

5 mMglucose-6-phosphate, and glucose-6-phosphate

dehy-drogenase (1 UÆmL)1) (Serva, Germany) The reaction was

stopped after 30 min by plunging the samples into boiling

water for 15–20 s Then the samples were mixed with 3 mL

of 100 mMsodium phosphate buffer, pH 7.2, with 0.05%

(v/v) Tween 20, and cholesterol oxidase (0.1 U per sample)

(Serva, Germany) was added to convert pregnenolone

formed from 22(R)-hydroxycholesterol into progesterone

After a 45-min incubation at 37°C, the samples were

treated with ethyl acetate to extract the steroids The

extracts were evaporated to dryness and the content of

progesterone was determined with an ELISA test system

based on antiprogesterone antibodies The test kits were

kindly provided by Dr A G Pryadko (Institute of

Bioorganic Chemistry, Belarus)

Results

Import of recombinant forms of CYP11A1 into yeast

mitochondriain vivo

cDNAs encoding pCYP11A1, CoxIV(1-25)-mCYP11A1,

and Su9(1–112)-mCYP11A1 have been inserted into the

yeast expressing shuttle vector pYeDP CoxIV(1–25) is the

N-terminal addressing presequence of subunit IV of yeast

mitochondrial cytochrome oxidase, and Su9(1–112) is the

N-terminal part of the precursor of subunit 9 of yeast

mitochondrial Fo-ATPase Yeast cells expressing the above

recombinant proteins were used to isolate mitochondria in

which various forms of CYP11A1 were detected by

immunoblotting with anti-P450sccIgG Figure 1 shows that

all the CYP11A1 forms were imported into mitochondria

As we failed to register CO difference spectra of yeast

mitochondria containing the recombinant proteins, we have

estimated their intramitochondrial contents by cytochrome P450scc-calibrated immunoblotting The values obtained for CoxIV(1–25)-mCYP11A1, Su9(1–112)-mCYP11A1, and pCYP11A1 were 0.3%, 0.5% and 0.1% of total mito-chondrial protein, respectively

Appreciable distinctions were found at the stage of processing of the imported proteins (Fig 1) All of Su9(1)112)-mCYP11A1 is processed to a single product somewhat larger than mCYP11A1 because of the 45-mer addition at the N-terminus CoxIV(1–25)-mCYP11A1 is also effectively processed, although immunoblotting reveals some precursor in yeast mitochondria Similar results have been obtained upon expression of CYP11A1 with the presequence of subunit VI of yeast cytochrome oxidase [8]

A more complicated pattern was observed for pCY-P11A1 with its own N-terminal presequence In this case one could see three forms of the protein, i.e precursor, mature protein and semiprocessed precursor The lower content of mCYP11A1 might have resulted from the poor match of the yeast mitochondrial processing peptidase to the indigenous processing site in pCYP11A1

Fig 1 Western blots of mitochondria prepared from recombinant yeast strains producing pCYP11A1, CoxIV(1– –25)-mCYP11A1, and Su9(1– –112)-mCYP11A1 The samples of mitochondria isolated from recombinant yeast producing CoxIV-mCYP11A1, pCYP11A1, and Su9-mCYP11A1 were probed with anti-(cytochrome P450 scc ) IgG Mitochondria from these strains were loaded at 25, 25, and 10 lg protein per lane, respectively Purified bovine cytochrome P450 scc was

a marker at 20 and 500 ng protein per lane for the left and right panels, respectively In the scheme, p, i, and m correspond to precursor, intermediate, and mature forms of CYP11A1 Light and dark shaded boxes are targeting signal (ts) and mature protein sequence, respect-ively; empty box is the transmembrane domain (tm) of the Su9 precursor.

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In as much as in the three above cases the mitochondrial

fraction mainly contained mature or semiprocessed forms of

CYP11A1, one may assume that this fraction was not

significantly contaminated with the recombinant protein in

a cytoplasmic aggregated form

Relationship between aggregated and

membrane-bound forms of CYP11A1 in yeast mitochondria

Obviously, early post-translocational changes of a protein

imported into mitochondria can be revealed only in the

short-term in vitro experiments It has been shown [9] that

upon import of the in vitro synthesized pCYP11A1 into

isolated yeast mitochondria, only a minor portion of the

protein remains in the inner membrane, whereas its bulk is

transferred into the matrix where it undergoes proteolysis or

aggregation Therefore, expressing CYP11A1 with various

addressing presequences in yeast cells, one would like to

know the relationship between aggregated and

membrane-bound forms of the protein imported into mitochondria, in

particular, to estimate the efficiency of conversion of the

imported protein into a catalytically active form in foreign

surroundings

In our experiments, mitochondria isolated from the

transformed yeast cells after 14-h induction of pCYP11A1,

CoxIV(1–25)-mCYP11A1, and Su9(1–112)-mCYP11A1

were subjected to hypotonic shock followed by sonication

and centrifugation at 12 000 g Figure 2 shows the contents

of CoxIV(1–25)-mCYP11A1 in whole mitochondria and

the corresponding 12 000 g supernatant determined by

SDS/PAGE and Western blotting of the samples containing

equal amounts of total protein According to these data, the

content of the imported protein in the supernatant was

0.025% of total protein of the fraction, whereas that in

mitochondria was approximately 0.3% of total protein

Obviously, the supernatant containing soluble

mitochond-rial proteins and membrane vesicles had 10 times less

mCYP11A1 In contrast to mCYP11A1, the specific

content of phosphate carrier, a marker protein of the inner

mitochondrial membrane, was almost the same in whole

mitochondria and in the 12 000 g supernatant (Fig 2) This suggests that CoxIV(1–25)-mCYP11A1 imported into yeast mitochondria does not accumulate in the inner membrane after its processing

Figure 2 shows also the data for the pCYP11A1 version expressed in yeast In this case the difference in the combined contents of pCYP11A1 and iCYP11A1 in mitochondria and the 12 000 g supernatant was not so great as for the CoxIV(1–25)-CYP11A1 version (0.1% in mitochondria vs 0.05% in the supernatant) It is remark-able that the 12 000 g supernatant does not contain a detectable amount of mCYP11A1 Perhaps, its content in whole mitochondria is too low to be detected in the supernatant

Unlike the above experiments with CoxIV(1–25)-mCY-P11A1 and pCYCoxIV(1–25)-mCY-P11A1 constructs, fractionation of mito-chondria containing Su9(1–112)-mCYP11A1 yielded a

12 000 g supernatant with a considerable amount of the recombinant protein (Fig 2) similar to that in whole mitochondria (0.5% of total protein in both cases) This suggests that the Su9(1–112) fragment ensures effective insertion or anchoring of mCYP11A1 in the inner mito-chondrial membrane Recall that this amino acid sequence governs the import of fused proteins into the mitochondrial matrix and subsequent reinsertion into the inner membrane The treatment of yeast mitochondria imported

COX-IV-mCYP11A1 with 1% (v/v) Triton X-100 failed to wash out an appreciable amount of mCYP11A1 (Fig 3), which was indicative of its predominant aggregation Similarly, the pCYP11A1 and iCYP11A1 forms were insignificantly solubilized with 1% (v/v) Triton X-100 from yeast mito-chondria that had imported pCYP11A1 (Fig 3), which testifies to their prevalent aggregation Only the iCYP11A1 form was easily identified in the supernatant under these particular conditions; probably, pCYP11A1 and iCY-P11A1, which are the main forms of the imported foreign protein, differently associate with mitochondrial mem-branes Figure 3 shows that Su9(67–112)-mCYP11A1 is also poorly solubilized from mitochondria with 1% (v/v) Triton X-100 Upon similar treatment of adrenocortical mitochondria, cytochrome P450scc was essentially solubi-lized (Fig 3) However, even in this case solubilization was incomplete; therefore, we could not use the above data to quantitatively estimate protein aggregation

Figure 4 demonstrates that when the 12 000 g super-natant of mitochondria that had imported CoxIV(1–25)-mCYP11A1 was centrifuged at 106 000 g, CoxIV(1–25)-mCYP11A1 and

a minor amount of the precursor were found in the pellet mainly containing the inner mitochondrial membrane fragments (sonic SMP) In the case of mitochondria from yeast cells expressing pCYP11A1, the analogous SMP fraction contained pCYP11A1 and iCYP11A1 In both experiments, we could not see any CYP11A1 form in the high-speed supernatant Specific contents of mCYP11A1, pCYP11A1, and iCYP11A1 were much lower in the SMP fraction than in whole mitochondria, though the SMP fraction was enriched in phosphate carrier, the inner membrane marker (Fig 4) In contrast, fractionation of adrenocortical mitochondria yielded SMP with a cyto-chrome P450scccontent higher than that in whole organelles [6] One may conclude that in yeast mitochondria only minor amounts of the above CYP11A1 forms are associated

Fig 2 Comparative Western blots of mitochondria and their 12 000 g

supernatant fractions from recombinant yeast producing CoxIV(1–

–25)-mCYP11A1, pCYP11A1 and Su9(1– –112)-mCYP11A1 The samples of

mitochondria (M) from yeast producing recombinant proteins and

corresponding 12 000 g supernatant fraction (S) were probed with

anti-(cytochrome P450 scc ) IgG In all cases 20 lg of protein were

loaded per lane Markers: phosphate carrier (PC), an integral protein

of the inner membrane; Mge1p, a soluble matrix protein.

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with the mitochondrial membrane(s) while the bulk thereof

aggregates in the matrix

Clearly different results were obtained for the Su9(1–

112)-mCYP11A1 construct As follows from Fig 4, the

specific contents of its processed form are close in whole

yeast mitochondria and the SMP fraction, which is similar

to the data for phosphate carrier Thus, in contrast to

pCYP11A1, iCYP11A1, and mCYP11A1,

Su9(67–112)-mCYP11A1 is predominantly harboured by mitochondrial

membranes The impossibility of solubilizing this

recom-binant protein by the Triton X-100 treatment of

mitochon-dria (see above) suggests that it forms membrane-associated

aggregates However, these aggregates, unlike those of

other CYP11A1 forms, are not sedimented at 12 000 g

This indirectly indicates that aggregates of pCYP11A1,

iCYP11A1, and mCYP11A1 are not associated with

mitochondrial membranes and accumulate in the matrix

It is known that in some cases the aggregation of proteins

imported into mitochondria can be attenuated by lowering

their concentration [18] With this in mind, we have constructed a plasmid for expression of cDNA for CoxIV(1–25)-mCYP11A1 in an integrative vector under the control of the constitutive phosphoglycerate kinase promoter In this case the content of recombinant protein in mitochondria was reduced by an order of magnitude However, the bulk of CYP11A1 was again in the form of aggregates insoluble in 1% (v/v) Triton X-100 (data not shown)

Assays of cholesterol side-chain cleavage activity

of recombinant forms of CYP11A1 in yeast mitochondria

Knowing that recombinant forms of CYP11A1 imported into yeast mitochondria can exert catalytic activity only in a nonaggregated state, we used for the assays the 12 000 g mitochondrial supernatants As the latter obviously contain CYP11A1s as components of SMP, the results below should be taken as activities of the membrane-bound proteins It follows from the Table 1 that the rates of conversion of 22(R)-hydroxycholesterol into pregnenolone related to the total protein content of the 12 000 g supernatant are similar for the cells expressing pCYP11A1 and CoxIV(1–25)-mCYP11A1 The activity of the 12 000 g supernatant containing Su9(67–112)-mCYP11A1) proved

to be much lower This became even more evident with the specific activities of CYP11A1s Table 1 shows that for pCYP11A1 and CoxIV(1–25)-mCYP11A1 both the con-tents of the proteins and their specific activities are quite close Within the experimental error, they match the values obtained for a system reconstituted of purified bovine cytochrome P450scc, adrenodoxin, and adrenodoxin reduc-tase (Table 1) or for the solubilized membrane fraction of

E coli cells expressing mCYP11A1 supplemented with appropriate components [14] Thus, once cotranslocation-ally inserted into the inner membrane of yeast mitochon-dria, CYP11A1 is efficiently converted into holocytochrome P450scc Especially remarkable is that in the experiments with pCYP11A1 the 12 000 g supernatant contains mainly pCYP11A1 and iCYP11A1, not mCYP11A1 As in this case the specific activity related to the total content of these

Fig 3 Treatment of mitochondria from recombinant yeast producing CoxIV(1– –25)-mCYP11A1, pCYP11A1, or Su9-mCYP11A1, and adrenocortical mitochondria with Triton X-100 Mitochondria were treated with 1% (v/v) Triton X-100 and centrifuged at 25 000 g The resulting pellet (P) and supernatant (S) were probed with anti-(cytochrome P450 scc ) IgG or anti-(cytochrome P450 scc ) and anti-Hsp78 IgGs The presented P and S samples are from 50, 50, 60 and 20 lg of mitochondria including CoxIV-mCYP11A1, pCYP11A1, Su9-mCYP11A1, and cytochrome P450 scc , respectively Probing with anti-Hsp78 IgG was made to demonstrate complete separation of soluble and aggregated/complexed proteins.

Fig 4 Comparative Western blots of mitochondria and SMP fractions

from recombinant yeast strains producing CoxIV(1– –25)-mCYP11A1,

pCYP11A1, and Su9(1– –112)-mCYP11A1 The samples of

mitochon-dria (M) and corresponding SMP fractions (SMP) from yeast

produ-cing recombinant proteins were probed with anti-(cytochrome P450 scc )

IgG The M and SMP samples contained equal amounts of total

protein, which were 18, 25 and 20 lg per lane in the experiments with

mitochondria including CoxIV-mCYP11A1, pCYP11A1, and

Su9-mCYP11A1, respectively Markers: phosphate carrier (PC), an integral

protein of the inner membrane; Mge1p, a soluble matrix protein.

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forms was close to the activity of cytochrome P450sccin the

reconstituted system, we suggest that pCYP11A1 and/or

iCYP11A1 are catalytically active

The content of Su9(67–112)-mCYP11A1 was 10–20 times

higher while its specific activity was several hundred times

lower than the above values Hence, the Su9(1–112)

targeting signal governs effective binding of

Su9(67–112)-mCYP11A1 to the inner mitochondrial membrane

How-ever, such binding does not result in proper folding of the

polypeptide chain and formation of the active holoenzyme

Of course, cytochrome P450scc-calibrated immunoblotting

is only semiquantitative, but the observed effects exceed all

possible experimental errors

Discussion

In adrenocortical mitochondria, cytochrome P450scc is

known to be an integral protein of the inner membrane

[2,19] Two ways of achieving such localization have thus far

been considered [reviewed in 20]: the stop-transfer

mech-anism, i.e cotranslocational insertion of a polypeptide chain

into the inner membrane using stretches of hydrophobic and

nonpolar residues; and the re-export mechanism, i.e total

or partial translocation of a polypeptide chain into the

matrix followed by its processing and reinsertion into the

membrane by means of a specific N-terminal hydrophobic

stretch Analysis of the amino acid sequence of mCYP11A1

[21] shows that this protein has no regions capable of

realizing either of the above mechanisms Thus, the mode of

membrane insertion of CYP11A1 still remains puzzling It

has earlier been reported [10] that CYP11A1 perhaps

remains membrane-bound during the entire period of its

translocation into adrenocortical mitochondria and

matur-ation This might be accounted for by the presence of a

specific mitochondrial partner of pCYP11A1 that retards its

transmembrane translocation Besides, the addressing

pre-sequence of pCYP11A1 can be somehow involved in

confining this protein in the inner mitochondrial membrane

This specific presequence has an N-terminal region enriched

in hydrophobic amino acid residues, and negatively charged

residues in its C-terminal region [21], which is not typical of

the canonical presequences of cytoplasmically made

mito-chondrial precursor proteins

In contrast to adrenocortical mitochondria, the in vitro

synthesized pCYP11A1 is easily translocated into the matrix

of isolated yeast mitochondria, as follows from the results of

precise digitonin fractionation of mitochondria and the high

sensitivity of either form of imported pCYP11A1 to the

matrix protease Pim1p [9] In this work we show that upon expression of pCYP11A1 and CoxIV(1–25)-mCYP11A1 in yeast the precursor, semiprocessed, and mature proteins (CYP11A1s) accumulate in mitochondria mainly as aggre-gates Nevertheless, some amounts of CYP11A1s were found in the mitochondrial membranes We suggest that in yeast mitochondria the CYP11A1 precursors meet no serious hindrances to their translocation and thus largely

slip into the matrix, where the processed proteins cannot be properly folded and undergo proteolysis or aggregation To

a lesser degree this concerns pCYP11A1, which, owing to its noncanonical presequence, is better confined in the mem-brane of yeast mitochondria (Fig 5) This can explain why the SMP fraction contains mainly the pCYP11A1 and iCYP11A1 forms, and why their insertion into the mito-chondrial membranes is more effective than that of Cox (1–25)-mCYP11A1

Table 1 The content and specific cholesterol side-chain cleavage activity of various forms of recombinant CYP11A1 imported into yeast mitochondria The measurements were carried out with 12 000 g supernatants of mitochondria from yeast cells producing recombinant forms of CYP11A1 The activity is defined as the rate of conversion of 22(R)-hydroxycholesterol into pregnenolone at 37 °C The latter was quantitatively oxidized to progesterone with cholesterol oxidase.

Initial forms of

recombinant proteins

Content of CYP11A1 in assay sample, nmole (% total protein)

Activity (10 4 nmole progesteroneÆmin)1Æmg)1 total protein)

Activity (10 3 nmole progesterone min)1Ænmol)1 CYP11A1)

Fig 5 Putative pathways of import of various CYP11A1 precursor versions into yeast mitochondria and their intramitochondrial sorting (1) Su9(1–112)-mCYP11A1 is translocated into the matrix, processed, and reinserted into the membrane (yeast mitochondria); (2) CoxIV(1–25)-mCYP11A1 is rapidly processed and translocated into the matrix, with

a smaller portion being inserted into the membrane (yeast mitochondria); (3) pCYP11A1 is rather slowly processed and trans-located into the matrix, with the more essential portion being cotranslocationally inserted into the membrane (yeast mitochondria); (4) pCYP11A1 is cotranslocationally processed and inserted into the inner membrane (adrenocortical mitochondria).

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It has earlier been found [8] that, upon expression of

the pCoxVI-mCYP11A1 construct in S cerevisiae,

mCYP11A1 resides in the inner mitochondrial membrane

and the intermembrane contact sites However, these

gradient centrifugation experiments with mitochondrial

homogenates could not have quantitatively assessed the

balance of the import As follows from the present work, a

considerable amount of the imported CYP11A1 could have

been missed

The cytochrome P450scc-calibrated immunoblotting

allowed one to analyse the balance between the processes

of membrane insertion of CYP11A1s and their aggregation,

which takes place in the matrix, as follows from the in vitro

experiments [9] As the total protein contents in an aliquot

of yeast mitochondria and the corresponding 12 000 g

supernatant are close, one can compare the contents of

CYP11A1s Such a comparison clearly shows that for

CoxIV(1–25)-mCYP11A1 the content of the imported and

nonproteolysed protein is almost 10-fold higher in

mito-chondria than in the 12 000 g supernatant, which suggests

that only one-tenth of mCYP11A1 is inserted into the

membrane, thus escaping aggregation in the matrix It is

evident that for pCYP11A1 the combined contents of

precursor and intermediate forms of the recombinant

protein in mitochondria and 12 000 g supernatant differ

at least twofold

All the above membrane-bound CYP11A1s are

effi-ciently converted into the catalytically active form This

follows from their high specific side-chain cleavage activities,

which are of the same order of magnitude as that estimated

for mCYP11A1 expressed in E coli cells [14]

As to the Su9(67–112)-mCYP11A1 imported into

mito-chondria, it almost completely accumulates in the

mem-brane fraction: the mitochondria and the 12 000 g

supernatant contain equal relative amounts of the

recom-binant protein The Su9(1–112) segment is known to ensure

the initial stage of the import of Su9 into the matrix

followed by two-stage processing and reinsertion of its

N-terminus into the inner membrane [11] Most plausibly,

the Su9(67–112)-mCYP11A1 sequence imported into yeast

mitochondria misfolds in the matrix and does not refold

upon reinsertion into the inner membrane through the

Su9(67–112) fragment In fact, Su9(67–112)-mCYP11A1

detected mainly in the SMP fraction exhibits very low

side-chain cleavage activity and is poorly solubilized with

1% (v/v) Triton X-100

Thus, different topogenic signals in the CYP11A1

versions predetermine their sorting and conversions in

yeast mitochondria (Fig 5) Catalytically active

cyto-chrome P450scccan be accumulated in yeast mitochondria

in as much as the protein escapes aggregation in the

matrix However, some basic features of the import of its

precursor into mitochondria inevitably result in a low

yield of the active protein Analysis of the above data

definitely shows that the crucial moment in the

topo-genesis of cytochrome P450scc is not reception,

trans-membrane translocation, or proteolytic processing of its

precursor, but rather the confinement of the protein in the

inner membrane upon its import into adrenocortical

mitochondria This still unknown mechanism cannot be

adequately implemented in some foreign (e.g yeast)

mitochondria A search for such a mechanism may extend

the conventional notions on the topogenesis of mito-chondrial proteins

Acknowledgements

The authors are indebted to S A Saveliev for the help in preparing the pYeDP/pCYP11A1 and to A V Galkin for editing the text This work was supported by the Russian Foundation for Basic Research (grants 99-04-48003 and 00-15-97942 to VNL).

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