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R E V I E W A R T I C L Einto the mechanisms of protein maturation, folding, and stability Yoshihiro Sambongi1, Susumu Uchiyama2,*, Yuji Kobayashi2, Yasuo Igarashi3and Jun Hasegawa4 1 Gr

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R E V I E W A R T I C L E

into the mechanisms of protein maturation, folding, and stability

Yoshihiro Sambongi1, Susumu Uchiyama2,*, Yuji Kobayashi2, Yasuo Igarashi3and Jun Hasegawa4

1

Graduate School of Biosphere Sciences, Hiroshima University, Japan;2Faculty of Pharmaceutical Science, Osaka University, Japan;

3 Department of Biotechnology, University of Tokyo, Japan; 4 Daiichi Pharmaceutical Co., Ltd, Tokyo, Japan

Cytochrome c is widely distributed in bacterial species,from

mesophiles to thermophiles,and is one of the

best-charac-terized redox proteins in terms of

biogenesis,folding,struc-ture,function,and evolution Experimental molecular

biology techniques (gene cloning and expression) have

become applicable to cytochrome c,enabling its engineering

and manipulation Heterologous expression systems for

cytochromes c in bacteria,for use in mutagenesis studies,

have been established by extensive investigation of the

bio-logical process by which the functional structure is formed

Mutagenesis and structure analyses based on comparative studies using a thermophile Hydrogenobacter thermophilus cytochrome c-552 and its mesophilic counterpart have pro-vided substantial clues to the mechanism underlying protein stability at the amino-acid level The molecular mechanisms underlying protein maturation,folding,and stability in bacterial cytochromes c are beginning to be understood Keywords: bacteria; biogenesis; cytochrome c; Hydrogeno-bacter thermophilus; mutations; protein stability

In mitochondria,electrons shuttle between the

membrane-bound cytochrome bc1complex and cytochrome oxidase via

a water-soluble protein cytochrome c This process is

required for generation of an electrochemical proton

gradient across the membrane (proton-motive force),which

drives the synthesis of ATP,a biological energy currency

Bacteria also have soluble monoheme Class I cytochromes c

functioning as similar electron carriers on the peripheral

surface of the cytoplasmic membrane This type of

cytochrome c is widely distributed in bacteria,from

meso-philes to thermomeso-philes,and is one of the best-characterized

proteins in terms of biogenesis,folding,structure,function,

and evolution

Cytochrome c is unique among heme proteins in having a

heme covalently attached to the polypeptide chain via two

thioether bonds,formed from the vinyl groups of the heme

and the two cysteine residues in the consensus

Cys-X-X-Cys-His motif [1–4] Site-directed mutagenesis studies of

cytochrome c are at a relatively early stage,because of the

difficulty of expressing the holoprotein heterologously

However,during the last decade,it has been found that

bacteria have specific cellular apparatus for covalent attachment of a heme to the cytochrome c polypeptide [1–4] Knowledge obtained from studies on the cytochrome c biogenesis pathway in bacteria has been used to produce heterologous cytochromes c in large quantities,which has facilitated mutagenesis studies and structure analysis

In this review,we will summarize how bacterial cyto-chromes c have been used in recent mutagenesis and structure studies to elucidate protein stability Through such investigations,we have gained insight into the molecular mechanisms underlying cytochrome c maturation and folding as well as stability Bacterial cytochrome c has contributed greatly to our understanding in these areas,and

is one of the most successful model proteins The basic ideas obtained with cytochromes c should be applicable to other proteins of industrial and/or medical interest

C Y T O C H R O M E c A S A M O D E L F O R

P R O T E I N S T A B I L I T Y S T U D I E S Proteins isolated from thermophilic bacteria are usually stable to heat and chemical denaturants,indicating that they must have most of the determinants of protein stability Initial clues to the relationship between protein structure and stability can be obtained by pairwise sequence com-parison of homologous proteins from thermophiles and mesophiles The cytochromes c,which play a central role in electron-transport chains in both thermophilic and mesophilic bacteria as well as in eukaryotes,are useful in investigations of the structural basis of protein stability at the amino-acid level

Highly homologous cytochromesc from thermophiles and mesophiles

For pairwise comparison to elucidate protein structure– stability relationships using cytochromes c,we must find

Correspondence to Y Sambongi,Graduate School of Biosphere

Sciences,Hiroshima University,1-4-4 Kagamiyama,

Higashi-Hiroshima,Hiroshima 739-8528,Japan.

Fax/Tel.: +81 824 24 7924,

E-mail: sambongi@hiroshima-u.ac.jp

Abbreviations: AA c-555, Aquifex aeolicus cytochrome c-555 s ;

HT c-552, Hydrogenobacter thermophilus cytochrome c-552;

PA c-551, Pseudomonas aeruginosa cytochrome c-551; TT c-552,

Thermus thermophilus cytochrome c-552.

*Present address: Department of Biotechnology,Faculty of

Engine-ering,Osaka University,2-1 Yamadaoka,Suita,Osaka 565-0871,

Japan.

(Received 24 January 2002,revised 5 June 2002,

accepted 10 June 2002)

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homologues in thermophiles and mesophiles that exhibit

high sequence identity Cytochrome c-552 from a

thermo-philic bacterium, Hydrogenobacter thermophilus (HT c-552),

is an 80-amino-acid protein with a heme covalently attached

to the polypeptide chain [5] The amino-acid sequence

(Fig 1) and main chain folding (Fig 2) of HT c-552 from

this thermophile closely resemble those of the 82-amino-acid

cytochrome c-551 from a mesophile, Pseudomonas

aerugi-nosa(PA c-551) Comparisons of the two proteins indicated

that the amino-acid residues are 56% identical [5],and that

the root mean square deviation for their main chain folding is

within 1 A˚ [6] However,as expected from the difference in

their optimal growth temperatures (H thermophilus,70C;

P aeruginosa,37C),HT c-552 is more stable to heat and chemical denaturants than PA c-551 [7–9] For instance,the former has a significantly higher mid-point denaturation temperature than the latter,as judged both spectrophoto-metrically and calorispectrophoto-metrically As described below,inves-tigation of the relationship between the 3D structures and thermodynamic properties accompanying protein denatu-ration of HT c-552 and PA c-551 (wild-types and mutants) has revealed the molecular mechanism underlying protein stability The difference in stability between them can be attributed to differences in side-chain interactions in a few select regions [9] (see below)

Other homologous cytochromesc

To determine the amino-acid residues responsible for protein stability,it would be better if sequence information was available from a larger number of homologous proteins

in a variety of bacteria that differ in optimal growth temperature Many homologous cytochromes c that exhibit sequence identity with HT c-552 and PA c-551 of more than 50% are also known in other mesophiles,e.g Pseudomonas, Azotobacter,and Nitrosomonas species (Fig 1) On direct sequence comparison of these proteins,

we can find amino-acid residues that exist in HT c-552,but not in most of the others at the corresponding positions It is likely that some of them are the residues responsible for stability,and in fact Ala7,Met13,and Tyr43 in HT c-552 have been shown,by mutagenesis and 3D structure analyses,to be determinants of the higher stability of

HT c-552 (see below) For clarity,the residue numbers used for HT c-552 are those of PA c-551

A hyperthermophile, Aquifex aeolicus,has an 86-amino-acid cytochrome c-555s(AA c-555),in its mature form [10] The sequence identity between HT c-552 and AA c-555 (33%) is not as high as that between HT c-552 and

PA c-551,but these three proteins still exhibit high sequence similarity (more than 50%,Fig 1),therefore their main-chain folding may be similar Furthermore,the optimal growth temperature greatly differs between H thermophilus and A aeolicus (70C and 95 C,respectively) Therefore,

AA c-555 should be included in the group of cytochromes c

to be examined for protein stability Thermodynamic analysis of AA c-555 will be of great interest

The homologous cytochromes c listed in Fig 1 are excellent models for determining the structural origin of protein stability Experimental data on the stability and 3D structure of these proteins in addition to their amino-acid sequences will provide valuable information on the mech-anism underlying protein stability

B I O G E N E S I S O F B A C T E R I A L

C Y T O C H R O M E S c

To confirm experimentally the amino-acid residues respon-sible for the stability indicated by sequence and 3D structure comparisons,site-directed mutagenesis needs to be per-formed on a series of homologous cytochromes c For this, the cytochrome c gene needs to be heterologously expressed

as a holoprotein that has a covalently attached heme and is

in a correctly folded form,like the authentic proteins isolated from the original bacteria Heme attachment,which must occur regardless of whether the cytochrome c gene is

Fig 1 Amino-acid sequence comparison of cytochromes c homologous

to HT c-552 Amino-acid sequences are aligned using residue numbers

(every 10) of PA c-551 HT, Hydrogenobacter thermophilus; PA,

Pseudomonas aeruginosa; PS, Pseudomonas stutzeri; PZ, Pseudomonas

stutzeri Zobell; PM, Pseudomonas mendocina; PD, Pseudomonas

denitrificans; PF, Pseudomonas fluorescens; AV, Azotobacter vinelandii;

NE, Nitrosomonas europaea; AA, Aquifex aeolicus.

Fig 2 3Dstructural comparison of HT c-552 and PA c-551 The

main-chain folding of HT c-552 (red) is overlaid with that of PA c-551

(green).

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endogenous or exogenous,is a unique step during

cyto-chrome c biogenesis To be able to attempt heterologous

expression of the cytochrome c gene,it is important to

determine how the heme attaches to the polypeptide in the

cell and how its functional structure is formed The

path-way of cytochrome c biogenesis has been extensively studied

[1–4] In this section,we briefly summarize recent advances

in our understanding of bacterial cytochrome c biogenesis

Genes required for cytochromec biogenesis in bacteria

Cytochrome c heme lyase has been identified as the enzyme

responsible for heme attachment to the mitochondrial

cytochrome c polypeptide [11] The apparatus for

cyto-chrome c biogenesis in bacteria is not analogous to that in

mitochondria,because no orthologue of the cytochrome c

heme lyase has been found in the genomes of bacteria that

can synthesize cytochrome c Instead,genetic evidence has

suggested that at least 12 genes (ccm,cytochrome c

maturation; dsb,disulphide bond formation) are required

for bacterial cytochrome c biogenesis [1–4,12] It would be

interesting to compare the biogenesis apparatus for

cytochromes c and nonheme iron-sulfur proteins (Nif

proteins involved in Fe/S cluster formation),as the latter

appears to be common in prokaryotes and eukaryotes to

some extent [13] Among the ccm gene products, Escherichia

coliCcmE was first biochemically characterized as a factor

transferring a heme to the cytochrome c apopolypeptide

[14] A subsequent biochemical study indicated that E coli

CcmC can interact with CcmE during heme transfer [15] It

was predicted that heme transfer occurs in the periplasm

in vivo A site-directed mutagenesis study on bacterial

cytochrome c polypeptides also supported the idea that

heme attachment takes place after the apoprotein has left

the cytoplasm [16]

Effect of thiol–disulfide redox conditions

A defect in an integral membrane protein,DsbD (also

known as DipZ),was first characterized as an E coli

mutation that prevented the synthesis of mature

cyto-chrome c in the periplasm [17] DsbD contains a domain

with potential disulfide isomerase activity facing the

periplasm [18–20] Other Dsb proteins,DsbA and DsbB,

which have been determined to oxidize cysteine thiols to

form the internal disulfide bonds of many proteins in the

E coli periplasm,are also required for cytochrome c

biogenesis [21,22]

Cytochrome c biogenesis in dsbD mutant cells can be

restored by adding low-molecular-mass thiol compounds to

the growth medium [17],and that in dsbA and dsbB mutants

by adding disulfide compounds [22] These

complementa-tion results are consistent with the general role of Dsb

proteins in the regulation of the thiol–disulfide redox

conditions during periplasmic protein folding Although

no biochemical evidence for the requirement of the Dsb

system during cytochrome c biogenesis has been obtained

yet,the genetic evidence suggests that thiol–disulfide redox

control is also essential for cytochrome c biogenesis in the

periplasm Importantly,these results indicated that the level

of cytochrome c production could easily be controlled by

the thiol–disulfide redox potential,and this is the case,as

described below

E X P R E S S I O N O F E X O G E N O U S

C Y T O C H R O M E c G E N E S I N B A C T E R I A For mutagenesis studies and structure analysis,it is necessary to obtain heterologously expressed,mature holo-cytochromes c in large quantities Knowledge obtained from studies on bacterial cytochrome c biogenesis needs to

be extended for the efficient production of mature holocyto-chromes c

Targeting to the periplasm The functional regions of gene products (Ccm and Dsb proteins) required for cytochrome c biogenesis are located

in the periplasm of bacteria Thus,cytochrome c apo-polypeptide targeting to the periplasm is a physiologically essential feature for its maturation This is also indicated

by the presence of a typical signal peptide in the precursor of bacterial soluble cytochrome c Requirement

of the signal peptide was experimentally verified by heterologous gene expression of Paracoccus denitrificans cytochrome c-550 in E coli; the holoprotein is produced

in the periplasm if the gene retains the coding region for

a native signal peptide,but the cytoplasmic apoprotein is produced if this signal is removed [23] Even a mitoch-ondrial soluble cytochrome c can be expressed as a holoprotein in the E coli periplasm when the eukaryotic gene product is targeted to the periplasm by fusing the signal peptide of bacterial cytochrome c at the N-terminus [16]

Periplasmically expressed exogenous cytochromes c in host cells so far have spectrophotometric characteristics identical with those of the authentic proteins produced in the original organisms [8,23–26] These findings indicate that the heme attachment mode and protein folding are correct during heterologous gene expression and protein maturation Thus,heterologously expressed cytochromes c, which are quality-controlled in the bacterial periplasm,can

be used for further biochemical analysis as if we are dealing with the native proteins

Control of production level The yields of heterologously expressed cytochromes c may depend on the copy numbers of the plasmids used In addition,coexpression with ccm genes is effective for higher levels of plasmid-borne cytochrome c gene expression [27] Not only the protein factors functioning in the periplasm, but also low-molecular-mass thiol/disulfide compounds, which can maintain the periplasmic redox balance [28], successfully control the cytochrome c production level For instance,the yields of exogenous and endogenous cytochromes c reach about 10% of the total periplasmic protein fraction in E coli with the addition of disulfide compounds to the medium [22] Furthermore,a certain

E coli strain,JCB7120,can produce exogenous holo-(PA c-551) up to 30% of the total periplasmic protein level [8],although the mechanism underlying this high expression

in this strain is not yet known Now,using bacterial expression systems,we can obtain large amounts of holocytochromes c,which in terms of visible absorption spectra and other properties are indistinguishable from the native proteins This progress has facilitated mutagenesis

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and structure analyses of bacterial cytochromes c,including

HT c-552 and PA c-551

M U T A G E N E S I S S T U D I E S F O R

S T A B I L I T Y

In general,site-directed mutagenesis is a powerful tool for

investigating the relationship between protein structure and

function Experimental techniques for mutagenesis are

applicable to bacterial cytochrome c,as discussed above

We are able to dissect the molecular mechanisms of

cytochrome c,in terms of biogenesis,protein folding,redox

properties,electron-transfer kinetics,and stability,at the

amino-acid residue level through mutagenesis studies In

this section,we describe successful mutagenesis using

PA c-551 variants modeled by the homologous and more

stable HT c-552

Amino-acid residues responsible for stability

As HT c-552 and PA c-551 have almost identical main-chain folding [6],subtle differences in the side-main-chain interactions must explain the remarkable difference in their stabilities By careful comparison of their 3D structures [6],

we found that aromatic amino-acid interactions uniquely occur between Arg37 and Tyr34 and/or Tyr43,the latter also having hydrophobic contacts with the side chains of Tyr34,Ala40,and Leu44 in HT c-552 These interactions are not found in PA c-551 Small hydrophobic cores formed

by the side chains of Ala7,Met13,and Ile78 in HT c-552 are more tightly packed than the corresponding regions formed

by Phe7,Val13,and Val78 in PA c-551 All these residues are distributed in three separate regions (Fig 3) We expected that these multiple residues spread over the separate regions of HT c-552 would cause overall protein

Fig 3 Side-chain packing in the regions responsible for the stability of HT c-552, and the corresponding regions in the wild-type PA c-551 and its quintuple mutant The mutated residues in the quintuple mutant,and the corresponding ones of the wild-type PA c-551 and HT c-552 are colored purple,green,and red,respectively (A) The region around A7/M13 in HT c-552 and the corresponding regions (B) The region around Y34/Y43 in

HT c-552 and the corresponding regions (C) The region around I78 and the heme (denoted as HEM) in HT c-552 and the corresponding regions.

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stability in an additive manner,and hoped that we would be

able to clearly determine the stabilizing factors by

muta-genesis studies

Engineering stable proteins

To characterize the factors that affect protein stability,we

attempted to achieve maximal enhancement of the stability

of PA c-551 by introducing minimal mutations into

spa-tially separate regions Five amino-acid residues in

PA c-551,which were selected on structure comparison,

were substituted with those found at the corresponding

positions in HT c-552,and the stabilities of the resulting

PA c-551 mutants were measured A single mutation [Val78

to Ile (V78I)] and double mutations [Phe7 to Ala/Val13 to

Met (F7A/V13M) and Phe34 to Tyr/Glu43 to Tyr (F34Y/

E43Y)] in the three regions of PA c-551 each individually

enhanced the overall protein stability [8] These studies,

together with structure analysis,provided substantial clues

to the mechanism of protein stability in HT c-552 Ala7/

Met13 and Ile78 fill small spaces present in the

correspond-ing regions of PA c-551,and Tyr34/Tyr43 cause a favorable

electrostatic interaction These side-chain interactions may

contribute to the enhanced stability of HT c-552 It would

be worth trying to mutate HT c-552 so that it has the

amino-acid residues found in PA c-551,and to examine whether

the resulting HT c-552 mutants have decreased stability

Surprisingly,a PA c-551 variant simultaneously carrying

the five mutations in the three separate regions (F7A/

V13M/F34Y/E43Y/V78I,quintuple mutant,Fig 3)

exhib-its almost the same stability as that of natural HT c-552

(Fig 4) [9] This demonstrates that it is possible to convert a

mesophilic protein into an artificial one with stability similar

to that of the natural thermophile by replacing a few

amino-acid residues Therefore,the thermophilic character of

HT c-552 may depend on a few strong noncovalent

interactions We further found that the increase in the

stability of the quintuple mutant is almost the same as the sum of the three individual stabilities [9,29] Thus, the mutation(s) in each of the three regions contribute to the overall stability in an additive manner The multiple mutations in the separate regions of PA c-551 provide experimental evidence on the mechanism underlying the enhanced stability of HT c-552,the relationship between local side-chain interactions,and overall protein stability The rational design resulting from careful structural comparison of HT c-552 and PA c-551 makes it possible to select a set of amino-acid residues that are completely responsible for the stability of a thermophilic protein Only

a small number of mutant proteins is required to experi-mentally confirm which amino acids are responsible for overall protein stability This is the advantage of structural comparison of highly homologous proteins If

we randomly selected five of the 35 amino-acid residues that differ between the two cytochromes c,we would have tested (35· 34 · 33 · 32 · 31)/(5 · 4 · 3 · 2 · 1) ¼

324 632 variants

What we can learn from thermophilic proteins The strategies used by thermophilic proteins to enhance stability are,for example,relatively increased polarity of the solvent-exposed surface area,increased packing density and core hydrophobicity,and generation of ion pairs or hydrogen bonds between polar residues [30–32] However, these interactions are often related to each other in a protein molecule,and subtle changes in them can affect the overall stability Thus,it is usually difficult to identify the exact factors that contribute to the enhanced stability of the proteins from thermophilic bacteria This is reasonable because proteins in the native state are stabilized by 10–

20 kJÆmol)1compared with those in the denatured state; the energy is equivalent to the formation of only a few hydrogen bonds Therefore,to understand protein stability,it is necessary to carry out precise comparative studies using homologues exhibiting high sequence identity with similar 3D structures,but differing in stability From such comparisons,we must carefully detect subtle differences in side-chain interactions,and examine their contributions to the overall protein stability by mutagenesis studies If the interactions are spatially separated,their contributions may

be additive,in which case we can clearly identify protein-stabilizing factors The combination of precise comparison

of the structures of thermophilic and mesophilic homolog-ous proteins and selection for multiple mutations in separate regions is a valuable approach to elucidating the relation-ship between structure and stability This approach has been successfully applied to cytochrome c (as discussed above), triose phosphate isomerase [33],ribonuclease HI [34],and cold shock protein [35]

Recent advances in genome projects have revealed the gene resources of thermophilic bacteria,providing further opportunity for systematic comparisons of homologous proteins A similar approach to that used for cytochromes c will be applicable to other proteins Not only sequence comparison to identify the determinants of protein stability, but also the thermophilic proteins themselves can be used to elucidate the basic mechanisms underlying protein functions because they are usually purified and handled more easily than their mesophilic counterparts Although thermophilic

Fig 4 Thermal stability of wild-type PA c-551, the quintuple mutant,

and HT c-552 The heat capacity curves obtained by differential

scanning calorimetry of wild-type PA c-551,the quintuple mutant,and

HT c-552 at pH 5.0 with 1.5 M guanidine hydrochloride are shown.

The peak temperatures represent respective denaturation

tempera-tures.

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proteins are isolated from diverse extreme environments,

they should reveal general features of protein structure,

function,and stability

M U T A G E N E S I S F O R M A T U R A T I O N

S T U D I E S

In addition to protein stability,mutagenesis studies with

cytochrome c have contributed to the understanding of

protein maturation As discussed above,physiological

attachment of heme to apo-(cytochrome c) takes place in

the periplasm An exception to periplasmic covalent heme

attachment was first found for HT c-552 a decade ago

through a mutagenesis study [36] This unique case has

unexpectedly shed light on the chemical aspects of heme

attachment and apoprotein folding through the follow-up

experiments

Heterologous holo-(HT c-552),which has the heme

attached covalently,is found in the cytoplasm when the

truncated gene coding for the mature protein without the

original signal sequence is transformed into E coli [23,36]

An apo-(HT c-552) variant carrying mutations at the heme

covalent binding site (C12A/C15A) has also been expressed

in the E coli cytoplasm This gene product was found to

have a compactly folded structure,which apparently differs

from that of the natural holoprotein with the heme attached

covalently [37] The folded apoprotein aggregates into

amyloid fibrillar structures over a long time period [38],but,

in the presence of excess heme,it retains the prosthetic

group noncovalently like a b-type cytochrome [39] In

contrast,mesophilic apocytochromes c seem to form a

random coil structure,and holoprotein formation does not

occur in the bacterial cytoplasm [23] These observations

suggest that apo-(HT c-552) has a sufficiently folded

structure to incorporate a heme at moderate temperature,

possibly because of its thermostable properties After

insertion of the heme,cytochrome c folding occurs

There-fore,heme is not only required for the redox properties of

cytochrome c,but is also essential for correct protein

folding during cytochrome c biogenesis

The unique case of cytoplasmic heme attachment also

leads to the hypothesis that covalent thioether bond

formation itself can proceed spontaneously without

enzy-matic assistance once the heme is inserted into the

apopro-tein [23,40] Recently, a thermophile, Thermus thermophilus,

cytochrome c-552 (TT c-552) was produced as a

holopro-tein in the E coli cytoplasm similar to the case of HT c-552

[26] Although the cytoplasmic holo-(TT c-552) has the

same function and spectra as the authentic one,the

cytoplasmic soluble protein fraction also contains a minor

product,which has a heme attached covalently but differs in

heme-binding mode [41] This heterogeneity found in the

cytoplasmic products also appears to indicate that the heme

attachment itself is not catalyzed enzymatically

C O N C L U S I O N S A N D P E R S P E C T I V E

Highly homologous monoheme Class I cytochromes c are

available from a wide range of bacteria,from mesophiles to

thermophiles Their small size and high sequence identity are

advantageous for determining the amino-acid residues

responsible for protein stability The heterologous expression

systems established for cytochrome c, together with the

results from rapidly increasing X-ray crystal and NMR analyses,have stimulated mutagenesis studies,which con-tribute to the understanding of the mechanisms underlying protein maturation,folding,and stability

A mutagenesis study has also been carried out on cytochrome c to investigate its redox properties [42] The redox function of stable HT c-552 will be promising for electrochemical applications,such as the creation of a useful molecular device A cytochrome c folding study will also reveal the basic features of protein conformational diseases,

as first demonstrated with a HT c-552 mutant [38] Various technologies,such as NMR relaxation analysis,temperature jump methods,high pressure NMR,and stopped flow and single–molecule analyses,have been established,and are applicable to the elucidation of the dynamic features of cytochromes c It is of interest to characterize cytochrome c with respect to protein maturation,folding,stability,and function using a variety of combined experimental tech-niques Cytochrome c molecules will become very well understood through such interdisciplinary methods

A C K N O W L E D G E M E N T S

We thank Ikuo Ueda for his support and encouragement,and Kazuaki Nishio and Yuko Iko for critical reading of the manuscript.

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