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Matura-tion of c-type cytochromes requires covalent attachment of the heme cofac-tor to the protein, and there are at least five distinct biogenesis systems that catalyze this post-transl

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Order within a mosaic distribution of mitochondrial c-type cytochrome biogenesis systems?

James W A Allen1, Andrew P Jackson2, Daniel J Rigden3, Antony C Willis4, Stuart J Ferguson1 and Michael L Ginger5,6

1 Department of Biochemistry, University of Oxford, UK

2 Wellcome Trust Sanger Institute, Hinxton, Cambridgeshire, UK

3 School of Biological Sciences, University of Liverpool, UK

4 MRC Immunochemistry Unit, Department of Biochemistry, University of Oxford, UK

5 Sir William Dunn School of Pathology, University of Oxford, UK

6 Department of Biological Sciences, Lancaster University, UK

Keywords

bioinformatics; Ccm system; cytochrome c;

Diplonema papillatum; evolution; heme

lyase; lateral gene transfer; mitochondria;

post-translational modification; Trypanosoma

Correspondence

M Ginger, Department of Biological

Sciences, Lancaster University, Lancaster

LA1 4YQ, UK

Fax: +44 1524 593192

Tel: +44 1524 593922

E-mail: m.ginger@lancaster.ac.uk

(Received 7 February 2008, revised 3 March

2008, accepted 5 March 2008)

doi:10.1111/j.1742-4658.2008.06380.x

Mitochondrial cytochromes c and c1 are present in all eukaryotes that use oxygen as the terminal electron acceptor in the respiratory chain Matura-tion of c-type cytochromes requires covalent attachment of the heme cofac-tor to the protein, and there are at least five distinct biogenesis systems that catalyze this post-translational modification in different organisms and organelles In this study, we use biochemical data, comparative genomic and structural bioinformatics investigations to provide a holistic view of mitochondrial c-type cytochrome biogenesis and its evolution There are three pathways for mitochondrial c-type cytochrome maturation, only one

of which is present in prokaryotes We analyze the evolutionary distribu-tion of these biogenesis systems, which include the Ccm system (System I) and the enzyme heme lyase (System III) We conclude that heme lyase evolved once and, in many lineages, replaced the multicomponent Ccm sys-tem (present in the proto-mitochondrial endosymbiont), probably as a con-sequence of lateral gene transfer We find no evidence of a System III precursor in prokaryotes, and argue that System III is incompatible with multi-heme cytochromes common to bacteria, but absent from eukaryotes The evolution of the eukaryotic-specific protein heme lyase is strikingly unusual, given that this protein provides a function (thioether bond forma-tion) that is also ubiquitous in prokaryotes The absence of any known c-type cytochrome biogenesis system from the sequenced genomes of various trypanosome species indicates the presence of a third distinct mito-chondrial pathway Interestingly, this system attaches heme to mitochon-drial cytochromes c that contain only one cysteine residue, rather than the usual two, within the heme-binding motif The isolation of single-cysteine-containing mitochondrial cytochromes c from free-living kinetoplastids, Euglena and the marine flagellate Diplonema papillatum suggests that this unique form of heme attachment is restricted to, but conserved throughout, the protist phylum Euglenozoa

Abbreviations

ccm, cytochrome c maturation; EF-1a, elongation factor-1a; EST, expressed sequence tag; IMS, intermembrane space; KH test, Kishino– Hasegawa test; LGT, lateral gene transfer; ML, maximum likelihood; SOD, superoxide dismutase.

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Multiple pathways for heme cysteine

attachment

The c-type cytochromes are characterized by the

cova-lent attachment of heme to the apocytochrome through

thioether (carbon–sulfur) bonds (Fig 1) Numerous

examples of distinct c-type cytochromes have been

described in Bacteria and, more recently, in some

Ar-chaea, where they typically function in electron transfer

or at the catalytic sites of certain enzymes [2–9] {In

agreement with the nomenclature proposed in [1], we

refer to the three domains of life as Bacteria (formerly

Eubacteria), Archaea (formerly Archaebacteria) and

Eucarya (the eukaryotes) When using the expression

‘Bacteria’, we therefore refer to the domain; when

using the term ‘bacteria’, we refer generically to

non-archaean prokaryotes} However, the best known

examples from the c-type cytochrome family are

mito-chondrial cytochromes c and c1, which function as

essential electron transfer components of the

respira-tory chain [7,8,10]

The covalent attachment of two vinyl groups from

the heme cofactor to the thiols in the CXXCH

heme-binding motif of apocytochromes c is chemically far

from facile (X is any amino acid, except cysteine), and

there are multiple systems which catalyze this

post-translational modification in biology [2,4,11–15]

Sys-tems I and II are modular and widely distributed

amongst bacteria [2,4,6,12,14]; they have been studied

using a combination of genetic and biochemical

approaches [2,4,14,16,17] System I is understood best

in Escherichia coli, where it consists of eight dedicated essential proteins, named CcmA–H (Fig 2A), and a number of accessory proteins CcmA–H are all mem-brane anchored or integral memmem-brane proteins, and collectively function in the periplasm The biogenesis

of c-type cytochromes is a spatial and temporal prob-lem; in bacteria, both heme and apoprotein are synthe-sized in the cytoplasm and must be transported to the periplasm, where heme attachment occurs The apocy-tochrome polypeptide is translocated by the general type II secretion (Sec) proteins [18] How heme is transported remains an intriguing mystery

CcmA and CcmB are reminiscent of an ATP-dependent (ABC-type) transporter, and CcmA has been shown to hydrolyze ATP [19] However, no transport substrate has yet been identified; heme has been proposed, but much evidence weighs against this possibility [19–22] A more recent hypothesis is that CcmA and CcmB are required to release heme from the heme chaperone CcmE by coupling the free energy gained from ATP hydrolysis [21] CcmE is a key player in the Ccm system; it binds heme cova-lently as an intermediate in the cytochrome c biogen-esis pathway [23] This remarkable heme attachment occurs between a histidine residue and a heme vinyl group Heme attachment to CcmE is dependent on CcmC [24], an integral membrane protein with a number of interesting phenotypes arising from muta-tion in ccmC, some of which may be unrelated to c-type cytochrome biogenesis [25] CcmD is a very small ( 60 amino acids) integral membrane protein

Fig 1 Structures of (A) heme (Fe-protoporphyrin IX) and (B) heme bound to a polypeptide chain as in a typical c-type cytochrome, in which the vinyl groups of the heme are saturated by the addition of cysteine thiols that occur in a Cys-Xxx-Xxx-Cys-His motif (only the sulfur atoms

of the cysteines are shown), forming covalent bonds between heme and protein (C) Cartoon representation of heme attachment to protein

in mitochondrial cytochrome c The porphyrin ring is shown in blue and the heme iron atom in brown The cysteines of the CXXCH motif form covalent bonds to the heme, and the histidine acts as a ligand to the heme iron atom via a nitrogen atom The sixth ligand to the iron atom is the sulfur of a methionine residue located distantly from the CXXCH motif in the primary structure of the protein In bacterial c-type cytochromes, histidine (rather than methionine) is often the sixth iron ligand, and there are examples with cysteine, an N-terminal amino group, asparagine, lysine or a vacant coordination site There are few restrictions on the nature of the Xxx-Xxx residues.

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that mediates complex formation between CcmC and

CcmE [26] CcmF and CcmH are implicated in the

transfer of heme from holo-CcmE to

apocyto-chrome c, including the covalent heme attachment

step to produce the product holocytochrome E coli

CcmH is a fusion protein which includes the proteins

known as CcmH and CcmI in many bacteria CcmG

is a thioredoxin-like protein [27] that forms part of

an electron transfer chain Electrons are transferred

from the cytoplasmic protein thioredoxin, via the

multidomain membrane protein DsbD, to CcmG,

and then to the apocytochrome to reduce a disulfide bond that forms between the cysteines of the apocyto-chrome CXXCH heme-binding motif; these thiols must

be reduced for heme attachment to occur (reviewed in [2]) Such a reductive pathway is thought to be neces-sary in E coli, partly because the periplasm contains the strong, indiscriminate, disulfide-oxidizing protein DsbA

System II (Fig 2B) is less well understood than System I at the molecular level, but it seems very likely

to consist of four proteins [28] {Note: The

nomencla-Fig 2 Cytochrome c biogenesis systems found in bacteria Each of these systems can mature a wide variety of c-type cytochromes, including those with multiple hemes (A) System I (the Ccm system) in Escherichia coli Some uncertainties are designated with ‘?’; for example, what, if anything, is transported by the ABC-type transporter CcmAB, and how is heme transported from its site of synthesis in the cytoplasm to the periplasm? DsbD has two thiols amongst its eight transmembrane helices which are believed to accept reducing equiv-alents from thioredoxin (TrxA) These thiols, in turn, pass on the reducing power to periplasmic C- and N-terminal domains From there, reductant passes to the c-type cytochrome biogenesis apparatus, tentatively by the route shown; CcmG has been shown in some schemes

to be the electron acceptor from CcmH but, although there is experimental evidence for this order, more evidence indicates the arrange-ment shown in the figure DsbA is a strong, non-specific disulfide bond-oxidizing protein found in the periplasm of E coli Ultimately, the cysteine thiols of the apocytochrome CXXCH heme-binding motif become reduced to allow heme attachment Heme becomes covalently attached to the chaperone CcmE as an intermediate in the pathway The specific covalent attachment of heme to apocytochrome c is believed to involve CcmF and H (B) Cytochrome c biogenesis System II in a Gram-negative bacterium In some species, CcdA is replaced

by the protein DsbD shown in Fig 2A CcdA and ResA provide a pathway by which reductant is transferred to the apocytochrome to reduce

a disulfide bond in the CXXCH heme-binding motif ResB and ResC provide the covalent heme attachment function to produce the product holocytochrome c Heme delivery to the periplasm from the cytoplasm may also occur through the ResBC complex, but this is presently not certain Other names are in common use for ResA ⁄ B ⁄ C (ResA = CcsX = HCF164; ResB = CcsB = Ccs1; ResC = CcsA; and CcdA = CcsC).

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ture for System II c-type cytochrome biogenesis

pro-teins is somewhat inconsistent in the literature Here,

we adopt the names used for the various biogenesis

proteins found in Bacillus subtilis, i.e ResA (also

called CcsX or HCF164), ResB (also called CcsB or

Ccs1), ResC (also called CcsA) and CcdA (also called

CcsC)} These include a thioredoxin-like protein called

ResA (similar in structure to CcmG) [29] and CcdA, a

functional analogue of DsbD, or DsbD itself

(depend-ing on the organism); together, these apparently form

a pathway analogous to that observed in System I for

reducing a disulfide bond in the apocytochrome

CXXCH motif The heme attachment (and possibly

heme delivery) function of System II is catalyzed by

ResB and ResC Indeed, a fusion protein cloned from

Helicobacter pylori containing elements of ResB and

ResC was sufficient to mature c-type cytochromes

when expressed in the periplasm of a ccm deletion

strain of E coli [30]

Several recent studies have provided insight into the

flexible organization of prokaryotic c-type cytochrome

biogenesis pathways For example, in the Archaea and

some bacteria, a divergent System I has recently been

described [3], and some bacteria contain components

of both System I and System II [3,4,6] Although the

presence of multiple cytochrome c biogenesis systems

in a single bacterium might hint at possible

redun-dancy, additional c-type cytochrome maturation

com-ponents are sometimes required for heme attachment

to specific substrates For example, in the

e-proteobac-terium Wolinella succinogenes, the ccsA1-encoded heme

lyase is required for thioether bond formation to

the remarkable CX15CH heme-binding motif of the

multi-heme c-type cytochrome MccA [31]

System III for cytochrome c maturation consists of

a single primary component, the enzyme heme lyase,

which is found only in the mitochondrial

intermem-brane space (IMS) of animals, fungi and some protists

[11,32] {The kingdom Protista refers to those

eukary-otes that cannot be classified as animals, plants or

fungi: it includes protozoa and algae The protozoa [or

‘first (proto-) animals (zoa)’] are unicellular eukaryotes,

which lack the chitinous cell wall found in fungi} At

least in fungi, heme lyase is supplemented by the

flavo-protein Cyc2, which is thought to provide reducing

equivalents for the heme attachment process [33] The

biochemical study of heme lyase has proved

challeng-ing, and the molecular details of its enzymology are

still largely unclear

Finally, a distinctive example of a biogenesis system

that is required for the dedicated maturation of a

partic-ular substrate is provided by the recent description of

System IV for cytochrome c maturation Heme is

attached through a single thioether linkage to cyto-chromes b6and b from the b6fand bc complexes of oxy-genic phototrophs (cyanobacteria, plants, algae) and certain Bacillus species, respectively [34,35] The mecha-nism by which covalent heme attachment to Bacillus cytochrome b occurs is not yet known, but the identifi-cation of gene products from the green alga Chlamydo-monas reinhardtii that restore cytochrome b6formation

in four ccb mutants constitutes the initial step in the characterization of System IV, which appears to be conserved in all oxygenic phototrophs [36]

In species from the phylum Euglenozoa, which includes Euglena gracilis and the medically relevant trypanosomatids (Trypanosoma brucei, T cruzi and pathogenic Leishmania species), heme is uniquely attached to the mitochondrial c-type cytochromes by a single thioether bond within a F⁄ AXXCH heme-bind-ing motif [37–41] In an earlier study, we determined that, in the trypanosomatids, the occurrence of single-cysteine-containing mitochondrial cytochromes c and

c1 correlates with the absence from both nuclear and mitochondrial genomes of genes encoding any compo-nent of the known c-type cytochrome maturation systems; we also provided experimental evidence that, for the single-cysteine-containing T brucei cyto-chrome c, spontaneous (i.e uncatalyzed) maturation is unlikely [41] These results indicate that at least one further pathway for cytochrome c maturation awaits discovery in the trypanosomatids

In this article, we draw on the resources that are provided through the availability of numerous com-plete genome sequences and several ab initio modeling programs We consider in detail the evolutionary dis-tribution of the machinery for mitochondrial cyto-chrome c assembly throughout the Eucarya, and the possible origins of heme lyase Although the origin of the exclusively eukaryotic heme lyase remains mysteri-ous, replacement of a proto-mitochondrial System I pathway for c-type cytochrome maturation occurred multiple times during protist evolution With rare exceptions, these replacements probably occurred as a result of eukaryote-to-eukaryote lateral gene transfer (LGT) or endosymbiotic gene transfer of heme lyase

We also approach defining the limits of the distribu-tion of the single-cysteine heme-binding motif found in some mitochondrial cytochromes c

Mapping character traits onto a consensus view of eukaryotic phylogeny

The origin of the first eukaryotic cell has been debated for many years; during the 1980s and early 1990s, the

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available experimental evidence was generally

consis-tent with an evolutionary model (called the Archezoa

theory), which posited two early phases to eukaryotic

evolution: an ancestral phase, in which the hallmark

features of the eukaryotic cytoskeleton, endomembrane

system and nucleus were evolved, followed by the

sec-ond critical phase, which saw the acquisition of the

a-proteobacterial endosymbiont and the evolution of

the proto-mitochondrion Although the results from

some phylogenetic analyses conflicted with the model

formulated by Cavalier-Smith (discussed in [42]), the

Archezoa theory generally received robust support in

phylogenetic trees derived from the analysis of small

subunit rRNA or translation elongation factor

pro-teins Grouped at the base of many of these trees were

several eukaryotic lineages, including diplomonads

(represented by Giardia), the parabasalids (represented

by Trichomonas) and the Microsporidia [43,44] (and

reviewed recently in [45,46]) The distinctive

ultrastruc-ture of these organisms suggested that they apparently

possessed neither mitochondria nor other hallmark

eukaryotic organelles, such as peroxisomes and golgi,

and their status as Archezoa denoted that they were

believed to be ancestrally without these organelles We

now know that this is not the case; more recent

phylo-genetic treatments have resulted in the repositioning of

at least some formerly basal or ‘primitive’ eukaryotes

elsewhere within the eukaryotic tree [46–48]

Further-more, although the secondary loss of peroxisomes has

occurred numerous times in evolution, the

aforemen-tioned organisms crucially retain mitochondria, golgi

and other classically eukaryotic subcellular

compart-ments that have merely been remodeled beyond

obvi-ous or easy recognition [49–53] Thus, there are no

known examples of contemporary eukaryotes that lack

double-membrane-bound organelles of mitochondrial

descent; indeed, although difficult to prove, a popular

current viewpoint is that the acquisition of the

proto-mitochondrial endosymbiont could have been

coinci-dent with eukaryotic origins (see, for example, [47,54]

for a further discussion)

Although the position of the root for eukaryotic

evolution remains a contentious issue – Cavalier-Smith

has argued that the last common ancestor of all extant

eukaryotes diverged with the unikont–bikont split

(Fig 3) [55–57]; other results have suggested that it is

still not possible to discount a previously long-standing

view that the diplomonads and parabasalids belong to

the earliest diverging eukaryotic lineage [46,47,58] –

comparative interrogations of various morphological

and molecular character traits, as well as phylogenies

based on the analysis of multiple gene sets, have

resulted in a seemingly robust resolution of eukaryotic

diversity into six major groupings ([59] and reviewed in [46,47,60,61]) The framework provided by this resolu-tion is increasingly being used to inform on the evolu-tion of various fundamental aspects of eukaryotic biology, both within and between these major group-ings [55–57,62–66] It is this consensus view of eukary-otic evolution on which the comparative analysis described below is based

A phylogeny for mitochondrial c-type cytochrome maturation

Using the complete or draft nuclear and mitochondrial genome sequences indicated in supplementary Doc S1,

we mapped the distribution of mitochondrial cyto-chrome c maturation pathways onto a consensus view

of eukaryotic phylogeny (Fig 3) Our aim was to assess whether there was any obvious order to the otherwise mosaic distribution of mitochondrial cyto-chrome c biogenesis machineries that has previously been hinted at [67,68]

The presence of the Ccm system in higher plants and some unicellular eukaryotes [e.g the deeply diver-gent jakobid Reclinomonas americana, ciliates and the rhodophyte (red alga) Cyanidioschyzon merolae] has been described previously [69–74], whereas other eukaryotes, such as the animals, the chlorophyte green alga C reinhardtii and the malarial parasite Plasmo-dium falciparum (an apicomplexan) have heme lyase for maturation of mitochondrial cytochromes c [2,15,32,75–77] The mitochondrial genome sequences

of various excavate, algal, plant and ciliate taxa very clearly point to the presence of System I within the a-proteobacterial endosymbiont from which mitochon-dria evolved [69,70,72,78,79] However, taking into account the generally robust support for relationships within and between the taxonomic groups shown in Fig 3, our comparative genomic analysis can be used

to provide new insight into the evolution of mitochon-drial cytochrome c maturation Observations that are key to the discussion that follows in subsequent sec-tions are: (a) there is no evidence for the occurrence of heme lyase within the bikont supergroup Excavata; (b)

in the unikonts, heme lyase is the only c-type cyto-chrome maturation system present; (c) there is a mosaic distribution of the Ccm system and heme lyase within the Chromoalveolata and Plantae; (d) wherever the multicomponent Ccm system is used for mitochon-drial cytochrome c maturation, it is always partially encoded on the mitochondrial genome; this is perhaps unsurprising given that CcmC and CcmF are mito-chondrial integral membrane proteins containing mul-tiple predicted transmembrane helices Where a

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mitochondrial genome sequence is complemented by

the availability of a complete or draft nuclear genome

sequence for the same organism, the following always

holds true: (i) if components of the Ccm system are

encoded in the mitochondrial genome, further

dedi-cated Ccm components are also encoded in the nuclear

genome; (ii) there are no examples of eukaryotes

possessing multiple systems for mitochondrial

cyto-chrome c maturation Thus, even without the availabil-ity of a sequenced nuclear genome, the absence from a protozoan or algal mitochondrial genome of genes encoding Ccm components almost certainly provides a reliable indication that System I will not be used for the maturation of mitochondrial cytochromes c and c1 There are several green, red (rhodophyte) and chromist algae (belonging to the Chromalveolata), plus other

Fig 3 The phylogenetic distribution of the different pathways used for mitochondrial c-type cytochrome maturation in eukaryotes (A) Rela-tionships within and between five of the six eukaryotic supergroups – no relevant data for c-type cytochrome maturation in the sixth super-group, Rhizaria, are currently available The unikonts comprise the Amoebozoa (to which Dictyostelium discoideum and the human pathogen Entamoeba histolytica belong) and the Opisthokonts (the animals, fungi and various protozoa) The unikonts differ from the bikonts (which include the algae, land plants and many different protozoa) in that they possess (probably ancestrally [55]) only a single centriole (the barrel-shaped structure from which flagellar basal bodies are derived and which, in many eukaryotes, is also involved in the organization of the mito-tic spindle) The phylogeny reveals that, within some groups (e.g Viridiplantae), some species contain System I, whereas others contain System III; there were no examples of eukaryotes that contained multiple systems for the maturation of mitochondrial c-type cytochromes A more detailed overview of the distribution of mitochondrial c-type cytochrome maturation pathways in the Plantae is provided in (B) Lineages belonging to the Streptophyta are highlighted by the grey background The evolutionary relationships shown represent a consensus view of published data A complete list of species used to produce the phylogeny, including the databases searched, is provided in supplementary Doc S1 Species for which the identification of the mitochondrial c-type cytochrome biogenesis apparatus is based on the interrogation of a complete genome sequence are as follows: the choanoflagellate Monsiga brevicolis (System III); the amoebozoan Dictyostelium discoideum (System III); the chlorophyte green algae Chlamydomonas reinhardtii, Volvox carteri, Ostreococcus lucimarinus and Ostreococcus tauri (all System III); the red alga Cyanidioschyzon merolae; the ciliates Tetrahymena thermophila and Paramecium tetraurelia (System I); the Apicom-plexans Plasmodium falciparum, Toxoplasma gondii and Theileria parva (System III); the dinoflagellate Perkinsus marinus (System III); the oomycetes Phytophthora ramorum and Phytophthora sojae and the diatoms Thalassiosira pseudonana and Phaeodactylum tricomutum (collectively belonging to a group known as the stramenopiles or chromists) (all System III); the Heterolobosean Naegleria gruberi (System I) Entamoeba histolytica (Amoebozoa), Encephalitozoon cuniculi (Microsporidia), Cryptosporidium parvum (Apicomplexa) and the diplomonad Giardia intestinalis all contain degenerate mitochondria known as mitosomes, and the parabasalid Trichomonas vaginalis possesses hydro-genosomes; such degenerate forms of mitochondria lack a respiratory chain and therefore do not contain c-type cytochromes.

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protozoan species (the amoebozoan Acanthamoeba

cas-tellanii), for which no nuclear genome sequence is

available, but there is an accessible or annotated [79]

mitochondrial genome sequence on which no

compo-nent of the Ccm system is encoded Similarly, there is

extensive sequence coverage for the uniquely organized

(many small linear chromosomes of less than 8.3 kb in

length) mitochondrial genome of the ichthyosporean

Amoebidium parasiticum (belonging to the

Opi-sthokonta); from the sequence released thus far, this

genome also lacks genes encoding Ccm components

[80] Assuming that none of these species contain

mitochondrial c-type cytochromes with atypical

heme-binding motifs, we suggest that it is likely that

nuclear-encoded heme lyase is used for the maturation of their

mitochondrial cytochromes c and c1

We found a eukaryotic cytochrome c biogenesis

Sys-tem II only in those eukaryotes that contain

chlorop-lasts (data not shown), and we assume that, in these

cases, System II is used for the maturation of the

chloroplast c-type cytochromes, given the ancestral

relationship between chloroplasts and System

II-con-taining cyanobacteria [81,82] Where System II was

observed, a second c-type cytochrome biogenesis

appa-ratus was always present and is presumed to be

responsible for maturing the mitochondrial

cyto-chromes c and c1(e.g System I in Arabidopsis thaliana,

System III in C reinhardtii and chromist algae)

Simi-larly, genes encoding the four chloroplast proteins

recently shown to be required for single-cysteine

attachment to cytochrome b6in Chlamydomonas –

Sys-tem IV for c-type cytochrome biogenesis – were also

only present in phototrophic eukaryotes [36] The

absence of heme lyase from the excavates, the possible

origins of heme lyase and the molecular basis for the

mosaic distribution of Systems I and III in

chromalve-olates and the Plantae are the critical issues upon

which we focus in the remainder of this article

Was heme lyase ever present in

the Excavata?

A number of important human pathogens, such as

try-panosomes, Giardia and Trichomonas, as well as a

diverse assortment of free-living protozoa, are included

in the supergroup Excavata The validity of this

classi-fication was initially based on a number of shared

morphological features, but has more recently received

modest support from a variety of molecular

phyloge-nies [59,83–85] Support for the monophyly of the

Excavata is, however, equivocal [86]; indeed, the

possi-bility that the earliest diverging eukaryote was an

ancestor of diplomonads (Giardia) and parabasalids

(Trichomonas) has not yet been entirely dismissed [46,58] Interestingly, if we accept the emerging evi-dence that groups the Excavata together, a deep-branching status for the supergroup can be inferred from a variety of character traits A prime example is the distinctive mitochondrial genome of the jakobid

R americanawhich, in terms of both gene content and genome organization, more closely resembles an a-pro-teobacterial genome than any other mitochondrial gen-ome that has presently been sequenced [70,87] Like Reclinomonas, some of the other excavates currently sampled (Naegleria and Malwimomonas) contain the Ccm system for cytochrome c maturation (Fig 3) Others (Trichomonas vaginalis and Giardia intestinalis) lack a capacity for respiration, and c-type cytochromes are accordingly absent from their degenerate mito-chondria, making it impossible to assess which system for cytochrome c maturation would have been present

in their last aerobic ancestors In trypanosomatids, cytochromes c and c1 are present, but there is no rec-ognizable c-type cytochrome maturation system Thus, there is no evidence that heme lyase was ever present within the excavate supergroup

The recently described absence [41] of any known cytochrome c biogenesis system from the various try-panosomatids represents a particularly intriguing sce-nario, as it correlates with the attachment of heme to single-cysteine XXXCH mitochondrial cytochromes in these organisms Such single cysteine cytochromes are also present in other kinetoplastids (the trypanosoma-tid family evolved from a kinetoplastrypanosoma-tid ancestor) and the euglenids Euglena gracilis and E viridis [37–41] All of these protists belong to the phylum Euglenozoa (Fig 4A), but, in addition to the euglenids and kine-toplastids, the Euglenozoa includes a third major taxo-nomic group, a family of mostly free-living marine flagellates known as the diplonemids Recent phyloge-nies suggest that the diplonemids are likely to be a sis-ter group to the Kinetoplastida [88] Although there is

no genome project for a diplonemid, we have used the relatively simple experiment of determining the type of mitochondrial cytochromes present (either CXXCH

or XXXCH heme attachment) to look further at the evolution of cytochrome c biogenesis in the Excavata From a combination of spectroscopic methods and N-terminal sequencing (Fig 4), Diplonema papillatum unambiguously contains a single-cysteine c-type cyto-chrome (AGQCH heme-binding motif) Thus, all three major taxonomic groups of the Euglenozoa (diplone-mids, kinetoplastids and euglenids) contain single-cysteine mitochondrial cytochromes c, and hence it is likely that they all contain the same, as yet unidenti-fied, apparatus for maturation of cytochromes c, which

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is distinct from that found in any other organisms.

Analysis of all the available genome sequences and all

publicly accessible expressed sequence tag (EST)

collec-tions (including ESTs for the excavates

Malawimon-as californiana, M jakobiformis and R americana)

using blast reveals that, strikingly,

single-cyste-ine attachment of heme to mitochondrial

cyto-chrome c remains a characteristic that is unique to species from the phylum Euglenozoa Crucially, these analyses included the use of the draft nuclear genome sequence for Naegleria gruberi, an amoeboflagellate with an aerobic metabolism from the phylum Hetero-lobosea, the eukaryotes with the closest evolution-ary relationship to the Euglenozoa [47,59,85] The

Fig 4 Diplonema cytochrome c has only a single cysteine in its heme-binding motif (A) Probable evolutionary relationships within the phy-lum Euglenozoa, as suggested by taxon-rich small subunit rRNA phylogeny (B) Absorption spectrum of semi-purified D papillatum cyto-chrome c, recorded at 25 C with the protein in 50 m M Tris ⁄ HCl (pH 8.0) containing a few grains of disodium dithionite to reduce the heme iron The protein was purified from a culture of D papillatum strain ATCC50162 by SP-Sepharose chromatography Absorption maxima were

at 419.5, 523.5 and 554.0 nm Inset: reduced pyridine hemochrome spectrum of the same protein Pyridine hemochrome analysis was con-ducted according to Bartsch [133]: final concentrations of hydroxide and pyridine were 0.2 M and 30% (v ⁄ v), respectively, and a few grains

of dithionite were added The a-band peak maximum at 553.0 nm (indicated by the vertical broken line) diagnostically indicates heme attach-ment to the polypeptide via one cysteine residue [37,39,41,133–135] Diplonema was cultured in artificial seawater as described previously [136], and subjected to detergent extraction [41] prior to isolation of cytochrome c (C) Sequence alignment of the N-terminal 40 amino acids

of Diplonema cytochrome c, as determined by Edman degradation, and the N-terminal regions of cytochromes c from other organisms: Cf, Crithidia fasciculata; Dp, Diplonema papillatum; Eg, Euglena gracilis; iso, isoform; Sc, Saccharomyces cerevisiae; Tb, Trypanosoma brucei The c-type cytochrome heme-binding motif is highlighted in bold for each cytochrome Underlined residues denote differences between the major and minor isoforms of mitochondrial cytochrome c in D papillatum: Dpiso1 is the major form (75% of the total protein) and Dpiso2 is the minor form (25%) Cytochrome c as analyzed in (B) was further purified using a CM-Sepharose column before N-terminal sequencing Cysteine gives a blank (X) in the sequencing reaction unless appropriately alkylated [137]; thus X is what is expected and observed for cyste-ine covalently bound to a heme in a c-type cytochrome It is, however, clear that the first residue of the heme-binding motif of D papillatum cytochrome c is alanine not cysteine, and thus the cytochrome has a single cysteine heme-binding motif of the type found in other Eugleno-zoaons, rather than CXXCH as observed in typical mitochondrial cytochromes c.

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N gruberimitochondrial cytochromes c and c1contain

CAQCH and CSACH motifs, respectively; these

cytochromes are matured by cytochrome c biogenesis

System I

We postulated previously [41] that the acquisition of a

novel mitochondrial cytochrome c biogenesis system in

the Euglenozoa provided not only a driving force for the

loss of a pre-existing maturation system, but also the

evolutionary pressure to move from CXXCH to

XXXCH cytochromes c If increased taxon sampling

fails to detect the existence of an excavate heme lyase,

this is likely to influence which of the models discussed

below most parsimoniously explains the distribution of

cytochrome c maturation systems shown in Fig 3

Probing the origins of heme lyase

Heme lyase has, since its discovery [15], remained a

rather enigmatic enzyme: the origin of this

eukaryotic-specific protein is obscure and little is known about

the biochemistry of System III-dependent

cyto-chrome c maturation [13] From the analysis shown in

Fig 3, it is clear that, although animals and

Dictyoste-liumeach encode a single form of heme lyase, two

iso-forms of heme lyase are found in other eukaryotes At

least in Saccharomyces cerevisiae, the presence of two

lyases reflects the distinct substrate preferences of each

enzyme: either cytochrome c or c1, respectively

[15,32,75] In order to obtain an insight into the origin

of heme lyase and to explore a molecular explanation

for its evolutionary distribution, we performed a

phy-logenetic analysis, and also applied a number of

bioin-formatics tools that can be used to detect remote

structural similarities between different proteins that

are undetectable even by sensitive iterative database

searches

Assuming that the presence of multiple heme lyases

always reflects, as it does in yeast, the deployment of

one enzyme to catalyze the maturation of each

mito-chondrial c-type cytochrome, one aim with the

phylog-eny was to determine whether the transition from a

single heme lyase with broad substrate specificity to

dual enzymes, each with their own specificity for either

cytochrome c or cytochrome c1 [15,32], was likely to

have occurred just once or on a number of occasions

With the exception of their N-termini, which were

lar-gely unique to each taxonomic group, heme lyase

pro-tein sequences were reliably aligned Following the

omission of sequences corresponding to putative heme

lyases from the choanoflagellate Monsiga brevicolis and

the dinoflagellate Perkinsus marinus, a bootstrapped

maximum likelihood (ML) phylogeny robustly resolved

distinct heme lyase clades for the metazoan, fungal,

algal and apicomplexan sequences These clades were supported by bootstrap values greater than 75 (Fig 5) However, the relationships between these clades were not robust, and therefore could not be resolved satis-factorily Clearly, the arrangement of the basal nodes towards the root of the phylogeny is crucial to an understanding of the evolution of the c–c1 heme lyase distinction, and the number of origins in particular However, all heme lyases from Apicomplexa clustered together with reasonable robustness (bootstrap value, 87), largely due to the distinct N-termini shared by these proteins

The monophyly of all apicomplexan heme lyases points towards at least two origins of the c–c1distinction amongst eukaryotes: one prior to the divergence of the fungi and one affecting the alveolates [the group that includes the ciliates, apicomplexans and dinoflagellates (Fig 3)] Further origins of the c–c1distinction affecting diatoms (Thalassiosira pseudonana and Phaeodacty-lum tricomutum) and chlorophyte algae are possible, but increased taxon sampling is necessary to allow the reso-lution of these possibilities In the example of Dictyoste-lium, we cannot know whether the presence of a single heme lyase represents an ancestral state or the reverse transition of going from two distinct lyases to a single lyase of broader substrate specificity However, with regard to the opisthokonts, the presence of a single heme lyase in animals, but multiple lyases in the choanoflagel-late Monsiga brevicolis (Fig 3) and the fungi, points either to multiple origins for the c–c1 dichotomy or a loss of a heme lyase isoform from animals with, presumably, relaxation of the substrate specificity

To determine whether the monophyly of the apicom-plexan sequences was an artifact introduced by the biased base composition common to apicomplexan genomes, a neighbor-joining phylogeny was estimated with logdet genetic distances [89], which correct for base composition imbalance Monophyly of apicom-plexan sequences was still recovered after correction for base composition The result of the Kishino–Ha-segawa (KH) test also corroborated the view that there have been multiple origins for the c–c1 distinction Here, to test whether the optimal topology obtained from the ML and Bayesian inference (BI) trees was significantly more likely than a ‘single-origin’ scenario, the likelihood score of an alternative tree, in which all c- and c1-type sequences were reciprocally monophy-letic (i.e one simulating a single origin for the c–c1 dis-tinction), was compared with the optimal ML estimate using a KH test [90] and phylip v3.65 [91] A signifi-cant reduction in likelihood score when this constraint was enforced demonstrated that a single origin of the c–c1 distinction could be rejected – the alternative ML

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tree topology had a likelihood score of )24248.9,

which was significantly worse than the unconstrained,

optimal tree topology (Dln L = )54.2, P < 0.001)

To seek insight into the possible origin of System III

for cytochrome c maturation, we used a variety of

bio-informatics tools (as described in supplementary

Doc S1) to search for protein families distantly related

to heme lyase The application of these approaches

served only to highlight further the enigmas that

sur-round this fundamentally important enzyme; however,

as cytochromes c and c1 are matured within the

mito-chondrial intermembrane space (IMS), two possible

candidate proteins identified are nonetheless worthy of

mention Thus, after the obvious match to the heme

lyase domain itself, the first HHPRED result initially

appeared interesting A small portion, 34 residues, of the heme lyase was matched to a region of a Pfam entry for the Erv1⁄ Alr family of IMS proteins involved

in protein import into the IMS and export of mito-chondrial Fe⁄ S clusters into the cytoplasm [92–95] However, the heme lyase secondary structure predic-tion was not in good agreement with the four helical bundle architecture of the Erv1⁄ Alr sulfhydryl oxidase, and no other fold recognition method (below) flagged

up this putative relationship

The best 3D-Jury consensus fold recognition scores were obtained for the conserved domain of the human heme lyase but, at up to 45, did not reach the bench-mark significance cut-off of 50 [96] Once again the matched protein, superoxide dismutase (SOD), was

Fig 5 An unrooted, maximum likelihood (ML) phylogeny of heme lyase protein sequences A WAG substitution matrix was applied with among-site rate heterogeneity described by a gamma distribution estimated from the data Branch lengths are measured in substitutions per site Non-parametric bootstrap values from the ML analysis over 50, and their corresponding posterior probabilities from the Bayesian analy-sis, are shown adjacent to the nodes An asterisk denotes bootstrap values > 95 and posterior probabilities of 1.00 Full details of the meth-ods used for phylogeny construction and in the predictive modeling of heme lyase are provided in supplementary Doc S1 Clades are color coded by taxon: Fungi (red; c-type heme lyases are shaded lighter); Metazoa (yellow); Apicomplexa (blue; lighter and darker shading highlight distinct subclades); algal ⁄ stramenophile (green; lighter and darker shading highlight distinct subclades).

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