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The cytoplasmic⁄ inner membrane complex is composed of three NTPases VirB4, VirB11 and VirD4, VirB6 and VirB8; the trans-membranes pore complex VirB7, 9, 10; also termed ‘the core comple

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Architecture of the Helicobacter pylori Cag-type IV

secretion system

Laurent Terradot1and Gabriel Waksman2

1 Institut de Biologie et Chimie des Prote´ines, Biologie Structurale des Complexes Macromole´culaires Bacte´riens, UMR 5086

CNRS Universite´ de Lyon, France

2 Institute of Structural and Molecular Biology, UCL and Birkbeck, London, UK

Introduction

Secretion systems are widespread in bacteria for which

they provide means of exchange between the

extra-and intracellular milieus Seven different systems have

been described in Gram-negative bacteria [1] Amongst

them, the type IV secretion systems (T4SS) have raised considerable attention during the past 10 years because

of their role in bacterial pathogenesis T4SS are macro-molecular devices that bacteria use to transport various

Keywords

bacterial pathogenesis; CagA; secretion

system; stomach cancer; translocation

Correspondence

L Terradot, Institut de Biologie et Chimie

des Prote´ines, Biologie Structurale des

Complexes Macromole´culaires Bacte´riens,

UMR 5086 CNRS Universite´ de Lyon,

IFR128, 7 Passage du Vercors, F-69367

Lyon Cedex 07, France

Fax: +33 472 722604

Tel: +33 472 722652

E-mail: laurent.terradot@ibcp.fr and

G Waksman, Institute of Structural and

Molecular Biology, UCL and Birkbeck, Malet

Street, London, WC1E 7HX, UK

Fax: +44 (0)207 631 6803

Tel: +44 (0)207 631 6833

E-mail: g.waksman@bbk.ac.uk or

g.waksman@ucl.ac.uk

(Received 15 November 2010, revised 18

January 2011, accepted 27 January 2011)

doi:10.1111/j.1742-4658.2011.08037.x

Type IV secretion systems (T4SS) are macromolecular assemblies used by bacteria to transport material across their membranes T4SS are generally composed of a set of twelve proteins (VirB1–11 and VirD4) This repre-sents a dynamic machine powered by three ATPases T4SS are widespread

in pathogenic bacteria where they are often used to deliver effectors into host cells For example, the human pathogen Helicobacter pylori encodes a T4SS, the Cag-T4SS, which mediates the injection of the toxin CagA We review the progress made in the past decade in our understanding of T4SS architecture We translate this new knowledge to derive an understanding

of the structure of the H pylori Cag system, and use recent protein–protein interaction data to refine this model

Abbreviations

cagPAI, cytotoxin-associated gene-pathogenicity island; CTD, C-terminal domain; EM, electron microscopy; NTD, N-terminal domain; T4SS, type IV secretion system.

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macromolecules, including protein, DNA or nucleic

acid⁄ protein complexes, across the cell envelope [2,3]

These systems are remarkably versatile and have been

classified into three different groups according to their

function [3] T4SS in the first group are used to

trans-fer DNA from one cell to another in a process retrans-ferred

to as conjugation [4] Incorporating new DNA

sequences is a major selective advantage for these

organisms, which can rapidly acquire new genetic

fea-tures and adapt to changes in the environment Such

mechanisms are involved in the spread of antibiotic

resistance genes among pathogenic bacteria [5] One of

the most studied T4SS of this group is encoded by the

Agrobacterium tumefaciens VirB⁄ D system, which is

able to deliver nucleoprotein complexes into plant cells

leading to crown gall disease [6] The artificial

utiliza-tion of a modified VirB⁄ D system has proved

instru-mental in the production of genetically modified plants

[7] The second group of T4SS, exemplified by the

ComB system of H pylori and the GGI system of

Nei-sseria gonorrhoeae, is used for DNA uptake and release

from and to the extracellular milieu [8] The third

group consists of T4SS that transfer protein effectors

and are used by numerous pathogenic bacteria,

includ-ing Bordetella pertusis, Legionella pneumophila,

Bru-cella spp., H pylori and Bartonella spp In these

species, T4SS can be described as molecular pumping

devices that facilitate host–pathogen interaction and⁄ or

inject toxins into the host cell [5]

In the human pathogen H pylori, T4SS of the three

different groups have been identified The most

con-served one is the so-called ComB system (group 2),

which is considered to mediate the import and

integra-tion of environmental DNA fragments into its genome

[9] Another one (named Tfs3, group 1) is less

con-served and appears to play important roles in

deter-mining the remarkable plasticity of the H pylori

genome [10–12] The last T4SS (group 3) is present

only in the most virulent H pylori strains that produce

a major toxin, the CagA protein [13] This toxin and

the T4SS apparatus responsible for its transfer to the

eukaryotic cell are encoded by the so-called

cytotoxin-associated gene-pathogenicity island (cagPAI), a

40 kbp DNA fragment that is transmitted horizontally;

see the accompanying review by Tegtmeyer et al [14 ]

CagA is considered as a paradigm for bacterial

carci-nogenesis [15] Briefly, once injected into the host cell,

CagA is phosphorylated and interacts with more than

20 different human proteins involved in signal

trans-duction [16] As a consequence of CagA action,

epithe-lial cells will have some of their major functions

disturbed, such as cell–cell adhesion, signalling,

adher-ence and proliferation [17] CagA translocation and

phosphorylation are required to trigger the so-called

‘hummingbird phenotype’, a form of cell scattering [18] The cagPAI system also delivers bacterial pepti-doglycan that triggers the Nod1-response and induc-tion of the nuclear factor-jB pathway [19]

How these effectors are injected into the host cell is still poorly understood However, the last decade has seen a number of spectacular advances in the struc-tural definition of individual components of the T4SS and, more recently, on how they associate into large macromolecular complexes We review these advances,

as well as how, when integrated within the larger con-text of the numerous functional studies on the Cag proteins, a clearer picture of the architecture and func-tion of the Cag-encoded T4SS can be derived

Architecture of T4SS The T4SS apparatus generally consists of twelve pro-teins named VirB1–11 and VirD4 based on the nomen-clature used for A tumefaciens T4SS (Fig 1) They assemble to form three interlinked subparts: a cyto-plasmic⁄ inner membrane complex, a double mem-brane-spanning channel and an external pilus (Fig 1) Variations exist among the different types of T4SS but the composition of the compartments is generally con-served The cytoplasmic⁄ inner membrane complex is composed of three NTPases (VirB4, VirB11 and VirD4), VirB6 and VirB8; the trans-membranes pore complex (VirB7, 9, 10; also termed ‘the core complex’) forms a channel from the inner to the outer mem-brane; and the external pilus generally consists of the VirB2 and VirB5 proteins Other components are essential for the formation of the T4SS complex: VirB1 allows for the insertion of the system in the periplasm and VirB3, the function of which is unknown, is often associated with VirB4 In certain bacteria, some of these components are absent This is the case for the

H pyloriComB system where the apparatus is used to import DNA at the outer membrane and relies on the other competence system ComEC to transport DNA into the cytoplasm [20]

The conserved components of the cagPAI encoded T4SS (Cag-T4SS) have been identified first by sequence comparison with those of the VirB⁄ D system; see the accompanying review by Fischer [21] However, although the archetypal VirB⁄ D system has 12 compo-nents, the cagPAI encodes for 27 proteins Note that several nomenclatures exist for cagPAI proteins, which are listed in the review by Fischer [21] Except for the VirB⁄ D system homologues, these proteins are unique

to H pylori; see the accompanying reviews by Fischer [21] and Cendron and Zanotti [22] The role of most

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Cag proteins in the T4SS apparatus is not clear,

although several of them are essential for the secretion

of CagA or the induction of interleukin-8 [23]

The NTPases battery of the Cag-T4SS

The Cag-T4SS powering machinery is composed of

three cytoplasmic NTPases, HP0525 (VirB11), HP0524

(VirD4) and HP0544 (VirB4 putative homologue),

which supply the energy necessary to assemble the

apparatus and secrete CagA Structural information is

available for the VirD4 homologue TrwB from

Escher-ichia coli conjugative plasmid R388 and for HP0525

but not for VirB4 These NTPases have the canonical

walker A and B motifs, and couple NTP hydrolysis to

conformational changes These might in turn be

cou-pled to unfolding or transfer of the substrate [24]

Coupling protein VirD4 (HP0524)

VirD4 proteins are also named ‘coupling’ proteins

because they can recruit substrates to the T4SS

appa-ratus, although this function is absent in some systems

The most studied VirD4 protein is probably TrwB,

which is required for the translocation of DNA by the

E coli R388 conjugation system The crystal structure

of TrwB showed that the protein is a globular hexamer with an orange-like shape, with each subunit forming

an orange segment (Fig 2A) [25] The protein is com-posed of a 70 residues N-terminal trans-membrane seg-ment (absent in the crystal structure) and two domains, a nucleotide binding domain and an all-a-domain TrwB binds to ATP at the interface between subunits and hydrolysis is stimulated by DNA In the Cag-T4SS, VirD4 is encoded by the hp0524 and the resulting protein is much larger (748 residues) HP0524

is essential for CagA translocation but not for interleu-kin-8 induction [23] Evidence has recently been pro-vided that HP0524 interact with CagA, suggesting that HP0524 might also act as a coupling protein in the Cag-T4SS, as in other systems [26,27]

VirB11 (HP0525) and its regulation HP0525 is essential for CagA secretion [23] The struc-ture of HP0525 showed that the protein also forms hexamers, with each subunit consisting of two domains, an N-terminal domain (NTD) and a RecA-like C-terminal domain (CTD) containing the motifs found in all members of the traffic ATPases family [2]

Fig 1 (A) Schematic view of the cagPAI encoded by the H pylori strain 26695 Numbers correspond to the HP0XXX number of the ORF [57] represented by arrows; see also the accompanying review by Fischer [21] (B) Schematic representation of the prototypal T4SS VirB ⁄ D from A tumefaciens (left) and comparison with components of the Cag-T4SS (right) Cytoplasmic NTPases are coloured in blue, proteins forming the core trans-membrane complex are indicated in various shades of green, and pilus components in yellow ⁄ orange Integral trans-membrane segments or proteins are depicted as squares Note the presence of additional components (coloured in pink) that have been shown to participate in the Cag-T4SS complex In addition, the effector CagA (coloured in black) has been located at the tip of the pilus.

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Each domain of HP0525 forms an hexameric ring that

surrounds a central chamber The CTD ring has a

grapple-like shape, with two helices from each of the

monomers pointing into the centre of the ring to form

the claws of the grapple [28] VirB11 proteins are

dynamic assemblies, the conformations of which

depend on their nucleotide binding state [29] Indeed,

in HP0525 crystal structures, the nucleotides bind at

the NTD–CTD interface and stabilize their interaction

By contrast, in the absence of nucleotide, the NTDs

become disordered and point outwards from the centre

of the hexamer, leaving an open NTD ring When

nu-cleotides are bound, the NTDs are ordered and point

inwards in a closed ring conformation Such important

structural changes generated by nucleotide binding,

together with functional analysis of other T4SS

com-ponents, suggest that VirB11 proteins play a role in

substrate translocation by participating in the local

unfolding of effector proteins during translocation [30]

Nucleotides are not solely responsible for structural

changes in HP0525 A protein, HP1451, was originally

identified to interact with HP0525 in a high

through-put yeast two-hybrid experiment and the interaction

was confirmed biochemically [31,32] The structure of the complex between HP0525 and a large portion of HP1451 revealed that two molecules of the latter inter-acted with the hexamer of HP0525 [33] (Fig 2C) The HP1451 monomer structure consists of two consecutive

KH domains that are used by the protein to interact with several parts of the HP0525 NTDs The two HP1451 molecules lock the HP0525 NTDs in the closed state and obstruct the chamber From this structure, HP1451 was suggested to play an inhibitor role for HP0525 ATPase activity and CagA transfer, a hypothesis supported by ATPase assays and in vivo observation of complex formation It is therefore likely that HP1451 acts as a negative regulator for toxin secretion [33], thereby controlling part of the secretion process

HP0544 (VirB3⁄ B4) Little is known about the HP0544 (also named CagE)

On the basis of sequence signature, HP0544 contains motif conserved in both VirB3 and VirB4 proteins [34] This is reminiscent of the Campylobacter jejuni

Fig 2 Structures of cytoplasmic NTPases Side and top views of the crystal structures of T4SS NTPases shown as ribbon (A) Structure of TrwB, the VirD4 homologue from E coli conjugative plasmid R388 [25] (B) Structure of HP0525 [28], the VirB11 homologue from H pylori Cag-T4SS with the NTDs and the CTDs coloured in light and dark blue, respectively (C) Structure of the HP0525 ⁄ HP1451 [33] HP0525 pro-tomers are coloured in light blue and are in the same orientation as in (B) The two molecules of HP1451 are coloured in pink and magenta.

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T4SS, where VirB3 and VirB4 appear to be fused into

a single protein Thus, it is possible that CagE may

also combine VirB3 and VirB4 functions In other

T4SS, VirB4 is known to make numerous interactions

with other T4SS components, including VirB3, VirB8,

VirB10, VirB11 and VirD4 [2] Yet, in A tumefaciens,

it does not interact with the substrate DNA.VirB4

activity is required for T4SS function and might also

have a structural role at the inner membrane

The translocation pore of the Cag-T4SS

Until recently, the periplasmic core of the T4SS was

considered to be composed of VirB8, VirB7, VirB9

and VirB10 The structures of these proteins or parts

of these proteins have been solved individually (VirB8,

VirB9 [35]) or in a complex (VirB9:VirB7 [36];

VirB7-VirB9-VirB10 [37,38]) and have provided important

information on the architecture of the periplasmic core

(Fig 3A) Compared to these structures, the

homo-logues from the cagPAI are significantly different

Indeed, similarities between VirB10 and HP0527 and VirB9 and HP0528 reside only in the C-terminal por-tion (Fig 3C) This discrepancy is particularly appar-ent for HP0527 that consists of almost 2000 residues,

of which only 400 at the C-terminus correspond to VirB10 (approximately 400 residues in all T4SS) The remaining 1600 residues have a unique composition with a number of tandem repeats regions; see the accompanying review by Fischer [21] HP0532 and HP0530 are considered as putative homologues of VirB7 and VirB8, respectively, although the similarities are very poor and it can be anticipated that their prop-erties might also be different (Fig 3B)

Structure of the translocation pore Recently, two major advances have provided the molecular details of the translocation pore of a T4SS First, the cryo-electron microscopy (EM) structure of a VirB7-VirB9-VirB10 complex, from the plasmid pKM101 T4SS, was determined at 15 A˚ resolution

Fig 3 Periplasmic core complex (A) Ribbon representation of the crystal structures of VirB8 (Brucella suis) and ComB10 (VirB10 homo-logue from H pylori) and NMR structure of the TraO ⁄ TraN complex (VirB7 ⁄ 9 homologues from the pKM101 plasmid) [35,36] (B) Cryo-EM structure of the T4SS core complex at 15 A ˚ resolution composed of TraO, TraN and TraF (VirB7 ⁄ 9 ⁄ 10) The 1.05 MDa complex spans the entire periplasmic space and forms channels in the inner and outer membranes It is subdivided into two layers: the I layer inserting into the inner membrane and the O layer inserting into the outer membrane [38] (C) Graphic representation of the VirB homologues from the Cag-T4SS: HP0527 (VirB10), HP0528 (VirB9) The coloured circles represent the homologous regions with VirB ⁄ D systems protein structures (D) Crystal structure of the O-layer with the individual components coloured as in (A) [37].

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(Fig 3C) The complex forms a large approximately

1 MDa complex spanning both the inner and the outer

membranes and containing 14 copies of each of the

three proteins [38] The structure is cylindrical (length

185 A˚, diameter 185 A˚) with two distinct layers termed

‘I’ and ‘O’ connected by narrow linkers and with a

central channel spanning the entire structure (Fig 3C)

The channel is open on the cytoplasmic side (55 A˚

opening) but constricted on the outer-membrane side

(10 A˚) Two chambers, one in each of the layers, are

clearly visible

The I and O layer connect, respectively, the inner

and outer membranes and each display double-walled

architecture but have different composition and

struc-tures [38] The I layer consists of the N-terminal part

of VirB9 and VirB10 and is inserted into the inner

membrane The I ring has a large central chamber

with a narrower 55 A˚ base that forms a cup The O

layer has two different parts, a main body and a cap

that is inserted in the outer membrane, and is formed

by the CTDs of VirB9 and VirB10 and the full-length

VirB7

The second advance has been the crystal structure of

the O-layer [37] This structure demonstrates a number

of surprising features First, VirB10 forms the outer

membrane channel Indeed, the inside of the O-layer is

lined by VirB10, and VirB10 contributes the part

crossing the outer membrane Because VirB10 is also

known to insert into the inner membrane, this endows

VirB10 with the remarkable and unique property of

spanning both membranes Second, the structure

span-ning the outer membrane is helical, instead of

b-stranded as are the vast majority of proteins forming

pores in the outer membrane Indeed, VirB10 projects

a helical segment through the outer membrane, and 14

of them form the outer membrane channel Although,

in the EM structure, this channel was

con-stricted⁄ closed (only a 10 A˚ hole was observed; see

above), in the X-ray crystal structure, the channel is

open Third, VirB9 interacts closely with VirB7 and 14

VirB7⁄ VirB9 complexes form an outer ring stabilizing

the VirB10 tetradecameric channel Finally, the CTD

of VirB10 exhibits an extended approximately 30

resi-dues sequence at its N-terminus, termed the ‘lever

arm’, which embraces three consecutive VirB10

subun-its in the tetradecameric structure and forms a

plat-form inside the O-layer Interestingly, this platplat-form

locates at a different level in the crystal and EM

struc-tures This observation, coupled with the fact that, in

the EM structure, the outer membrane channel is

closed, whereas, in the crystal structure, this channel is

open, has led to the suggestion that the lever arms

might regulate the open⁄ closed state of the channel

Although the VirB7-VirB9-VirB10 (HP0532-HP0528-HP0527) complex appears to be conserved, additional Cag-T4SS specific proteins participate in the periplasmic complex (Fig 1); for details, see the accompanying review by Fischer [21] For example, HP0532 (VirB7) does not interact directly with HP0528 (VirB9) but requires HP0537 (CagM) that sta-bilizes the translocation pore HP0528, HP0527 and HP0532 complex [34] Moreover, other protein–protein interactions occur between the core complex and the periplasmic proteins HP0538, HP0522, HP0530 and HP0537 In particular, HP0522 was found to be part

of a large complex involving several Cag proteins, including cytoplasmic, periplasmic and pilus compo-nents, and therefore might be an important part of the outer membrane complex of the T4SS [39]

The T4SS pilus T4SS pili are generally composed of two proteins, VirB2 and VirB5 VirB2 is considered the major pilin subunit and VirB5, although less abundant, decorates the external part of the appendage formed by VirB2

In the VirB⁄ D system, VirB2 is a small protein pro-cessed into a 7.2 kDa T-pilin that is cyclized before pilus formation [40,41] HP0546 was proposed to be a functional homologue of VirB2 based on sequence sim-ilarity [34,42,43] HP0546 is present in membrane frac-tions and at the bacterial surface but is only a minor component of the Cag-T4SS specific pilus (see below) [43]

The structure of TraC, the VirB5 homologue from the pKM101 T4SS, showed that the protein consists of

a helix bundle capped by a globular domain [44] (Fig 4) Mutational studies of TraC have suggested that VirB5 proteins play a role in adhesion, mediating cell–cell interaction during conjugation [44] There is

no obvious homologue of VirB5 in the cagPAI How-ever, a detailed analysis of the HP0539 (also named CagL) sequence suggested that it could be a structural homologue of VirB5, which is consistent with its inter-action with host-cell receptors and its location at the Cag-T4SS specific pilus (see below) [34,45]

Cag-T4SS specific pilus The Cag-T4SS pilus is remarkably unusual Its compo-sition appears more complex than the prototypal VirB2⁄ B5 pilus produced by other T4SS The Cag-T4SS pilus involves not only HP0546 and HP0539 pro-teins, but also HP0527, HP0528, HP0532, as well as CagA By contrast with other T4SS, there is no evi-dence suggesting that the VirB2 homologue HP0546 is

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the main component of the needle-like structure

described in two studies [46,47] Perhaps the most

sur-prising finding is that part of the translocation core

complex is also involved in the pilus external structure

Indeed, HP0527 (VirB10) and HP0532 (VirB7)

associ-ate with the pilus surface and were detected by

immu-nogold labelling [46,47] HP0527 is able to make

intramolecular interactions with itself [34] The central

region of HP0527 interacts with the C-terminal portion

and this interaction could provide a means of

oligo-merization to form a super-structure in direct

prolon-gation of the translocation pore (Fig 4) Pilus

formation might be coupled with receptor binding

because Cag-T4SS assembly first requires a contact

with epithelial cells [46] This receptor is likely to be

the a5b1 integrin Indeed, several Cag proteins,

includ-ing HP0527, HP0539, HP0540 and CagA itself, were

shown to bind to different domains of the integrin

a5b1 [48,49] This suggests that HP0540 might also be

exposed at the surface of the Cag-T4SS pilus Some of

these results are conflicting (see the accompanying

review by Fischer [21]) and a general consensus has yet

to emerge However, all studies emphasize the role of

the Cag-T4SS pilus in mediating interactions with

a5b1

Concluding remarks How the Cag-T4SS is assembled is still poorly under-stood Very recently, HP0523 was proposed to act as a lytic transglycosylase, suggesting that HP0523 might

be the H pylori homologue of VirB1 [50] VirB1 pro-teins are important with respect to piercing the pepti-doglycan layer in the periplasm Formation of the double-membrane spanning core complex formed by the HP0532-HP0528-HP0527 (VirB7-VirB9-VirB10) proteins is likely to occur next because these proteins assemble spontaneously in other T4SS [38] Because HP0527 is a major component of the Cag-T4SS pilus, core complex formation might be coupled with pilus formation Subsequent steps might include recruitment

of the cytoplasmic⁄ inner membrane ATPase complex Once assembled, the Cag-T4SS delivers two types of effectors: the CagA protein and peptidoglycan frag-ments These have different effects on the cell and it is unclear whether they are secreted together Little struc-tural information is available on the main effector CagA, which cannot be produced as a recombinant protein under standard conditions [51] However, a crystal structure of a C-terminal fragment of CagA in complex with mitogen-activated protein kinase was

Fig 4 A model of Cag-T4SS pilus assembly upon contact with the cellular receptor integrin a5b1 Protein directly interacting with the a5b1 integrins are HP0539 [49], HP0527, HP0540 and CagA [48] A possible function of these interactions would be to trigger a signal for oligo-merization of HP0527, assembly of the Cag-T4SS injection machinery, and locking of the a5b1 receptor, as suggested by [48] The pilus sub-structure is composed of the VirB2 functional homologue HP0546 (indicated by the yellow colour) of the pilus that is initially present only at some areas of the cell surface [43] The protein HP0539 (CagL) could be a homologue of VirB5 and binds to a5b1 via a RGD motif The structure of the VirB5 homologue TraC [44] is shown in ribbon representation (left) After sequential binding of CagA, HP0527 and possibly HP540 to the receptor, the assembly of the injection apparatus would take place and effectors (CagA and peptidoglycan fragments) could be translocated.

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recently determined [52] This structure was sufficient

to reveal 12 residues of CagA bound to the kinase

active site, demonstrating that the toxin inhibits the

enzyme by mimicking its natural substrate [52]

How-ever, only 12 of 120 residues bound to the kinase were

visible, suggesting that the remaining part of the

poly-peptide was unfolded in the crystal This is somehow

reminiscent of bacterial effectors delivered by other

systems that are unfolded during translocation [53] It

is therefore possible that a large part of CagA is

unfolded during and after translocation, although

more studies are necessary to decipher the structural

details of this process Although structural data are

accumulating on T4SS, a number of specific questions

remain unanswered concerning the Cag-T4SS

machin-ery This is illustrated by the structural studies of

‘not-T4SS’ Cag proteins, which have revealed that these

proteins do not resemble known structures [54–56]; see

the accompanying review by Cendron and Zanotti

[22] Therefore, although evolutionary related to other

T4SS, the Cag-T4SS displays numerous specific

fea-tures, and more studies will be necessary to obtain a

more complete understanding of this fascinating

machinery, which is involved in one of the main steps

of H pylori infection

Acknowledgements

This work was funded by grant 082227 from the

Well-come Trust to G.W and by an ATIP-Avenir and

Ligue contre le cancer grant to L.T

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