August2017

NASDAQ:AKTX

CopyrightAkariTherapeu=cs,Plc2017

COMPANYPRESENTATION

1

Cau$onaryNoteRegardingForward-LookingStatements

Certain statements in this presenta.on cons.tute "forward-lookingstatements"within themeaning of Sec.on 27Aof the Securi.esActand Sec.on 21E of the Securi.es Exchange Act and are usuallyiden.fied by the use of words such as "an.cipates," "believes,""es.mates," "expects," "intends," "may," "plans," "projects," "seeks,""should," "will," and varia.ons of suchwords or similar expressions.Weintendtheseforward-lookingstatementstobecoveredbythesafeharborprovisionsforforward-lookingstatementscontainedinSec.on27Aof the Securi.esAct and Sec.on21Eof the Securi.es ExchangeAct and are making this statement for purposes of complying withthosesafeharborprovisions.Theseforward-lookingstatementsreflectourcurrentviewsaboutourplans,inten.ons,expecta.ons,strategiesandprospects,whicharebasedontheinforma.oncurrentlyavailabletousandonassump.onswehavemade.Althoughwebelievethatourplans,inten.ons,expecta.ons,strategiesandprospectsasreflectedinorsuggestedbythoseforward-lookingstatementsarereasonable,wecan give no assurance that the plans, inten.ons, expecta.ons orstrategies will be aJained or achieved. Furthermore, actual resultsmay differ materially from those described in the forward-lookingstatementsandwillbeaffectedbyavarietyof risksand factors thatare beyond our control. Risks and uncertain.es for our companyinclude,butarenotlimitedto:needsforaddi.onalcapitaltofundouropera.ons; an inability or delay in obtaining required regulatoryapprovals forCoversinandanyotherproductcandidates,whichmayresult in unexpected cost expenditures; risks inherent in drugdevelopment in general; uncertain.es in obtaining successful clinicalresultsforCoversinandanyotherproductcandidatesandunexpectedcoststhatmayresulttherefrom;failuretorealizeanyvalueofCoversinandanyotherproductcandidatesdevelopedandbeingdeveloped inlightof inherent risksanddifficul.es involved insuccessfullybringingproductcandidatestomarket;inabilitytodevelopnewproduct

candidatesandsupportexis.ngproducts;theapprovalbytheFDAandEMA and any other similar foreign regulatory authori.es of othercompe.ngorsuperiorproductsbroughttomarket;risksresul.ngfromunforeseen side effects; risk that the market for Coversin or otherproductcandidatesmaynotbeaslargeasexpected;inabilitytoobtain,maintainandenforcepatentsandotherintellectualpropertyrightsorthe unexpected costs associatedwith such enforcement or li.ga.on;inability to obtain and maintain commercial manufacturingarrangementswith thirdpartymanufacturersorestablishcommercialscalemanufacturingcapabili.es;unexpectedcostincreasesandpricingpressures; and uncertainty of our ability to raise capital and ourinabilitytomeetworkingcapitalneeds.Manyofthesefactorsthatwilldetermine actual results are beyond our ability to control or predict.For a discussion of the factors that may cause our actual results,performance or achievements to differ materially from any futureresults, performance or achievements expressed or implied in suchforward-lookingstatements,seethe“RiskFactors”sec.onofourmostrecentlyfiled20F.Exis.ngandprospec.veinvestorsarecau.onednotto place undue reliance on these forward-looking statements, whichspeak only as of the date hereof. The statements made in thispresenta.onspeakonlyasof thedatestatedherein,andsubsequentevents and developmentsmay cause our expecta.ons and beliefs tochange.Unlessotherwiserequiredbyapplicablesecuri.eslaws,wedonotintend,nordoweundertakeanyobliga.on,toupdateorreviseanyforward-looking statements contained in this presenta.on to reflectsubsequentinforma.on,events,resultsorcircumstancesorotherwise.While we may elect to update these forward-looking statementspublicly at some point in the future, we specifically disclaim anyobliga.on to do so, whether as a result of new informa.on, futureeventsorotherwise,exceptasrequiredbylaw.

2

Developingthenextgenera$onofrare&orphanan$-inflammatorytherapies

AkariMissionStatement

Tickshaveundergone300millionyearsofnaturalselec.onto produce inhibitors that bind to key inflammatorymediatorsandarewell tolerated inhumans. Ouruniquemoleculesarederivedfromtheseinhibitors

3

Exploi$ngEvolu$onaryBenefitsofTick-DerivedProteins

Ticksalivaryproteinsworkbyinhibi.nghostimmuneresponses,enabling.cktorepeatedlyfeedwithoutdamagefromhostinflammatoryresponse

EFFICIENT

TARGETED

Targetearlyinflammatorymediators

Specificbindingavoidsoff-targeteffects

4

AkariHighlights

*PNH:Paroxysmalnocturnalhemoglobinuria;aHUS:Atypicalhemoly=c-uremicsyndrome;AKC:Atopickeratoconjunc=vi=s;BP:BullousPemphigoid

Developing.ck-derivedproteinstoinhibitearlyinflamma.on

Threeseparatedevelopmentprograms,eachfocusedondis.nctinflammatorypathways

1)Complementprogram•  PNH*-projectedPhaseIIIstart–4Q2017•  aHUS*-projectedPhaseIIstart–4Q2017•  Once-weeklydosingmoleculeindevelopment

2)DualC5&leukotrieneB4(LTB4)program•  AKC*&BP*-projectedPhaseIIstarts–1Q2018

3)Scien$ficdevelopmentprogram•  Other.ck-derivedandengineeredmolecules

5

StrategicPosi$oning

Ac.vePNH&upcomingaHUStrialsGrowinglistofothertargets

Poten.alfor20-30%marketshare

Poten.altoachievedominantmarketshareassoleprovider

Complementinhibi$on

Expectedtobe~$5billionpeakmarket

SubQdeliverydifferen.atedagainstcurrentIVstandardof

care

Leukotriene+Complementinhibi$on

Developingorphanmarket

Differen.atedMechanismofAc.on

Ac.veAKC,BP,Systemicprograms

andothertargetswithhighdegreeofunmetneed

6

PhaseIII

AkariPorYolioBuildsOnComplementExperience

TissueTargeted(NMJ)

ComplementSystem(C5)Coversin™

CoversinLA™

LTB4

BioamineSystem

LTB4(L-Coversin™)

LTB4LA

Histaminebinding

Serotoninbinding

PhaseIIPhaseI

Coversin-LTB4+C5

PreclinicalDiscovery

COMPLEMENTPROGRAM

TreatmentDura$on,Safety,andTolerabilityOfPa$entsTreatedwithCoversin

8

17months

Eculizumab-resistantPNHpa$ent(CONSENT)

Con$nuingPhase2PNHpa$ents(COBALT)*

Pa$entwhodidnotcompletePhase2study(COBALT)

•  Drugwelltoleratedbypa.ents•  NoSAEsrelatedtoCoversin•  CONSENTpa.entandthe4con.nuingCOBALTpa.entsareself-dosingandhavehadnotransfusionsduringorposttrial(todate)

•  Pa.entsdeveloplow.teran.bodiesbetween2-13weeksaqerstar.ngCoversin

•  An.bodiesnon-neutralizinginly.cassay

17Months

7Months

6Months

5Months

4Months

43Days

*Nowinlonger-termsafetystudy(CONSERVE)

9

ComplementPathwayPosi$oning

•  TwoongoingPhaseIIPNHprograms–CONSENTandCOBALT;andalong-termsafetystudy-CONSERVE

•  ResistantPNHtrial(CONSENT)–1pa.ent‒  Currentlyrecrui.ng‒  Pa.enthasbeenonCoversinsinceFebruary2016

•  NaïvePNHtrial(COBALT)-5pa.ents‒  All4con.nuingpa.entshavenowenteredlongterm

safetystudy(CONSERVE)‒  Onepa.entwithdrawnatDay43duetosuspected

comorbidityunrelatedtotreatment‒  Currentlyrecrui.ng2-3addi.onalpa.ents(protocolbeing

amendedtoinves.gatehigherdosing)

•  aHUSPhaseIItrial–upto10pa.ents

10

0

200

400

600

800

1,000

1,200

1,400

Posi$veResponseFor15Months*InEculizumab-ResistantPNHPa$entTreatedWithCoversin

CH50<8.0UEq/mL(belowlevelofdetec$on)Nohemoly$cepisodes;nodosechanges;symptomfree;twicedailyselfinjec$on

Pa=enttreatedpursuanttoanapprovedclinicalprotocolintheNetherlands2016EH,SaskiaLangemeijier,UniversityofRadboud,Nijmegen*Note:allavailableLDHdatashown;pa=enthasnowbeenontreatmentwithCoversinfor17months

LDH(IU

/ml)

Weeks8 16 24 320 40 48 56 64

1.5ULN

LDH

11

Phase2PNHTrialsUpdate

Allfivecon.nuingpa.ents(inCONSENTandCOBALT)onongoingtreatmenthaveexperienced:

•  Daily(COBALT)ortwicedaily(CONSENT)subQself-administra.on•  Noneutralizingan.bodies•  NoSAEsrelatedtoCoversin•  AEs(includingmild/moderateinjec.onsitereac.ons)

•  CH50belowlevelofquan.fica.on•  LDHreduc.on•  Notransfusions(3of5con.nuingpa.entsrequiredtransfusioninthe12monthspriortoreceivingCoversin)

•  Stablehemoglobin

12

LDH plotted as a multiple of ULN 4 patients who remained in COBALT

012345678

0 14 28 42 56 70 84

LDHxU

LN

Daysincefirstdose

MeanPatientAPatientBPatientCPatientD

0

20

40

60

80

100

120

140

0 14 28 42 56 70 84

AST(U/L)

Daysincefirstdose

MeanPatientAPatientBPatientCPatientD

Aspartate aminotransferase (AST) 4 patients who remained in COBALT

DeclinesinBloodMarkersofHemolysis–AllFourCon$nuingCOBALTPhaseIIPa$ents

Afiqhpa.ent(Pa.entE),withdrawnDay43duetosuspectedcomorbidity,hadbaselineLDHof4.8XULN&LDHof2.7XULN&2.8XULNatDay28&42,respec.vely;

baselineASTwas68U/Landfellto33and43U/LatDay28and42,respec.vely

MeanLDH1.8XULN(Day28-90)

AST83+19.2U/Latbaseline;28.5+3.3U/LDay90Coversin

NOTE:Day0dataforpa=entsAandDwasrecordedwithinsixweekspriortotrialentry

PackedRedBloodCells(PRBC)TransfusedPriortoandDuring90DayCOBALTTrial

MonthspriortostartofTrial

13

Pa.entsAandEhadnotransfusionsinthe12monthsprecedingtheCOBALTtrialorwhileinthetrial.Pa.entEwaswithdrawnfromthetrialatDay43

0

2

4

6

8

10

12

12-9 9-6 6-3 3-0 0-3

Trial

Pa.entB

Pa.entDPa.entC

Pa.entA

Pa.entE

14

PlannedPNHPhaseIIITrialsCAPSTONEandASSET

•  TwoPNHPhaseIIIclinicaltrials:‒  Naïvepa.ents(CAPSTONE)‒  Switch(ASSET)

•  CAPSTONE:Naïvepa.entsrandomizedtoCoversinplusstandardofcarevs.standardofcare

•  ASSET: Eculizumab-treatedpa.entsrandomizedtoremainoneculizumaborswitchtoCoversin

•  Indiscussionwithregulatorsregardingtrialdesigns•  CAPSTONEcurrentlyprojectedtocommence4Q2017

15

CoversinTarge$ngAtypicalHemoly$cUremicSyndrome(aHUS)

•  Chronicandlife-threateninggene.cdiseasecharacterizedbymicroangio-pathichemoly.canemia,thrombocytopenia,andkidneyinjury

•  EfficacyofC5inhibi.oninaHUSdemonstratedbytheapprovalofeculizumabforthisindica.on

•  aHUSphysicianshaveexpressedsupportforonce-dailyCoversin– Moretherapeu.cflexibilityforepisodicpa.ents– Pa.entconvenience

•  PhaseIItrial–upto10naïvepa.entsatsevensitesacrossEurope,projectedtobeginin4Q2017

DUALC5ANDLEUKOTRIENEB4(LTB4)PROGRAM

17

DualLeukotrieneandComplementInhibi$onbyCoversinHasPoten$alInWideRangeofDiseaseswithUnmetNeed

ComplementOnly

Leukotrine&Complement

Asthma

RheumatoidArthri.sPAH

Trauma

Sjögren’s

Bronchioli.sobliterans

COPD

AATD

BullousPemphigoid

Goodpasture’s

AKC

DryEye

PNH aHUS

MucousMembranePemphigoid

MG NMO

18

TwoPhaseIILeukotriene/ComplementTrialsProjectedtoStartin1Q2018

•  PhaseIIprograms‒  AKC(atopic

keratoconjunc.vi.s)-eye(topicaldrops)

‒  BP(bullouspemphigoid)–skin(subQ)

•  Severalseverelungcondi.onsareimpactedbycomplementandleukotrienepathwayswithzileutonandmontelukastbothprescribed

•  Dualac.vitycreatespoten.alforuniquetreatmentop.on

DatafromPneumolabs2017

Dualac$on(Coversin)moreeffec$vethanC5-only(saturatedCoversin)or

Zileutonaloneinmousemodel

Coversin

(inhibitsC5+LTB4

)SaturatedCo

versin

(inhibitsC5on

ly)

Zileuton

(inh

ibits

Leuk

otrie

ne/

LTB4

)

NeutrophilrecruitmenttomouselunginducedbyLPSinpresenceof

CoversinorZileuton

Coversin&L-CoversinPoten$allySuperiorStrategytoTargetLTB4

PreviousstrategiestotargetLTB4sufferedfromalackofselec$vity;benefitsofreducingLTB4wereoffsetbyoff-targeteffects

CoversinandL-CoversincaptureLTB4withinaninternalbindingsite•  Directlyandspecifically“mopup”LTB4

withoutperturbingotherpathways•  UniqueLTB4targe.ngselec.vity

‒  Expectednottoreducean.-inflammatorylipoxins

‒  DoesnotinhibitPGP(anan.-inflammatoryagent)degrada.on

Roversi,P.etal.,JBiol.Chem.2013

19

LTB4Interven$onStrategies

Arachidonicacid

LTA4

LipoxinsLTB4LTC4LTD4LTE4

5-LOFLAP

LTA4HLTC4S

Lipoxygenases

Pro-inflammatory

Anti-inflammatory

Spasmogenic

Zileuton

GSK2190915DG-031

MK-0633TA-270

5-LO/FLAPinhibitors

Reducesan.-inflammatorylipoxins

BLT1/BLT2antagonists

Realiza.onthatan.-inflammatorymediatorsalsosignalthroughBLT1/BLT2

LTA4Hinhibitors

Secondaryan.-inflammatoryroleforLTA4H

indegradingpro-inflammatory/remodelling

mediatorPGPBLT1 BLT2

LY-293111ONO-4057BIIL284CP105696

DG051JNJ-40929837AcebilutsatUbeniimex

20

Limita$onsofOtherLTB4Inhibitors

DUALACTIONEYE(AKC)ANDSKIN(BP)PROGRAM

Collabora.onwithMoorfieldsHospital(Ins.tuteofOphthalmology)EICpre-clinicalmodelofsevereeyesurfaceinflamma.on

DemonstratedBenefitinMouseModelLate-PhaseOcularInflamma$on

•  C57/Bl6micesensi=zedtoOVAfor14days•  OVAeyesurfacechallengefor6dayspostsensi=za=on(days0–6)•  Coversinappliedoncedailyondays3–6a_erinflammatoryresponsewellestablished•  Maximumeffectseena_er3daysofCoversintreatment(latephaseofinflamma=on)

•  Coversin-64%reduc$onininflammatoryscorecomparedtoplaceboinmousemodel

•  Timingindica.veofTcellresponse•  Historicalcomparison*:‒  CyclosporinA(0.1%)-43%reduc.on

‒  Betamethasone(0.1%)-nosignificantreduc.on0

12345678

Day3 Day4 Day5 Day6

Inflamma.

onsc

ore

EffectofCoversinonOVAinducedinflamma$on(n=16pergroup)

Placebo Coversin

p<0.0001

p<0.01

*ShiiD,NakagawaS,YoshimiMetal.2010;BiolPharmBull33(8):1314–1318 22

AtopicKeratoconjunc$vi$s(AKC)

•  Severeeyesurfaceinflamma.oncausinginfiltra.onofimmunecellssuchasneutrophilsandTcells.Acauseofblindnessworldwide

•  Topicaldrugs,suchassteroidsorcyclosporin,oqennoteffec.veorcannotbegivenchronically

•  InAKCdisease,despitebestcurrenttreatmentmanypa.entsprogresstoseverecornealinvolvement

•  BothcomplementandLTB4knowntobeinvolved

•  Progressestoaffectthecorneaandmayleadtolossofvision.Botheyesaffected

23

•  Therapeu.csuccessinthisindica.oncouldopenupothercicatrisingeyesurfacecondi.ons

•  PhaseI/IIrandomized,doublemasked,placebo-controlledtrialatMoorfieldsEyeHospitalandRoyalLiverpoolHospitalprojectedtostart1Q2018

CoversinDemonstratedBenefitinBullousPemphigoidModel

PreclinicalpassivemousemodelfromDr.SadikinLubeck,GermanyLeadingBullousPemphigoidcenter

P=0.0023betweenvehicleand250µg/kg

•  ~60%reduc.oninaffectedareaonCoversin(SubQ)comparedtovehicleorsteroidinmousemodel

•  Cleardoseresponse

Vehicle 250µg/kgCoversin

24

25

BullousPemphigoid(BP)SignificantUnmetNeed

•  Immunecomplexdeposi.onini.atescomplementcascadeandinflammatoryprocess•  LTB4recruitsneutrophilstodermis-epidermisjunc.onandamplifiesinflamma.on•  PhaseIIopenlabeltrialagainststandardofcareprojectedtostartin1Q2018inthreecentersinEurope

SCIENTIFICDEVELOPMENTPROGRAM

27

AkariDiscovery

PlaYorm

Coversinengineeredmoleculestargetedtocomplementpathway

OtherAkari.ck-derivedmolecules

•  Eicosanoidpathway(LTB4only)L-Coversin(lung)

•  Bioaminepathway

•  Extendedhalflife(LA)

‒ Ligandcapturepreven.ngac.ononmul.pleGPCRs

‒ Similarbiophysicalproper.estoCoversin

•  Tissuetarge.ng(NMJ*)(MyastheniaGravis)

*Neuromuscularjunc=on(NMJ)isthesiteofcommunica=onbetweenmotornerveaxonsandmusclefibers

Coversin

28

CoversinLA:OnceWeeklyFormula$on

0

20

40

60

80

100

120

0.01 0.1 1

%TotalLysis

CoversinEculizumabPASCoversinCoversin&Eculizumab

Drugconcentra$on(µM)

Inhibi$onofcomplementalterna$vepathwayrabbitredbloodcelllysis

PASCoversin

Coversin

5%

HumanPKsimula$onoffreeC5followingsingledoseinjec$onpoten$alforweeklydosing

•  Terminalhalflifeinhumanses.matedatfourdaysbasedonpharmacokine.c(PK)datainmiceandrats

•  Cleanini.altoxicologyprofilecomparabletounmodifiedCoversin•  PhaseIclinicalstudyplannedfor2018Sources:BASTInc.Limited,UCLLaboratories

29

FinancialSummaryMarch31,2017

• ADS*outstanding 11,776,934

• ADSFullyDiluted 12,583,641

•  CashandCashEquivalents** $35.1m(nodebt)

* EachADSrepresents100ordinaryshares**Includesshort-terminvestments

30

AkariClinicalProgramHighlights

Complementprogram

•  PNH-projectedPhaseIIIstart–4Q2017 -addi.onalPhaseIIpa.ents–4Q2017•  aHUS-projectedPhaseIIstart–4Q2017•  CoversinLA-projectedPhaseIstart–4Q2018

DualC5&leukotrieneB4(LTB4)program

• AKC&BP-projectedPhaseIIstarts–1Q2018

•  Threeofthefourcon.nuingpa.entswereupdosed– Pa.entAandBwereupdosedfrom30mgto45mgoncedailyatDays40and54,respec.vely– Pa.entCwasupdosedto22.5mgtwicedailyatDay24andmovedto45mgoncedailyatDay67– Pa.entB,thelastintodate,didnotseeadeclineinLDHwithupdosing,althoughhishemoglobinlevelroseaqerDay67

•  PrimaryendpointofLDH<1.8XULNatDay28wasachievedbytwoofthefivepa.ents

•  LDHasamul.pleofULN(xULN)forthe5pa.ents(A,B,C,DandE)atDay28wasrespec.vely1.4,2.2,2.5,1.4and2.7

•  Pa.entEwaswithdrawnatDay43•  Forthefourcon.nuingpa.ents,xULNatDay60was1.5,2.1,1.8and1.5;andatDay90,1.6,2.4,2.0and1.9

COBALTPhaseIIDosingandResponseSummary

31

August2017

NASDAQ:AKTX