Akari Therapeutics PLC · NASDAQ:AKTX

Akari Therapeutics — Investor Presentation (August 2017)

33 slides from Akari Therapeutics's investor presentation, August 2017

1. Slide 1: Company Presentation

   NASDAQ: AKTX COMPANY PRESENTATION August 2017 Copyright Akari Therapeu=cs, Plc 2017

2. Slide 2: Cautionary Note Regarding Forward-Looking Statements

   Cau$onary Note Regarding Forward-Looking Statements Certain statements in this presenta.on cons.tute "forward-looking candidates and support exis.ng products; the approval by the FDA and statements" within the meaning of Sec.on 27A of the Securi.es Act EMA and any other similar foreign regulatory au

3. Slide 3: Akari Mission Statement

   Akari Mission Statement Developing the next genera$on of rare & orphan an$-inflammatory therapies Ticks have undergone 300 million years of natural selec.on to produce inhibitors that bind to key inflammatory mediators and are well tolerated in humans. Our unique molecules are derived from these inhib

4. Slide 4: Exploiting Evolutionary Benefits of Tick-Derived Proteins

   Exploi$ng Evolu$onary Benefits of Tick-Derived Proteins Tick salivary proteins work by inhibi.ng host immune responses, enabling .ck to repeatedly feed without damage from host inflammatory response EFFICIENT Target early inflammatory mediators TARGETED Specific binding avoids off-target effects 3

5. Slide 5: Akari Highlights

   Akari Highlights Developing .ck-derived proteins to inhibit early inflamma.on Three separate development programs, each focused on dis.nct inflammatory pathways 1) Complement program • PNH* - projected Phase III start – 4Q 2017 • aHUS* - projected Phase II start – 4Q 2017 • Once-weekly dosing molecule

6. Slide 6: Strategic Positioning

   Strategic Posi$oning Leukotriene Complement inhibi$on + Complement inhibi$on Expected to be Developing ~$5 billion peak market orphan market Sub Q delivery Differen.ated differen.ated against Mechanism of Ac.on current IV standard of care Ac.ve AKC, BP, Systemic Ac.ve PNH & upcoming aHUS trials progra

7. Slide 7: Akari Portfolio Builds On Complement Experience

   Akari PorYolio Builds On Complement Experience Discovery Preclinical Phase I Phase II Phase III Complement System (C5) Coversin™ Coversin LA™ Tissue Targeted (NMJ) LTB4 Coversin - LTB4 + C5 LTB4 (L-Coversin™) LTB4 LA Bioamine System Histamine binding Serotonin binding 6

8. Slide 8: Complement Program

   COMPLEMENT PROGRAM

9. Slide 9: Treatment Duration, Safety, and Tolerability Of Patients Treated with Coversin

   Treatment Dura$on, Safety, and Tolerability Of Pa$ents Treated with Coversin Eculizumab-resistant PNH pa$ent (CONSENT) 17 Months • Drug well tolerated by pa.ents 17 months Con$nuing Phase 2 PNH pa$ents (COBALT)* • No SAEs related to Coversin • CONSENT pa.ent and the 4 7 Months con.nuing COBALT pa.en

10. Slide 10: Complement Pathway Positioning

    Complement Pathway Posi$oning • Two ongoing Phase II PNH programs – CONSENT and COBALT; and a long-term safety study - CONSERVE • Resistant PNH trial (CONSENT) – 1 pa.ent ‒ Currently recrui.ng ‒ Pa.ent has been on Coversin since February 2016 • Naïve PNH trial (COBALT) - 5 pa.ents ‒ All 4 con.nuing

11. Slide 11: Positive Response For Months In Eculizumab-Resistant PNH Patient Treated With Coversin

    Posi$ve Response For 15 Months* In Eculizumab- Resistant PNH Pa$ent Treated With Coversin 1,400 1,200 1,000 LDH (IU/ml) 800 LDH 600 400 200 1.5 ULN 0 0 8 16 24 32 40 48 56 64 Weeks CH50 <8.0 U Eq/mL (below level of detec$on) No hemoly$c episodes; no dose changes; symptom free; twice daily self injec

12. Slide 12: Phase 2 PNH Trials Update

    Phase 2 PNH Trials Update All five con.nuing pa.ents (in CONSENT and COBALT) on ongoing treatment have experienced: • Daily (COBALT) or twice daily (CONSENT) subQ self-administra.on • No neutralizing an.bodies • No SAEs related to Coversin • AEs (including mild/moderate injec.on site reac.ons) • CH50

13. Slide 13: Declines in Blood Markers of Hemolysis – All Four Continuing COBALT Patients

    Declines in Blood Markers of Hemolysis – All Four Con$nuing COBALT Phase II Pa$ents LDH plotted as a multiple of ULN Aspartate aminotransferase (AST) 4 patients who remained in COBALT 4 patients who remained in COBALT 8 140 Mean Mean 7 Patient A 120 Patient A 6 Patient B 100 Patient B LDH x ULN 5 Pa

14. Slide 14: Packed Red Blood Cells Transfused Prior to and During 90 Day COBALT Trial

    Packed Red Blood Cells (PRBC) Transfused Prior to and During 90 Day COBALT Trial 12 Pa.ent A 10 Pa.ent B Pa.ent C 8 Pa.ent D Pa.ent E 6 4 2 0 12-9 9-6 6-3 3-0 0-3 Months prior to start of Trial Trial Pa.ents A and E had no transfusions in the 12 months preceding the COBALT trial or while in the tria

15. Slide 15: Planned PNH Phase III Trials CAPSTONE and ASSET

    Planned PNH Phase III Trials CAPSTONE and ASSET • Two PNH Phase III clinical trials: ‒ Naïve pa.ents (CAPSTONE) ‒ Switch (ASSET) • CAPSTONE: Naïve pa.ents randomized to Coversin plus standard of care vs. standard of care • ASSET: Eculizumab-treated pa.ents randomized to remain on eculizumab or switc

16. Slide 16: Coversin Targeting Atypical Hemolytic Uremic Syndrome (aHUS)

    Coversin Targe$ng Atypical Hemoly$c Uremic Syndrome (aHUS) • Chronic and life-threatening gene.c disease characterized by microangio-pathic hemoly.c anemia, thrombocytopenia, and kidney injury • Efficacy of C5 inhibi.on in aHUS demonstrated by the approval of eculizumab for this indica.on • aHUS physi

17. Slide 17: Dual C5 and Leukotriene B4 (LTB4) Program

    DUAL C5 AND LEUKOTRIENE B4 (LTB4) PROGRAM

18. Slide 18: Dual Leukotriene and Complement Inhibition Has Potential In Wide Range of Diseases

    Dual Leukotriene and Complement Inhibi$on by Coversin Has Poten$al In Wide Range of Diseases with Unmet Need Complement Only PNH aHUS MG NMO Leukotrine & Complement Sjögren’s Bullous Pemphigoid Mucous Membrane Pemphigoid AKC Bronchioli.s obliterans Goodpasture’s AATD PAH Rheumatoid Arthri.s Dry Eye

19. Slide 19: Two Phase II Leukotriene/Complement Trials Projected to Start in 1Q 2018

    Two Phase II Leukotriene/Complement Trials Projected to Start in 1Q 2018 Dual ac$on (Coversin) more effec$ve • Phase II programs than C5-only (saturated Coversin) or ‒ AKC (atopic Zileuton alone in mouse model keratoconjunc.vi.s) - eye (topical drops) ‒ BP (bullous pemphigoid) – skin (sub Q) • Severa

20. Slide 20: Coversin & L-Coversin Potentially Superior Strategy to Target LTB4

    Coversin & L-Coversin Poten$ally Superior Strategy to Target LTB4 Previous strategies to target LTB4 suffered from a lack of selec$vity; benefits of reducing LTB4 were offset by off-target effects Coversin and L-Coversin capture LTB4 within an internal binding site • Directly and specifically “mop up” LTB

21. Slide 21: LTB4 Intervention Strategies

    LTB4 Interven$on Strategies Limita$ons of Other Arachidonic acid LTB4 Inhibitors Zileuton MK-0633 5-LO 5-LO / FLAP inhibitors FLAP DG051 TA-270 GSK2190915 Reduces an.-inflammatory JNJ-40929837 LTA4 DG-031 lipoxins Acebilutsat Ubeniimex Lipoxygenases BLT1/BLT2 antagonists LTA4H Realiza.on that an.- LT

22. Slide 22: Dual Action Eye (AKC) and Skin (BP) Program

    DUAL ACTION EYE (AKC) AND SKIN (BP) PROGRAM

23. Slide 23: Demonstrated Benefit in Mouse Model Late-Phase Ocular Inflammation

    Demonstrated Benefit in Mouse Model Late-Phase Ocular Inflamma$on Collabora.on with Moorfields Hospital (Ins.tute of Ophthalmology) EIC pre-clinical model of severe eye surface inflamma.on Effect of Coversin on OVA induced inflamma$on (n=16 per group) • Coversin - 64% reduc$on in inflammatory score compare

24. Slide 24: Atopic Keratoconjunctivitis (AKC)

    Atopic Keratoconjunc$vi$s (AKC) • Severe eye surface inflamma.on causing infiltra.on of immune cells such as neutrophils and T cells. A cause of blindness worldwide • Topical drugs, such as steroids or cyclosporin, oqen not effec.ve or cannot be given chronically • In AKC disease, despite best current

25. Slide 25: Coversin Demonstrated Benefit in Bullous Pemphigoid Model

    Coversin Demonstrated Benefit in Bullous Pemphigoid Model • ~60% reduc.on in affected area on Coversin (Sub Q) compared to vehicle or steroid in mouse model • Clear dose response P=0.0023 between vehicle and 250 µg/kg Vehicle 250µg/kg Coversin Preclinical passive mouse model from Dr. Sadik in Lubeck,

26. Slide 26: Bullous Pemphigoid (BP) Significant Unmet Need

    Bullous Pemphigoid (BP) Significant Unmet Need • Immune complex deposi.on ini.ates complement cascade and inflammatory process • LTB4 recruits neutrophils to dermis-epidermis junc.on and amplifies inflamma.on • Phase II open label trial against standard of care projected to start in 1Q 2018 in three cen

27. Slide 27: Scientific Development Program

    SCIENTIFIC DEVELOPMENT PROGRAM

28. Slide 28: Akari Discovery Platform

    Akari Discovery PlaYorm Coversin engineered molecules targeted to Other Akari .ck-derived complement pathway molecules • Extended half life (LA) • Eicosanoid pathway (LTB4 only) L-Coversin (lung) Coversin • Bioamine pathway • Tissue targe.ng (NMJ*) ‒ Ligand capture preven.ng (Myasthenia Gravis) ac.o

29. Slide 29: Coversin LA: Once Weekly Formulation

    Coversin LA: Once Weekly Formula$on Human PK simula$on of free C5 Inhibi$on of complement alterna$ve following single dose injec$on pathway rabbit red blood cell lysis poten$al for weekly dosing 120 Coversin Eculizumab 100 PAS Coversin Coversin &Eculizumab 80 % Total Lysis Coversin 60 40 5% PAS Cove

30. Slide 30: Financial Summary March 31, 2017

    Financial Summary March 31, 2017 • ADS* outstanding 11,776,934 • ADS Fully Diluted 12,583,641 • Cash and Cash Equivalents** $35.1m (no debt) * Each ADS represents 100 ordinary shares ** Includes short-term investments 29

31. Slide 31: Akari Clinical Program Highlights

    Akari Clinical Program Highlights Complement program • PNH - projected Phase III start – 4Q 2017 - addi.onal Phase II pa.ents – 4Q 2017 • aHUS - projected Phase II start – 4Q 2017 • Coversin LA - projected Phase I start – 4Q 2018 Dual C5 & leukotriene B4 (LTB4) program • AKC & BP - projected Phase I

32. Slide 32: COBALT Phase II Dosing and Response Summary

    COBALT Phase II Dosing and Response Summary • Three of the four con.nuing pa.ents were updosed – Pa.ent A and B were updosed from 30 mg to 45 mg once daily at Days 40 and 54, respec.vely – Pa.ent C was updosed to 22.5 mg twice daily at Day 24 and moved to 45 mg once daily at Day 67 – Pa.ent B, the l

33. Slide 33: Akari Therapeutics August 2017

    iN AKARI THERAPEUTICS NASDAQ: AKTX August 2017

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