Biohaven Ltd. · NYSE:BHVN
Biohaven — Investor Presentation (August 2026)
84 slides from Biohaven's investor presentation, August 2026
Slide 1: Corporate Presentation
Corporate Presentation CAMERON Living with Graves’ Disease August 2026 © 2026 Biohaven, Ltd. All rights reserved.
Slide 2: Forward-Looking Statement
Forward-Looking Statement This presentation includes forward-looking statements within the meaning of the Private Securities Litigation Reform Act of 1995, including statements about Biohaven Ltd. (the “Company”) and our planned and ongoing trials (including those for our taldefgrobep alfa, opakalim
Slide 3: Development pipeline
PRECLINICAL PHASE 1 PHASE 1B/PHASE 2 PHASE 3 MARKET IgG Degrader BHV-1300 Common Disease (Graves’, RA) Gd-IgA1 Degrader BHV-1400 IgA Nephropathy INFLAMMATION & IMMUNOLOGY β1AR AAb Degrader BHV-1600 Peripartum Cardiomyopathy TYK2/JAK1 Inhibitor BHV-8000 Parkinson’s Disease (brain-penetrant) Kv7 Activ
Slide 4: Targeting the Root Cause of Autoimmune Disease
DEGRADERS: MoDE AND TRAP Targeting the Root Cause of Autoimmune Disease
Slide 5: Innovation in Protein Degradation: Harnessing Hepatic ASGPR
Innovation in Protein Degradation: Harnessing Hepatic ASGPR for Efficient and Safe Removal of Circulating Pathogenic Targets BIFUNCTIONAL DEGRADERS MoDE DEGRADERS ASGPRs* ON HEPATOCYTE MoDE and TRAP 1 degraders bind circulating target and efficiently deliver to ASGPR on hepatocytes DEGRADATION TARGE
Slide 6: First Extracellular Protein Degraders in the Clinic
First Extracellular Protein Degraders in the Clinic Demonstrate MoDE and TRAP Rapid and Robust Pharmacodynamic Effects and Compelling DEGRAD ERS Safety in Nearly 200 Individuals Dosed BHV-1300 Safe Well-tolerated Highly selective Deep and rapid lowering Patient outcomes BHV-1400 Pivotal trials mid-2
Slide 7: MoDE and TRAP Degraders
~400K patients $11.1B MoDE MoDE and TRAP BHV-13 10 IgG Degrader gMG ~92K patients $2.6B >1M patients | $7.6B Systemic sclerosis Degraders AE ITP BHV-14 50 IgG4 degrader Pemphigus vulgaris MuSK MG IgG4-RD CIDP BHV-13 00 PI PELI NE ASSETS 8+ IgG degrader Graves’ disease · RA Sjögren's · Biologic failu
Slide 8: Targeting the Root Cause of Graves' Disease
IgG antibody IgG MoDE DEGRADER: BHV-1300 BHV-1300 Targeting the Root Cause of Graves’ Disease
Slide 9: Demographics: Graves' Disease Impacts 1% of the Global Population
Demographics: Graves’ Disease Impacts 1% of the Global Population DEGRADERS Worldwide incidence of Graves’ disease2 Graves’ disease affects nearly 1 in 100 Americans.1 Sweden F/M: 21/100K per уear 2003–2005 United Kingdom Denmark F: 50/100K per уear 1972–1993 F/M: 31/100K per уear 1997–2000 United S
Slide 10: The Under-Recognized Burden of Graves' Disease
The Under-Recognized Burden of Graves' Disease1 DEGRADERS Fatigue 93% Palpitations 64% 93% report multiple symptoms (≥2) Anxiety 64% Rapid heartbeat 64% Depression 54% 79% experience 4+ symptoms Bulging/irritated eyes 43% Insomnia 43% 1. Patient Burden in Graves’ Disease: Results From a Mixed Metho
Slide 11: TSHR Autoantibodies Cause Hyperthyroidism
TSHR Autoantibodies (TSHR-IgG1) Cause Hyperthyroidism DEGRADERS Autoimmune Hyperthyroidism Graves’ disease hypothyroidism (from a toxic nodule) hyperthyroidism (Hashimoto’s) Thyroid stimulating hormone (TSH) Thyroxine (T4) Thyronine (T3) Thyroid receptor antibody (TRAb) Thyroid stimulating immunoglo
Slide 12: Current Treatments for Graves' Disease
Current Treatments for Graves’ Disease DEGRADERS CONSERVATIVE I-131 Radiation 1 Use of thionamides (ATDs, like methimazole, carbimazole, ATDs and propylthiouracil (PTU) Aplastic anemia, agranulocytosis, peripheral neuritis, liver issues, secreted in breastmilk NSERVATIVE ABLA IVE 2 Use of steroids T
Slide 13: BHV-1300 MoDE Targets the Root Cause of Graves' Disease
BHV-1300 MoDE Targets the Root Cause of Graves' Disease and TSHR Autoantibody-Driven Diseases DEGRADERS EXCESS HORMONES S TA B I L I Z E D H O R M O N E S T3 T4 TSHR T3 T4 AUTOANTIBODY DRIVEN DISEASES Thyroid eye disease BHV-1300 Graves’ embryopathy Thyroid dermopathy Graves’ BHV-1300 degrades disea
Slide 14: Not an FcRn Inhibitor: Novel MOA
Not an FcRn Inhibitor: Biohaven IgG MoDE Degrader Differentiates as a Novel MOA, Potential Paradigm Shifting Therapy DEGRADERS 83% IgG LOWERING • 74.6% IgG lowering after load, 68.8% BY DAY 18 IMAAVY in maintenance in Vivacity MG-31,2 • IV infusion IgG antibody • Increased cholesterol (24%), muscle
Slide 15: BHV-1300 Removes TSHR Autoantibody and Normalizes Thyroid Hormones
BHV-1300 MoDE Deeply Removes Disease-Driving TSHR Autoantibody and Normalizes Thyroid Hormones DEGRADERS TRAb Change from Baseline Change from Baseline Free T4 Change from Baseline Free T3 20 45 20 19 18 18 TSHR Autoantibody 40 Free T4 Free T3 17 16 16 14 35 15 14 12 13 BHV-1300 30 12 10 RAPIDLY REM
Slide 16: BHV-1300 Rapidly Normalizes Thyroid Hormones in Graves' Hyperthyroidism
BHV-1300 Rapidly Normalizes Thyroid Hormones in Patients With Graves’ Hyperthyroidism DEGRADERS Kaplan-Meier Curve of Time to Kaplan-Meier Curve of Time to Normalization of Free T4 Normalization of Free T3 1 1 Free T4 Free T3 0.8 0.8 Survival Probability Survival Probability 0.6 0.6 0.4 0.4 0.2 0.2
Slide 17: Lead MoDE Degrader BHV-1300 Enters Phase 3
Lead MoDE Degrader, BHV-1300, Enters Phase 3 DEGRADERS DATA READOUT Placebo Screening Biohaven IgG degrader 26 weeks Normal TSH, T3, T4 (26 Weeks) off ATD Study Design: Randomized, double-blind, placebo-controlled trial KEY STUDY Population: Male and female adults with Graves’ disease DETAILS Endpoi
Slide 18: Targeting the Root Cause of IgA Nephropathy
Gd-IgA1 Gd-IgA1 TRAP DEGRADER: BHV-1400 BHV-1400 Targeting the Root Cause of IgA Nephropathy
Slide 19: Kidney Function Already Compromised at IgAN Diagnosis
By the Time IgAN Is Diagnosed, Kidney Function Is Already Significantly Compromised DEGRADERS IgAN IgAN predominantly affects people in their MOST PRODUCTIVE YEARS OF LIFE Camille IgA Diagnosed with nephropathy 20s 30s IgAN in 2020 at age 25 MED IAN AGE MOST COMMON AT DIAGNOS IS1 AGE RANGE1 A rare c
Slide 20: KDIGO Guidelines Focus on Removal of Pathogenic Gd-IgA1
Guidelines Focus on Removal of Pathogenic Gd-IgA1 KDIGO DEGRADERS 20 August 2026 Biohaven Corporate Presentation
Slide 21: BHV-1400: Selective Removal of Gd-IgA1 vs Market Competitors
BHV-1400: Selective Removal of Disease-Causing Gd-IgA1 Without Immunosuppression Compared to Market Competitors DEGRADERS TRAP Degrader Gd-IgA1 BROAD TARPEVO® BHV-1400 IMMUNOSUPRESSION IgD SELECTIVELY DEGRADES ONLY Gd-IgA1 Targeting the INHIBIT COMPLEMENT SYSTEM WITH BROAD IMMUNOSUPPRESSION pathogen
Slide 22: BHV-1400 Selectively Removes Gd-IgA1 Without Suppressing Healthy IgA
BHV-1400 500 mg SC Bi-Monthly Deeply and Selectively Removes Gd-IgA1 Without Suppression of Normal Healthy IgA in Patients With IgAN DEGRADERS Healthy Immunoglobulins Gd-IgA1 20% Day 3 Day 17 Day 31 0% STABLE 0 n=5 n=6 n=3 FROM % Change from Baseline ~48 Hours Post-Dose -20% -40% BASELINE IgA -60% D
Slide 23: Competitors Show Long-Term Immunosuppression
Competitors Show Long-Term Immunosuppression1,2 DEGRADERS Mezagitamab 600 mg SC 2,4 Atacicept 150 mg2,5 VOYXACT SC2,6 20% 20% 20% 10% 10% 10% 0% 0% 0% -10% 62% DROP -10% 64% DROP -10% 69% DROP -20% FROM -20% FROM -20% FROM -30% -30% -30% V OY X AC T FDA L A B E L7 -40% BASELINE -40% BASELINE -40% BA
Slide 24: BHV-1400 Hematuria Case Report
BHV-1400 Hematuria Case Report: De-Challenge Re-Challenge Recaptures Complete Hematuria Resolution DEGRADERS Drug Drug de-challenge re-challenge 3 3+ 2 2+ Hematuria 1 1+ Resolved 0 Resolved 1 17 31 45 51 173 192 235 DAY DOSE DOSE DOSE DOSE DOSE DOSE DOSE DAY 1 DAY 15 DAY 29 DAY 192 DAY 206 DAY 220 D
Slide 25: BHV-1400 Phase 1b IgAN Safety Summary
BHV-1400 Phase 1b IgA Nephropathy Patient Cohort — Safety Summary DEGRADERS • 10 participants with IgA nephropathy • Most AEs were mild and self-resolving • No treatment discontinuations for adverse events • No clinically significant trends in vitals, ECGs, or labs (including AST/ALT/Tbili) • No cli
Slide 26: Lead TRAP Degrader BHV-1400 Planned Phase 3 Initiation
Lead TRAP Degrader BHV-1400 Planned Phase 3 Initiation in 2026 DEGRADERS Updated DATA READOUT 52-week Treatment Phase 52 Weeks BHV-1400 (n=280) Screening BHV-1400 OL Placebo (n=140) Study Design: Randomized, double-blind, placebo-controlled trial KEY STUDY Population: Male and female adults with bio
Slide 27: Revolutionizing Epilepsy Treatment With a Modern Kv7 Activator
OPAKALIM Kv7 ACTIVATOR SELECTIVE Kv7 ACTIVATOR: OPAKALIM ION CHANNEL Revolutionizing Epilepsy Treatment With a Modern Kv7 Activator
Slide 28: An Epilepsy Treatment Designed With Patients and Physicians in Mind
An Epilepsy Treatment Designed With Patients and Physicians in Mind Kv7 Opakalim offers potential for easy-to-use, once-daily treatment with no titration to control seizures without the burdensome side effects frequently reported with approved ASMs and those in development Selectively activates Kv7.
Slide 29: Idiopathic Generalized Epilepsy Time-to-Event Study Design
Idiopathic Generalized Epilepsy Time-to-Event Study Design Kv7 Double-blind Phase 24-weeks Time-to-Event Endpoint: Scenarios Randomization (1:1) Week Week 0 24 Up-to 45 Days Opakalim 75 mg OLE Phase Completes 24 weeks 0 GTC seizures Enters OLE ✓ Screening Opakalim 75 mg 1s t Completes 24 weeks 1 GTC
Slide 30: Demographics and Baseline Disease Characteristics
Demographics and Baseline Disease Characteristics Kv7 Opakalim 75 mg Placebo n=15 n=12* Age (mean) 37 43 Sex (% female) 73% 83% Region (% US) 47% 25% BMI (mean) 27 28 Number of epilepsy treatments at screening 1 to 2 60% 67% 3 to 4 40% 33% Number of previous and current ASMs ≤6 11 (73%) 10 (83%) >6
Slide 31: Opakalim Prolongs Time-to-2nd GTC Seizure
Opakalim Prolongs Time-to-2nd GTC Seizure Kv7 M E D I A N T I M E - T O - 2 ND G T C S E I Z U R E OPAKALIM Opakalim n=15 141DAYS PLACEBO Placebo n=11* 47DAYS 3x LONGER FOR SUBJECTS ON OPAKALIM VS. PLACEBO 0 20 40 60 80 100 120 140 160 Days * In placebo group, 1 subject discontinued early due to stu
Slide 32: Opakalim Prolongs Time-to-2nd GTC Seizure and Time in Double-Blind
Opakalim Prolongs Time-to-2nd GTC Seizure and Time in Double-Blind Kv7 OPAKALIM PLACEBO 1st GTC End of Double-Blind Phase Individual Subjects 0 20 40 60 80 100 120 140 160 180 Days in Double-Blind Phase 32 August 2026 Biohaven Corporate Presentation
Slide 33: Opakalim Prolongs Time in Double-Blind and Enhances Seizure Control
Opakalim Prolongs Time in Double-Blind and Enhances Seizure Control Kv7 33% of Opakalim-Treated Subjects Completed 20% of Opakalim-Treated Subjects Completed Six-Month Double-Blind Phase Without 2nd GTC Double-Blind Phase Seizure Free 40 40 Completion Rate Without 2nd GTC (%) Completion Rate Without
Slide 34: Promising Case of Broad-Spectrum Generalized Seizure Efficacy
Promising Case of Broad-Spectrum Generalized Seizure Efficacy Kv7 Our patient has very difficult-to-treat IGE, since entering the opakalim trial she has experienced reduction in all seizure types. GTC Myoclonic Absence 50 42 10 200 8 159 40 8 43-day 150 SCREENING phase 30 6 50-day 100 68 20 4 DOUBLE
Slide 35: Efficacy Signal Demonstrated in KCNQ2-DEE
Efficacy Signal Demonstrated in KCNQ2-DEE Kv7 • Heterozygous for Kv7.2 G281E mutation 9-year-old boy with • Daily tonic seizures at baseline despite 3 ASMs including 1 st gen Kv7 activator • Refractory KCNQ2-DEE • Prior attempts to taper 1st gen Kv7 activator resulted in status epilepticus, ICU • Kv
Slide 36: Efficacy Signal Observed in Focal Epilepsy Open-Label Data
Efficacy Signal Observed in Focal Epilepsy Open-Label Data Kv7 Double-blind Phase 8-Weeks RISE 2* OLE Phase Observation Phase (OP) Opakalim 75 mg RISE 3 SEIZURE FREQUENCY SEIZURE FREQUENCY vs. Pretreatment Baseline in OP On Treatment with Opakalim 75 mg in OLE >54% 50% RESPONSE OVER ANY CONSECUTIVE
Slide 37: Exceptional Tolerability Observed in Focal Epilepsy Open-Label Data
Exceptional Tolerability Observed in Focal Epilepsy Open-Label Data Kv7 Preferred Term Opakalim 50 mg Opakalim 75 mg Opakalim Pooled Headache 4.5% 6.4% 5.7% Nasopharyngitis 4.5% 6.4% 5.7% Seizure 5.3% 3.7% 4.3% Dizziness 3.0% 5.0% 4.3% Fatigue 3.0% 4.1% 3.7% Fall 2.3% 4.6% 3.7% Upper respiratory tra
Slide 38: Opakalim Favorable Tolerability vs. Azetukalner
Opakalim Demonstrates Favorable Tolerability vs. Azetukalner in Focal Epilepsy Open-Label Trials Kv7 Opakalim Azetukalner Dizziness 4% 25% Headache 6% 19% Somnolence 3% 17% Fall 4% 15% Memory impairment <1% 11% Weight gain - 11% Gait disturbance - 10% Seizure/change in seizure 4% 10% Fatigue 4% 9% N
Slide 39: Opakalim Favorable Tolerability vs. Approved and Investigational ASMs
Opakalim Demonstrates Favorable Tolerability vs. Approved and Investigational ASMs Kv7 30% Percent of Subjects Reporting Adverse Event 26% Opakalim Azetukalner Cenobamate Lacosamide Levetiracetam 25% 25% 25% 25% 25% 23% 24% 24% 23% 23% 20% 19% 17% 15% 13% 13% 12% 10% 11% 11% 10% 9% 6% 5% 5% 4% 3% 3%
Slide 40: 50% Responder Rate in OLE Trials of ASMs
50% Responder Rate in OLE Trials of Several Approved and Investigational ASMs Kv7 80% 72% 70% 63% 60% 60% 56% 56% 54% 53% 50% 53% 48% 44% 40% 37% 33% 34% 30% 20% Opakalim Azetukalner Brivaracetam Cenobamate Eslicarbazepine Lacosamide Perampanel Opakalim ongoing focal epilepsy open-label preliminary
Slide 41: Opakalim Is Easy-to-Use With Projected Favorable Tolerability
Opakalim Is Easy-to-Use With a Projected Favorable Tolerability Compared to Approved ASMs Kv7 Low Favorable CNS Low metabolic / Low SJS or No titration neuropsychiatric tolerability electrolyte AEs DRESS risk AEs Lamotrigine ✘✘ ✓ ✓ ✓ ✘✘ ✓ ✓ ✘ ✓ L1 Levetiracetam ~ Oxcarbazepine ✘ ✘ ✓ ✘ ✘ Lacosamide ✘
Slide 42: Targeting High-Quality Weight Loss in Obesity
TALDEFGROBEP / MYOSTATIN MYOSTATIN ACTIVIN PATHWAY INHIBITOR: COMPLEX TALDEFGROBEP ALFA Targeting High-Quality Weight Loss in Obesity
Slide 43: Targeting High-Quality Weight Loss With Myostatin-Activin Inhibition
Targeting High-Quality Weight Loss With Myostatin-Activin Inhibition TALDEFGROBEP Taldefgrobep directly targets fat and muscle while avoiding intolerable adverse effects Novel myostatin-activin MOA for healthy weight loss Inhibits ActRII signaling in muscle and adipose tissue FAT Favorable safety pr
Slide 44: Taldefgrobep Is a Novel Competitive Inhibitor of ActRII Signaling
Taldefgrobep Is a Novel Competitive Inhibitor of ActRII Signaling TALDEFGROBEP ActRII LIGANDS NON-PRODUCTIVE TALDEFGROBEP/ MYOSTATIN COMPLEX REGULATING FAT AND MUSCLE METABOLISM RECEPTOR BINDING blocks both free of the Taldefgrobep/Myostatin myostatin AND activin complex to ActRII receptor signaling
Slide 45: Unmet Needs Remain in Obesity Management Despite New Therapies
Unmet Needs Remain in Obesity Management Despite New Therapies TALDEFGROBEP Unprecedented reductions in Opportunity in obesity total body weight are limited by: management: Significant muscle loss Increase muscle mass and bone Up to 40% of GLP-1-induced weight density while reducing fat mass loss is
Slide 46: Myostatin Activin Inhibitor Class Optimal Profile
Clinical Data Has Validated Myostatin Activin Inhibitor Class Uncovering Optimal Profile for Efficacy and Safety in Obesity TALDEFGROBEP MOA Directly Targeting Potential Fat Weight Favorable SC Monthly Adipose Skeletal Reduction Loss Safety Profile Dosing Tissue Muscle Taldefgrobep (Biohaven) Bimagr
Slide 47: Myostatin Activin Inhibition Benefits Weight, Fat and Muscle
Myostatin Activin Pathway Inhibition Demonstrates Benefits in Total Body Weight, Fat and Muscle TALDEFGROBEP BELIEVE: 1-Year Trial of Bimagrumab in Adults Living with Overweight or Obesity Bimagrumab Semaglutide Combination Placebo (30 mg iv) (2.4 mg sc) (bima + sema) EFFICACY Total body weight (W48
Slide 48: Bimagrumab Efficacy Limited by Safety/Tolerability
Bimagrumab Provides Reason To “Believe” With Compelling Efficacy but Limited by Safety/Tolerability TALDEFGROBEP Placebo Bimagrumab (30 mg IV) Semaglutide (2.4 mg SC) Muscle Spasms 5.5% 73.7% 8.9% Diarrhea 5.5% 49.1% 35.7% Poor tolerability due to Acne 3.6% 43.9% 8.9% irreversible ActRIIB binding Pe
Slide 49: Taldefgrobep Avoids GI- and Muscle-Related AEs
Taldefgrobep Avoids GI- and Muscle-Related AEs Commonly Reported in Bimagrumab Clinical Trials TALDEFGROBEP Taldefgrobep Bimagrumab Bimagrumab SAD/MAD Pooled1 30 mg/kg2 10 mg/kg3 Q4W 15-180 mg Single Dose Study Multi-dose Study Muscle-/GI-Related AEs n=103 n=10 n=37 Acne 0% 30% 3% Muscle spasm 3% 30
Slide 50: Taldefgrobep/Myostatin Complex Competes With Activin Signaling
Taldefgrobep/Myostatin Complex Competes With Activin Signaling To Improve Muscle Differentiation and Fat Storage TALDEFGROBEP T/M Complex Inhibits Activin-Induced Signaling Through ActRII no taldefgrobep with taldefgrobep Activin A Activin E Normalized pSMAD2/3 (as a % of ligand only) Normalized pSM
Slide 51: Obese Mouse Model: Fat Mass Reduction, Lean Mass Increase
In an Obese Mouse Model, Taldefgrobep Demonstrated Significant Reductions in Fat Mass While Increasing Lean Mass TALDEFGROBEP Change in Total Body Weight, Change in Fat Mass Change in Lean Muscle Mass From Baseline at 8 Weeks From Baseline at 8 Weeks From Baseline at 8 Weeks 8 **** *** ** **** Chang
Slide 52: Taldefgrobep Improves Body Composition With Potential Monthly Dosing
Taldefgrobep Improves Body Composition in Non-Obese Adults With Potential for Monthly Dosing in Obesity TALDEFGROBEP Peak Plasma Concentrations of In Healthy Adults at One Month Post Dosing, Taldefgrobep Demonstrates: Taldefgrobep-Myostatin Complex Occurs 20-30 Days Post Dose Fat Body Mass Lean Body
Slide 53: Taldefgrobep Has an Established, Favorable Safety Profile
Taldefgrobep Has an Established, Favorable Safety Profile TALDEFGROBEP Safety database includes more LOW RATES of SAEs and AEs than 700 treated trial participants leading to discontinuation LOW RATES of GI- and muscle- Assessed across a wide dose related AEs commonly reported range (4 mg to 180 mg S
Slide 54: Ongoing Phase 2 Monotherapy Dose-Ranging Study
Ongoing Phase 2 Monotherapy Dose-Ranging (Q1W + Q4W) Study TALDEFGROBEP INTERIM PRIMARY ANALYSIS ANALYSIS Randomization (2:2:1:1) 24-Week DBT Period Taldefgrobep 100 mg Q1W (n=50) 24-Week OLE 4 Weeks Taldefgrobep Q1W 8 Weeks Placebo Q1W (n=25) Randomization Post-Dose (n~150) Follow-Up Taldefgrobep 1
Slide 55: Blinded Interim Data: No Adverse Effects on Lipids
Blinded Interim Data by Q1W vs. Q4W, Taldefgrobep + Placebo No Adverse Effects on LIPIDS at Week 12 TALDEFGROBEP Total Cholesterol LDL Cholesterol Triglycerides Q1W n=27 Q1W n=27 Q1W n=27 215 135 130 Q4W n=30 Q4W n=30 Q4W n=30 210 125 130 206.87 120 205 205.89 126.3 126.04 115.4 125 116.57 115 113.5
Slide 56: Interim PK/PD Data Supports Weekly and Monthly Dosing
Interim Phase 2 PK/PD Data Supports Weekly and Monthly Dosing in Obesity TALDEFGROBEP % Myostatin Suppression at Week 12 Taldefgrobep-Myostatin Complex at AT WEEK 12: in Overweight/Obese Adults Week 12 in Overweight/Obese Adults 100 • Observed mean taldefgrobep concentrations are consistent 99% % My
Slide 57: Taldefgrobep Offers a Novel Approach for People Living With Obesity
Taldefgrobep Offers a Novel Approach To Address the Needs for People Living With Obesity TALDEFGROBEP Total body weight Fat mass loss loss meeting current comparable to GLP-1 regulatory standards therapies Benefit as monotherapy Visceral adipose and in combination tissue loss favorable with GLP-1 th
Slide 58: Taldefgrobep Can Benefit a Broad Spectrum of People Living With Obesity
Taldefgrobep Can Benefit a Broad Spectrum of People Living With Obesity TALDEFGROBEP 6.6M–10M 5–8M 5–7M 5–10M Older patients at risk of People intolerant or People living with People unable to tolerate sarcopenia refractory to GLP-1 BMI ≥40 high dose GLP-1 MONO THER APY GL P-1 ADJUN CT Source: BHVN
Slide 59: Modular Next-Generation ADC Technologies
ONCOLOGY Modular Next-Generation ADC Technologies: Optimizing for Clinical Performance
Slide 60: Innovative Technologies Enabling Differentiated Next-Generation ADCs
Innovative Biohaven Technologies Enabling Differentiated Next-Generation ADCs ONCOLOGY OPTIMIZED Clinical Proof-of-Principle Establishing MONO AND BISPECIFIC the Power and Flexibility of the Platform to MABS Optimize Across Broad Range of ADC Designs and Combination Strategies Including CPI First cl
Slide 61: BHV-1510 + Cemiplimab Rapid, Deep and Durable Responses
BHV-1510 + Cemiplimab Leads to Rapid, Deep and Durable Responses in Heavily Pretreated Patients, Majority With Prior Anti-PD(L)1 ONCOLOGY BHV-1510 + Cemiplimab*: BHV-1510 + Cemiplimab: Treatment Duration Percent Change in Target Lesions NSCLC NSCLC EC NSCLC NSCLC NSCLC NSCLC NSCLC NSCLC NSCLC NSCLC
Slide 62: BHV-1510 Favorable PK Profile With Highly Stable ADC
BHV-1510 Demonstrates a Favorable PK Profile With Highly Stable ADC ONCOLOGY BHV-1510 (ADC) and Payload Concentration-Time Plots for Cycle 1 (with or without Cemiplimab) MONO COMBO 1000000 Mean (SD) concentration (ng/mL) ANALYTE BHV-1510 10000 payload 100 DOSE 2 mg/kg Q3W 1 2.5 mg/kg Q3W 2.75 mg/kg
Slide 63: Early Clinical Activity of BHV-1510 + Cemiplimab
Encouraging Early Clinical Activity and High Response Rates of BHV-1510 + Cemiplimab Enable First Tumor-Specific Expansions ONCOLOGY • Dose escalation complete: Compelling efficacy BHV-1510 2.5 mg/kg + Cemiplimab Q3W: in difficult-to-treat patients Target Lesion Change From Baseline • Responses in h
Slide 64: BHV-1530: Favorable Early Clinical Profile
BHV-1530: Favorable Early Clinical Profile — No FGFR-Class Toxicities and Early Signs of Antitumor Activity ONCOLOGY FGFR3 Overexpression and Alteration (%) Compelling preclinical efficacy across FGFR3-altered in Urothelial Carcinoma and FGFR3-overexpressing tumor models: Demonstrated as monotherapy
Slide 65: BHV-1530: Phase 1 Study in Advanced Tumors
BHV-1530: Phase 1 Study in Advanced Tumors ONCOLOGY KEY ELIGIBILITY PHASE 1 Higher dose Select advanced refractory tumors Dose escalation following Dose RD (FGFR3 expression level BOIN design Level not required for study entry) Higher dose Study Design: Open label, dose escalation (Ph1) KEY STUDY Po
Slide 66: Case Narrative: Metastatic Urothelial Cancer
Case Narrative: Metastatic Urothelial Cancer ONCOLOGY Baseline Week 12 • 28 y/o female with urothelial cancer with a FGFR3::TACC3 fusion, with metastasis to lungs and thoracic lymph node • 4 prior lines of therapy including • Padcev and Keytruda and 2 FGFR Baseline Week 12 targeting small molecules-
Slide 67: BHV-1530: Potential First FGFR3 ADC
BHV-1530: Potential First FGFR3 ADC — Broader Reach, DEVELOPMENT ROADMAP No Class Toxicities, Clear Development Path BHV-1530 ADC vs. Small-Molecule FGFR3 Inhibitor PH1 DOSE ESCALATION NOW • ACTIVE • No DLTs; no FGFR-class toxicities • Early tumor reduction in altered + WT overexpressing Small Molec
Slide 68: Leveraging Validated Immune Targets To Stop Neuroinflammation
BRAIN-PENETRANT TYK2/JAK1 INHIBITOR: BHV-8000 BHV-8000 Leveraging Validated Immune Targets To Stop Neuroinflammation
Slide 69: Brain-Penetrant TYK2/JAK1 Inhibitor To Treat Parkinson's Disease
Brain-Penetrant TYK2/JAK1 Inhibitor To Treat Parkinson’s Disease TYK2/JAK1 BHV-8000 addresses the widespread immune dysregulation that drives the onset and progression of neurodegenerative disorders Suppresses innate and adaptive immune cell activation and infiltration High selectivity for TYK2/JAK1
Slide 70: Widespread Immune Dysregulation Is a Hallmark of PD
Widespread Immune Dysregulation Is a Hallmark of PD TYK2/JAK1 PD meets criteria for PD animal models demonstrate autoimmunity based on immune dysregulation drives pathophysiology and genetics neurodegeneration 6 • Misidentification of self-proteins (⍺-synuclein) • In mouse models, T cells specific t
Slide 71: BHV-8000 Improves Function in AAV-α-syn Mouse Model
BHV-8000 Reduces Glial Activation, Increases Neuronal Survival, and Improves Function in AAV-⍺-syn Mouse Model of Parkinson’s Disease TYK2/JAK1 AAV-⍺-SYN BHV-8000 dosing Behavioral injection 10 and 30 mg/kg BID assessments Immunostaining AAV-α-synuclein Mouse Model DAY -7 AC C LIMATIO N 1 3 42 45 Re
Slide 72: BHV-8000 Target Engagement, Brain Penetration and Safety in Phase 1
BHV-8000 Demonstrates Target Engagement, Robust Brain Penetration and a Promising Safety Profile in Phase 1 Studies TYK2/JAK1 Phase 1 program completed BHV-8000 Brain Exposures at 20 mg QD • Includes SAD/MAD study in healthy adults Well-tolerated • No SAEs or severe AEs • No adverse laboratory trend
Slide 73: BHV-8000: Phase 2/3 Study in Early Parkinson's Disease Ongoing
BHV- 000: Phase 2/3 Study in Early Parkinson’s Disease Ongoing TYK2/JAK1 48-Week Time-to-event 56-Days BHV-8000 20 mg PO Once Daily 48-Weeks Screening BHV-8000 10 mg PO Once Daily Extension Study Placebo Study Design: Phase 2/3, randomized 1:1:1, double-blind study KEY STUDY Population: Early untrea
Slide 74: Targeting Brain Hypometabolism to Treat Neurodegenerative Diseases
BRAIN-PENETRANT PKM2 ACTIVATOR: BHV-8100 Targeting Brain Hypometabolism to Treat Neurodegenerative Diseases
Slide 75: The Brain Has Considerable Energetic Requirements
The Brain Has Considerable Energetic Requirements DISCOVERY 15% of cardiac output 2% of body 25% of whole-body YET glucose mass utilization 20% of oxygen consumption 75 August 2026 Biohaven Corporate Presentation
Slide 76: Aerobic Glycolysis Is Reduced in the Aging Brain
Aerobic Glycolysis Is Reduced in the Aging Brain DISCOVERY • Aerobic glycolysis, the metabolic process of glucose primarily promoted by astrocytes in the brain, is reduced in the aging brain1 • Reduction of aerobic glycolysis is pronounced in frontal and temporal regions 2 Healthy Adults Alzheimer's
Slide 77: Activation of Pyruvate Kinase Increases Glucose Consumption and ATP Production
Activation of Pyruvate Kinase Increases Glucose Consumption and ATP Production While Reducing Deleterious Metabolism DISCOVERY Less Active State More Active State GLUCOSE Immune Activation Increased Glycolysis Biomass Production Increased biosynthesis Increased Energy Fibrosis of cellular building P
Slide 78: BHV-8100: First Brain-Penetrant PKM2 Activator
BHV-8100: First Brain-Penetrant PKM2 Activator DISCOVERY Oral small molecule medicine with multiple potential indications Retinal degeneration Neurodegeneration and inflammation Age-related changes: Neuroinflammation central/peripheral Peripheral Brain ischemia inflammation KEY First-in-human dosing
Slide 79: BHV-8100: Mitigation of Neuroinflammation and Restoration of Energetics
BHV-8100: Experimental Mitigation of Neuroinflammation and Restoration of Energetic Deficiency DISCOVERY BHV-8100 Shows Robust Reduction BHV-8100 Demonstrates Striking Efficacy of Disease Burden in EAE in Cuprizone Model Clinical Score (EAE Therapeutic Model) Distance Traveled Myelin-Basic Protein i
Slide 80: BHV-8100 Sustained Efficacy in Human Brains With Alzheimer's
BHV-8100 Demonstrates Sustained Efficacy in Human Brains With Documented Alzheimer’s Disease and All-cause Dementia DISCOVERY Reperfused human brains (Brainex ) allow precise study of brain penetrance, pharmacology, pharmacokinetics, pharmacodynamics and biomarkers in brains with documented diseases
Slide 81: Early FIH Data of BHV-8100 Shows Safety and Tolerability
Early FIH Data of BHV-8100 in SAD Shows Safety and Tolerability in Healthy Participants DISCOVERY No SAEs or severe AEs Most AEs mild and spontaneously resolving No clinically significant ECG changes No clinically significant trends in safety labs including LFTs PK allows QD dosing & achieves projec
Slide 82: Financial Update
Financial Update CGRP 151.0M 2 $270.5M 1 1. Includes cash, cash equivalents and marketable securities as of June 30, 2026; 2. As of June 30, 2026. 3. Cap reached if aggregate annual US net sales of rimegepant and zavegepant amount to $8.15B. Royalty payments would be in respect of years ended on or
Slide 83: Key Milestones Anticipated in 2026
Key Milestones Anticipated in 2026 1H 2026 2H 2026 Gd-IgA1 Degrader | BHV-1400 IgA Nephropathy Initiate Pivotal IgAN INFLAMMATION & Initiated Pivotal Graves’ IgG Degrader | BHV-1300 Common Disease (Graves’, RA) IMMUNOLOGY TYK2/JAK1 Inhibitor | Parkinson’s Disease Ongoing Phase 2/3 Trial BHV-8000 (br
Slide 84: “Days
“Days .M ATT E R™ 7 . biohaven ye ES