BerGenBio

Investor Presentation • May 23, 2017

BerGenBio

Developing first-in-class drugs to treat aggressive cancer

First Quarter 2017 presentation May 23rd 2017

Disclaimer

Certain statements contained in this presentation constitute forward-looking statements. Forward-looking statements are statements that are not historical facts and they can be identified by the use of forward-looking terminology, including the words "anticipate", "believe", "intend", "estimate", "expect", "will", "may", "should" and words of similar meaning. By their nature, forward-looking statements involve a number of risks, uncertainties and assumptions that could cause actual results or events to differ materially from those expressed or implied by the forward-looking statements. Accordingly, no assurance is given that such forward-looking statements will prove to have been correct. They speak only as at the date of the presentation and no representation or warranty, expressed or implied, is made by BerGenBio ASA or its affiliates ("BerGenBio"), or by any of their respective members, directors, officers or employees that any of these forward-looking statements or forecasts will come to pass or that any forecast result will be achieved and you are cautioned not to place any undue influence on any forward-looking statement. BerGenBio is making no representation or warranty, expressed or implied, as to the accuracy, reliability or completeness of this presentation, and neither BerGenBio nor any of its directors, officers or employees will have any liability to you or any other person resulting from the use of this presentation.

Copyright of all published material, including photographs, drawings and images in this presentation remain with BerGenBio and relevant third parties, as appropriate. Consequently, no reproduction in any form of the presentation, or parts thereof, is permitted without the prior written permission, and only with appropriate acknowledgements.

Q1 Achievements

R&D programs are progressing to plan - solid foundation to build value

	Phase II clinical development program opened and enrolling Â
	Lung cancer study in combination study with erlotinib opened first and second line cohorts. Â
	Lung cancer study in combination with docetaxel opened & dosed first patients Â
BGB324	Melanoma study in combination with targeted & I-O therapies opened and dosed first patients Â
	Collaborative agreement with Merck & Co (MSD) Â
	Phase II combination trials (2) with MSD's immune checkpoint inhibitor KEYTRUDA® Â (pembrolizumab) in patients with advanced lung and triple negative breast cancer
	Clinical candidate BGB149 was nominated, a humanized anti-Axl monoclonal antibody Â
Pipeline	Cell line development and manufacturing of the antibody is underway with a leading biologics  contract manufacturer.
AACR*	Two presentations at AACR 2017: 1) Randomized Phase II Melanoma study 2) BGB324 blocks  resistance to check point inhibitors
	IPO
	Â Closed 7 April, gross proceeds of NOK 400 million
	Â Ticker : ' BGBIO '
Corporate	 Cash of NOK 95.4 million at end of Q1 2017 (excludes proceeds from IPO)
	Â Stein H. Annexstad was elected Chair of the Board
	Â Registered wholly owned subsidiary BerGenBio Limited, to facilitate UK organization Â

BerGenBio – First-in-class Axl inhibitors for multiple aggressive cancers

90% of cancer deaths result from tumors spreading, becoming immune evasive and drug resistant

Axl is a key mediator of these traits in a broad range of cancers

BerGenBio is a world-leader in Axl biology and is developing an exciting pipeline of Axl inhibitors

BGB324 initially addressing an annual market potential of USD 11 Billion

BGB324 – First-in-class, highly selective oral Axl inhibitor

Investigational Medicinal Product

• Â 100mg capsules, standard pharmaceutical formulation
• Â 3yr shelf life
• Â Low 'cost of goods' (COGs)
• Â Patients take medicines home, one-a-day dose

§ Drug substance

• Â Licensed from Rigel Inc. 2011
• Â Highly selective and potent
• Â Orally bioavailable
• Â Orphan status in US for AML
• Â Well tolerated: suitable for long term therapy
• Â Wide therapeutic index: suitable for combination with existing drugs

Mode of Action

Strategic pipeline will drive value creation

	Discovery	Preclinical	Phase I	Phase II	Phase III
BGB324 – Axl kinase inhibitor
AML / MDS	Phase Ib / II –	Single agent / Combination
NSCLC (mutation driven)	Phase Ib / II –	Combination with TARCEVA® (erlotinib)
NSCLC (adenocarcinoma)		Phase II Combination with KEYTRUDA® (pembrolizumab)
TNBC		Phase II Combination with KEYTRUDA® (pembrolizumab)
Investigator-sponsored trials
NSCLC		Phase II BGB324 in combination with Docetaxel			6
Melanoma			Phase II BGB324 in combination with current standard therapies, incl. CPIs
Antibody programs
BGB149 – Oncology	Anti-Axl mAb
BGB601 – Metastatic cancer (Partnered)	ADC
Discovery Pipeline –	small molecule inhibitors
BGB002 – Oncology
BGB003 – Oncology

Compelling Phase Ib clinical data for BGB324

BGB324 has generated strong efficacy data in patients with no other existing treatment options

Q1 Status of BGB324 clinical trials

BerGenBio sponsored clinical trials

BGB324 - Blockbuster potential – addressable market ~\$11bn in selected indications

1) SEER Program – National Cancer Institute (National Institute of Health) http://seer.cancer.gov/; 3) Cancer.net; 4) Figure for male and female breast cancer; 5) Excluding rectum; 6) Estimates by Alacrita Consulting

Annual Market Potential for BGB324 in Leukemia

Target patient population			Target patient population (prevalence)
	USA (2017)1		3,848
	Axl +ve (45%)		1,731
	Cost		of comparable drugs (US cost/pt/yr)2
AML relapsed/refractory Â	Gleevec (CML)	(Imatinib)	\$230k
Age 60 or older Â	Iclusig (CML)		\$224.5k	Total market	\$635m
Actively receiving treatment  pAxl + Â	Sprycel (CML)		\$165.4k	potential
	Prevalence in other Major Markets1		Average price compared to USA3
	Japan	1,546	50%
	EU5	2,998	50%
	Total Axl +ve (45%)	2,045

¹ Datamonitor Healthcare ("DMHC AML treatment datapack_12.19.2016.xls") Prevalent R/R AML patients over 60 years of age that are actively receiving treatment.; 2 PriceRx September 2016; assumes patient treated for 12 months; 3 "Biotech Forecasting & Valuation" David FS et al (2016)

Annual Market Potential for BGB324 in Myeloid Dysplastic Syndrome

Patient population4	Patient population (prevalence)
	USA (2011)1		60,000
	High risk (23%)2		13,800
	Axl +ve (45%)		6,210
	Cost	of comparable drugs (US cost/pt/yr)3
High risk MDS Â	Revlimid (MDS)		(USA) \$240k	Total market	\$2.25bn
pAxl + Â	Prevalence in other Major Markets1		Average price compared to USA6	potential
	Japan	nd5	50%
	EU5	60,000	50%
	Total high risk Axl +ve (45%)	6,210

¹ Mikkael, 2011; 2 IWG 2012 data, www.dacogen.com/MDS-Incidence-and-Prevalence.aspx; 3 PriceRx September 2016; assumes patient treated for 12 months; 4 Proportion of patients that are second line, decitabine failures is not available; 5 Prevalence not easily available in Japan; 6 "Biotech Forecasting & Valuation" David FS et al (2016)

Annual Market Potential for BGB324 in Lung Cancer in combination with erlotinib

Target patient population			Target patient population (prevalence)1
	USA (2017)		13,883
	Cost		of comparable drugs (US cost/pt/yr)2
NSCLC, non-squamous Â	Tarceva (NSCLC EGFR+)		\$106k
EGFR+ patients Â	Tagrisso (NSCLC, T790M)		\$200k	Total
Stage III and IV and distant  relapse First line or maintenance Â	Gilotrif 19 deletion or exon 20 L858R)	(NSCLC, EGFR exon	\$110k	market potential	\$4.7bn
therapy	Prevalence in other Major Markets1		Average price compared to USA4
	Japan	8,234	50%
	EU5	12,223	50%

¹ Datamonitor Healthcare ("Non-Small Cell Lung Cancer Epidemiology Forecast.xls"): Prevalent patient population, EGFR+ NSCLC, Stage III and IV, pharmacologically treated with first line or maintenance therapy; 2 PriceRx September 2016; assumes patient treated for 12 months; 3 "Biotech Forecasting & Valuation" David FS et al (2016); 4 "Biotech Forecasting & Valuation" David FS et al (2016)

Annual Market Potential for BGB324 in Lung Cancer in combination with KEYTRUDA

Target patient population			Target patient population (prevalence)
	USA (2017)1		11,065
	PD-L1+ (30%)3		3,320
	Cost		of comparable drugs (US cost/pt/yr)2
NSCLC Â Stage IV + distant relapse Â	Opdivo (NSCLC)		\$185k
First line Â	Keytruda (NSCLC)		\$136.4k	Total market	\$1.6bn
Not EGFR+ Â	Crizoinib (ALK+)		\$115k	potential
Not ALK+ Â PD-L1 >50% Â	Prevalence in other Major Markets1		Average price compared to USA4
	Japan	10,792	50%
	EU5	25,024	50%
	PDL1+ve	10,745

¹ Datamonitor Healthcare ("Non-Small Cell Lung Cancer Epidemiology Forecast.xls"): Prevalent patients, with Stage IV receiving first line pharmacologic therapy, not EGFR+ or ALK+; 2 PriceRx September 2016; assumes patient treated for 12 months; 3 https://www.keytruda.com/hcp/nsclc/efficacy-first-line-treatment/; 4 "Biotech Forecasting & Valuation" David FS et al (2016)

Annual Market Potential for BGB324 in Breast Cancer in combination with KEYTRUDA

Target patient population			Target patient population (prevalence)
	USA (2017)1		8,852
	Axl+ve	(50%)3	4,426
	Cost		of comparable drugs (US cost/pt/yr)2
TNBC Stage IV patients & Â	Herceptin (Her2+ breast)		\$253k	Total
distant relapse	Tykerb (Her2+ breast)		\$93k	market	\$2bn
receiving second line therapy  and beyond	Afinitor	(advanced HR+ breast)	\$188k	potential
pAxl+ 50%3 Â	Prevalence in other Major Markets1		Average price compared to USA4
	Japan	2,283	50%
	EU5	15,000	50%
	Axl+ve	8,640

¹ Datamonitor Healthcare ("Breast Cancer Epidemiology Forecast.xls"): Prevalent Stage IV TNBC patients receiving second line, third line or fourth line therapy; 2 PriceRx September 2016; 3 Breast Cancer (2016) 2, 16033 ("Axl-associated tumor inflammation as a poor prognostic signature in chemotherapy-treated triple-negative breast cancer patients"); 4 "Biotech Forecasting & Valuation" David FS et al (2016)

Strong rationale for combining BGB324 with checkpoint inhibitors

Source: Chouaib, 2014; Hugo, 2016

Phase II studies in combination with KEYTRUDATM

Collaboration with Merck & Co. (MSD)

• Â Clinical collaboration to evaluate BGB324 in combination with Merck's checkpoint inhibitor KEYTRUDA
• Â Axl's role in suppressing immune response provides strong rationale for evaluating BGB324 with KEYTRUDA – complementary modes of action could provide clinical synergies
• Â BGB324 will prevent EMT and allow CTLs to engage with aggressive mesenchymal cancer cells. By blocking the Axl signal the cancer cells will not be able to limit the immune infiltrates or function in the tumor micro environment
• Â BerGenBio is sponsoring two Phase II clinical trials (see below)

KEYTRUDATM (pembrolizumab)

KEYTRUDA is a therapeutic antibody that increases the ability of the body's immune system to detect and destroy tumor cells.

KEYTRUDA blocks the drug target PD-1 thereby activates T lymphocytes (CTLs)

KEYTRUDA is approved in the US for the treatment of:

• first-line treatment of metastatic NSCLC high PD-L1 expression
• metastatic NSCLC where the tumors express PD-L1
• unresectable or metastatic melanoma
• recurrent or metastatic head and neck squamous cell carcinoma
• Hodgkin's lymphoma

Sales of KEYTRUDA were USD 1.4bn in 2016

BGB324/KEYTRUDA Combination Trials – planned to start in 1H 2017

• NSCLC – Phase II multi-centre study in patients with previously treated unresectable adenocarcinoma of the lung
• TNBC – Phase II multi-centre study in patients with previously treated, locally advanced TNBC.
• Biomarker studies will be conducted in parallel to support the development of companion diagnostics to identify patients most suitable for treatment with a combination of BGB324 and KEYTRUDA, ie patients that are Axl + and PD-L1 +

PD-L1 and PD-L2 Block T Cells from Attacking Cancer Cells

PD-1 inhibition with Keytruda reactivates T cells to attack and kill cancer cells

• Â PD-1 is an antigen expressed on the surface of activated T-cells
• Â PD-1 interacts with its ligands PD-L1 and PD-L2 expressed on cancer and surrounding cells
• Â This inhibits activation of T lymphocytes and prevents an anti-tumor immune response

Normal immune response When functioning properly, T cells are activated and can attack tumor cells.

Tumor evasion and T-cell deactivation Some tumors can evade the immune system through the PD-1 pathway. The PD-L1 and PD-L2 ligands on tumors can bind with PD-1 receptors on T cells to inactivate the T cells

T-cell reactivation with KEYTRUDA KEYTRUDA binds to the PD-1 receptor and blocks its interaction with PD-L1 and PD-L2, which helps restore the immune response. While having an effect on the tumor, this could also affect normal, healthy cells

Real-world Randomised Phase II in Melanoma Investigator Led Studies: additional value drivers

§ Study objectives

§ Assess the safety and efficacy of BGB324 given together with standard treatment, pembrolizumab or dabrafenib and trametinib, compared to standard treatment alone

Primary outcome :

• § Objective Response Rate
• § Number of participants with treatment-related adverse events Secondary outcome :
• § Progression Free Survival
• § Duration of response
• § Overall Survival

§ Design

Experimental:

• § Arm 1:
• § BGB324 + pembrolizumab (first line)
• § Arm 2:
• § BGB324 + dabrafenib and trametinib (first line)

Active Comparators:

• § Pembrolizumab
• § Dabrafenib and trametinib

§ Participants and collaborators

• § Comprehensive programme of explorative biomarker analyses to complement the clinical assessments
• § Norwegian clinical investigators
• § Massachusetts Institute of Technology (MIT)
• § Harvard Medical School
• § NOK17 million grant
• § awarded by the Norwegian Health Authorities
• § recognition of the high degree of innovation, excellent clinical rationale and high scientific value

§ Significant AACR coverage

• § Poster presented at AACR in April by the Principal Investigator Dr. Oddbjørn Straume, consultant oncologist at Haukeland University Hospital and Professor at the University of Bergen Center for Cancer Biomarkers
• § Attracting significant attention by leading experts in melanoma treatment. Also covered on AACR – TV with an interview and presentation.

Phase II Melanoma Trial Real-world study with BGB324 in a randomized controlled design

Companion diagnostics reduce risk, add significant clinical and regulatory advantage

trial

• Â Companion diagnostics are used to select patients that are expected to benefit from a particular drug
• o Significantly increases the likelihood of a positive response
• Â Allows for smaller and faster clinical trials
• o Significant value added to NPV calculations
• Â Targeted therapies with patient selection diagnostic more likely to achieve a premium price

Potential benefits from a successful companion diagnostic

	Accelerated	Patients in pivotal trial	Patients in safety assessment	Program duration (years)
Increased likelihood of	Nivolumab (Hodgkin's Disease)	95	263	4
accelerated	Venetoclax (CLL with 17p deletion)	106	240	5
approval With	Alectinib (ALK + NSCLC)	225	253	4
enriched	Traditional
smaller trials	Cabozantinib (RCC)	658	331	11
	Elotuzumab (multiple myeloma)	646	318	7
	Ramucirumab (gastric cancer)	355	568	8

Parallel development of companion diagnostic A high value product in its own

H-score validation during phase II

Prototype Companion Diagnostic Predictive biomarker allows selection of patients that respond

• Â Activated Axl (pAxl) down after 21 days
• Â Less activated downstream signalling proteins

• Â pAxl positive patients show objective clinical response, as determined by pre-treatment screen of bone marrow

• Â Activated Axl (pAxl) absent pre-drug

• Â More activated downstream signalling proteins

Summary of our Clinical development plan to deliver Phase II data in high-value indications

	2017	2018
		Phase II	Phase II Clinical Data
	Ongoing	AML and MDS – Single agent / Combination	P
BGB324	Ongoing	P
		NSCLC (adenocarcinoma) – Combination with KEYTRUDA	P
		TNBC – Combination with KEYTRUDA	P
Companion diagnostic	Ongoing	Method validation validation and collection of reference data set	Validated CDx P
BGB149		First-in-man trials Manufacture and IND enabling work	Phase I Clinical Data P

BGB324 – Set to become a highly attractive and valuable asset

Commercializing strategy for BGB324

• Â Maintain clinical development and progression into registration trials
• Â Clear commercialization route for smaller indications in certain regions
• Â First-in-class drug with broad clinical application potentially triggering interest from bigger pharma

Commercial planning in parallel with registration trails

Game changing technology Early stage Traditional mode of Action Me2 Me better Compelling Phase II data AA/BT/PRIME application Game changing Compelling clinical development plan First-in-class Competitor space > SOC > Phase III NDA ready Clear positioning Reimbursement Competitor free Game changing > Phase III NDA ready Clear positioning Reimbursement Competitor free AA/BT/PRIME granted 2017 2018 2019 2020 2021 Conventional technology Time Closeness to revenue Low Medium High

Late stage, first-in-class assets will be highly sought after

Significant corporate development activities in Q1

IPO and listing on OSE

• ÂCompleted 7 April 2017, ticker BGBIO
• ÂRaise of NOK 400 million in gross proceeds
• ÂNew and existing investors participated (approximately 2,000 shareholders)

Board of Directors strengthened

• ÂStein Holst Annexstad appointed as Non-executive Chair
• § Senior industry experience at executive and board levels, including former executive of Dyno Industrier AS, CEO of Nycomed AS (subsequently merged with Amersham Plc and thereafter merged with GE), and Chairman of Algeta ASA

UK operations established

• ÂBerGenBio Ltd established in Oxford, UK
• § To facilitate efficient management of UK based staff and facilities

Key financials Q1 2017

(NOK million)	Q1 2017	Q1 2016	FY 2016
Operating revenues	-	-	-
Operating expenses	65.8	20.7	131.6
Operating profit (loss)	(65.8)	(20.7)	(131.6)
Profit (loss) after tax	(65.1)	(20.3)	(129.8)
Basic and diluted earnings (loss) per share (NOK)	(1.93)	(75.21)	(419.68)
Cash position end of period	95.4	163.2	161.8

Cash flow

Operating loss Cash position

• Â Operating expenses in Q1 2017 impacted by NOK 27.8 million (USD 3.3 million) Phase II milestone payment to Rigel Pharmaceuticals Inc.
• Â Net proceeds from the IPO approximately NOK 375 million received in April
• Â NOK 15.7 million grant awarded from the Research Council of Norway to support investigator-led studies terms being negotiated

Shareholder base (post IPO)

SHAREHOLDER	# SHARES	%
METEVA AS	14,923,000	30.00
INVESTINOR AS	6,609,800	13.29
SARSIA SEED AS	2,117,900	4.26
VERDIPAPIRFONDET ALFRED BERG GAMBA	1,852,500	3.72
MP PENSJON PK	1,780,300	3.58
NORSK INNOVASJONSKAPITAL II AS	1,273,100	2.56
JPMORGAN CHASE BANK, N.A., LONDON	1,272,000	2.56
DATUM INVEST AS	1,209,200	2.43
SARSIA DEVELOPMENT AS	1,195,000	2.40
BERA AS	1,084,800	2.18
VPF NORDEA AVKASTNING	972,354	1.95
VERDIPAPIRFONDET ALFRED BERG NORGE	845,000	1.70
KLP AKSJENORGE	830,067	1.67
VERDIPAPIRFONDET HANDELSBANKEN	720,000	1.45
VPF NORDEA KAPITAL	700,000	1.41
KOMMUNAL LANDSPENSJONSKASSE	627,188	1.26
VERDIPAPIRFONDET ALFRED BERG AKTIV	552,500	1.11
BIRK VENTURE AS	552,063	1.11
STATOIL PENSJON	440,000	0.88
VERDIPAPIRFONDET NORDEA NORGE PLUS	360,000	0.72

Share facts
NOK
Oslo (NOK)
NO0010650013
BGBIO
Biotechnology
NOK 1.1 bn
49,742,200
1,854

Shareholding by investor type in IPO

Strategic value drivers

• Â First-in-class drugs targeting aggressive cancers
• Â \$11bn addressable market just from ongoing sponsored studies

 BGB324 in multiple phase II studies

• Â Single agent & in combination with current standard of care and checkpoint inhibitors
• Â Demonstrate broad potential of BGB324 in many different cancers
• Â Collaboration with Merck
• Â Companion Diagnostic to enrich future trials, accelerate approval, higher reimbursement
• Â Clear Phase III & registration strategy
• Â Pipeline of drug candidates (in addition to BGB324 )
• Â BGB149, an anti-Axl monoclonal antibody, differentiated from BGB324
• Â Axl ADC drug candidate partnered program with ADC Therapeutics S.a.r.L.
• Â Commercialization: Strategic flexibility retained:
• Â High value, first-in-class drug candidates are attractive targets for partnering and M&A
• Â Go-to market possibilities in enriched patient populations
• Â High visibility with strong news flow and multiple value driving inflection points

Key progress and future milestones

Lung cancer (NSCLC) study with BGB324 in combination with TARCEVA opened (first and second line cohorts)	✔
IPO – NOK 400m to fund BGB324 Phase II clinical program and BGB149 into the clinic	✔
Data presentations as American Association for Cancer Research (AACR)	✔
Investigator-led Phase II trial opened, first NSCLC patients dosed with BGB324 in combination with docetaxel	✔
Investigator-led Phase II trial opened, first melanoma patients dosed with BGB324 in combination with KEYTRUDA or targeted therapy	✔
Phase II – TNBC study with BGB324 in combination with KEYTRUDA	Q2 2017
Phase II – Advanced lung cancer study with BGB324 in combination with KEYTRUDA	Q2 2017
Presentation of interim data from Phase II study of BGB324 in AML/MDS	2H 2017
Presentation of Interim data from Phase II study of BGB324 in EGFR+ NSCLC	2H 2017
Initiation of Phase I for BGB149	2H 2018
Phase II Clinical proof-of-concept data from BGB324 studies
- AML/MDS - single agent/combination
- NSCLC (EGFR+) – combination with erlotinib	2H 2018
- NSCLC (adenocarcinoma) – combination with KEYTRUDA
- TNBC – combination with KEYTRUDA

Summary and outlook

 Multiple Phase II programs with BGB324 are open and recruiting (4/6)

 Strong financial position to advance clinical and pipeline development through high-value inflection points

 Strong news flow drives value and supports high industry profile

 Continued corporate development to strengthen teams and drive the strategy

 Clear strategy to develop and commercialize assets

Thank you.

For further information please visit www.bergenbio.com

Developing first-in-class drugs to treat aggressive cancer

Glossary

AA	Accelerated approval	FDA	US Food and Drug Administration
ADC	Antibody drug conjugate	GLP	Good Laboratory Practice
ALK	Alkaline phosphatase	IHC	Immunohistochemistry
AML	Acute myeloid leukemia	mAb	Monoclonal antibody
BLA	Biologic license application	MDS	Myeloid dysplastic syndrome
BT	Breakthrough therapy	NDA	New drug application
CAB	Clinical advisory board	NSCLC	Non-small cell lung cancer
CBR	Clinical benefit rate	pAxl	Phosphorylated Axl (activated Axl)
CDx	Companion diagnostic	PD	Progressive disease
CLIA	Clinical Laboratory Improvement Amendments	PR	Partial response
CLL	Chronic lymphocytic leukemia	RCC	Renal carcinoma
CPI	Checkpoint inhibitor	RP2D	Recommended Phase II Dose
CR	Complete response	RTK	Receptor tyrosine kinase
CTL	Cytotoxic T-lymphocytes	TAM	Tyro, Axl, Mer (family of kinases)
ECG	Electrocardiogram	TNBC	Triple negative breast cancer
EGFR	Epidermal growth factor receptor	sAxl	Soluble Axl
ELISA	Enzyme-linked immunosorbent assay	SD	Stable disease
EMT	Epithelial-to-mesenchymal transition	SoC	Standard of Care
EU5	France, Germany, Italy, Spain, United Kingdom	QTcF	QT inverval, a measure of time in the heart's electrical cycle