Our vision andthree commitments
November 12, 2021
NIPPON SHINYAKU CO., LTD.

Our vision and three commitments
The name of the company, which means “new drugs from Japan,” reflects thefounder’s desire to produce high-quality medicines with Japanese expertise,and build a company that would develop its business overseas.
The role and mission of Nippon Shinyaku is to help as many people aspossible at the earliest juncture to become a company with a meaningful
existence in the global healthcare field.
VisionThree commitments to accomplish our vision①
Continue tolaunch at least
one distinctive
product each year②③

Generate
at least 50% ofconsolidated sales
from overseas

Target at least
100% gains in
net sales andoperating profit
2For further growth
By succeeding to launch at least one distinctive product each year,
we have proceeded to accomplish increase in both net sales andoperating profit for nine fiscal years in a row.

Net sales
OperatingprofitStock
price

FY2006FY202056,320121,885（million yen）

（million yen）

5,220

26,134（million yen）

（million yen）

994yen8,230yen(as of 2007/3/30）(as of 2021/3/31）

3Commitment ①
Continue to launch at least one
distinctive product each year
Challenge with a sense of urgency

In-house
drug discovery

In-licensing

PLCM

PLCM: Product Life Cycle Management 4

Overview of pharmaceutical markets

Advancementof lowbirthrate
andaging society

Tight
healthcare
finance

Promotionof use ofgeneric
drugs

Increasingpressure of
drug pricereduction

Increaseof
population

Developof
economyIncrease inhealthImprove
healthcare consciousinfrastructure ness

Forecast global market to increase
Forecast domestic market to show
a little to no growth

Pharmaceutical market in Japan

Global pharmaceutical market

It is vital that we develop a stronger presence in overseas markets,
such as US, Europe, China and other areas, if we are to forge sustained
growth in the future.
5

Commitment ②
Generate at least 50% ofconsolidated sales from overseas
Overseas
sales
26%（FY2020）

Domestic
sales

within 10 years

Overseas Domestic
sales
sales

50%

Our policy must be to develop operations not just in the US, but also in
Europe, China and other markets.
We will develop and launch a continual stream of distinctive new
products in the Japanese market.
6Commitment ③
Target at least 100% gains in net
sales and operating profit
FY202010 years later

Net sales

121,885

Double or
more

Operatingprofit26,134（million yen）

（million yen）

Double ormore

We will build a foundation to expand our net sales in the future to
300 billion yen, and from there to 500 billion yen.
7

Specific policies in each businessPharmaceuticals business

Functional food business

Focus areas

Focus areas

Health food ingredients
Preservatives
Protein preparations

Supplements
Focusing mainly on ourfour focus areas, we will seek
to develop our R&D pipeline.

Focusing mainly on ourfour focus areas, we will supply
high-value-added products.

In pharmaceuticals business, we will invest
aggressively to upgrade our R&D capabilities
through advanced drug discovery technologies,
and to develop new drug-discovery modalities
outside of nucleic acid medicines.

In our functional food business, we will use ouradvanced expertise as a manufacturer of
medicines to produce products which willaddress societal needs such as reduction offood waste and efforts to combat aging.
8

Initiatives for promoting digital transformation
Digital Vision

Nippon Shinyaku aims to fill the world with smiles by utilizing digital technology and dataand creating highly original products and services in the healthcare sector.

Digital strategies

As a milestone to accelerate digital transformation, we are working to obtainthe “DX Certification” granted by the Ministry of Economy, Trade and Industryof Japan (METI) and having the company’s stock selected to be on the list of“DX Stocks” within the framework of the METI and Tokyo Stock Exchange.
9

Our vision
Every employee will
pursue originality
and challenge with a sense of urgency

targeting to create distinctive products of high quality

10Outline of Consolidated Financial Results
for the 2nd Quarter Ended September 30, 2021

1H FY2021 Summary◆ Net sales：

71,568 million yen （+23.8% ）◆ Operating profit：

21,504 million yen （+93.0% ）◆ Ordinary profit：

22,250 million yen （+97.8% ）Profit attributable◆ to owners of parent：

16,561 million yen （+105.1%）（million yen）

12

Segmental Review - Pharmaceuticals 50,296+ 1,595
Revenues from the licensing ofindustrial property rights+11,195+343+13,133

63,430（Million yen）
Ethical drugs
Revenues from the licensing ofindustrial property rights

Profit in co-promotion
Net sales

1H FY2020

YoY Change

1H FY2021

Results

Ratio

Results

Ratio

Amt

%

36,40672.4%

38,00159.9%+1,595

+4.4%

9,769

19.4%

20,96433.1%+11,195+114.6%

4,121

8.2%

4,464

7.0%

+343

+8.3%

50,296100.0%

63,430100.0%+13,133+26.1%Net sales increased by 26.1% through growth of ethical drugs such as “Vidaza”, “Viltepso”, ”Uptravi”,
and revenues from the licensing of industrial property rights which contains of royalty revenue from
Uptravi’s overseas sales and gain on sales from the priority review voucher.
13

Segmental Review - Functional Food 7,521
+280
+110
+29
+196
+616

8,138
（Million yen）

1H FY2020

1H FY2021

YoY Change

Results

Ratio

Results

Ratio

Amt

%

Protein preparations

5,058

67.3%

5,339

65.6%

+280

+5.5%

Preservatives

1,271

16.9%

1,382

17.0%

+110

+8.7%

Health food ingredients

493

6.6%

523

6.4%

+29

+6.0%

Others

697

9.2%

893

11.0%

+196

+28.1%Net sales

7,521

100.0%

8,138

100.0%

+616

+8.2%

Net sales increased by 8.2% through sales from protein preparations,preservatives, and health food ingredients.
14

Operating profit
11,140+13,133+616+405-1,445-2,345+10,364

21,504（Million yen）

1H FY2020

1H FY2021

YoY Change

Results

Ratio

Results

Ratio

Amt

%

57,817100.0%

71,568100.0%+13,750+23.8%(Pharmaceuticals)(50,296)(87.0%)(63,430)(88.6%)(+13,133)(+26.1%)(Functional Food)(7,521)(13.0%)(8,138)(11.4%)(+616)(+8.2%)

Operating expenses

46,67780.7%

50,06370.0%+3,386+7.3%

Cost of sales

24,99443.2%

24,589

34.4%

-405

-1.6%

SG&A expenses

14,02324.3%

15,46821.6%+1,445+10.3%

R&D expenses

7,659

13.2%

10,00514.0%+2,345+30.6%

Operating profit

11,14019.3%

21,50430.0%+10,364+93.0%Net sales

15

Profit attributable to owners of parent
8,073＋10,364+527+111-2,514
16,561（Million yen）
Operating profit

1H FY2019

1H FY2020

Results

Results+8,487
YoY Change
Amt%

11,14021,504+10,364+93.0%

Non-operating income

600

1,127

+527

+87.8%

Non-operating expenses

492

380

-111

-22.6%

Ordinary profit

11,24822,250+11,002+97.8%

Income taxes, etc

3,174

5,689

+2,514+79.2%

8,073

16,561+8,487+105.1%

Profit attributable toowners of parent

16

Business Forecast for FY2021
FY2020(Million yen)

FY2021YoY Change

1H

FY

1H

FY

Results

Results

Results

Forecasts

Net sales

57,817

121,885

71,568Operating profit

11,14026,134

Ordinary profit

11,248
8,073

Profit attributable toowners of parent

Amt％

135,000+13,115+10.8%

21,50428,000+1,866+7.1%

26,76022,25028,500+1,740+6.5%

20,702

16,561

21,000

+298

+1.4%

We look for net sales, operating profit, ordinary profit andprofit attributable to owners of parent to increase year on year.

17

Segmental Forecast - Pharmaceuticals FY2020(Million yen)

Ethical drugs
Revenues from the licensing ofindustrial property rights

Profit in co-promotion
Net sales

FY2021YoY Change

1H

FY

1H

FY

Results

Results

Results

Forecasts

36,40673,69738,001

9,769

24,3384,121
50,296

Amt%

78,300+4,603+6.2%

20,96432,000+7,662+31.5%

8,442

4,464

9,000

+558

+6.6%

106,478

63,430

119,300+12,822+12.0%

Due to growth of new products, revenues from the licensing of industrial
property rights which contains of royalty revenue from Uptravi’s
overseas sales, and profit in co-promotion, our net sales will increase.

18

Segmental Forecast - Functional Food FY2020(Million yen)

FY2021YoY Change

1H

FY

1H

FY

Results

Results

Results

Forecasts

Protein preparations

5,058

10,121

5,339

9,850

-271

-2.7%

Preservatives

1,271

2,597

1,382

2,700

+103

+4.0%

Health food ingredients

493

1,257

523

1,100

-157

-12.5%

Others

697

1,431

893

2,050

+619

+43.2%Net sales

7,521

15,4068,138

15,700

+294

+1.9%

Amt

%

We expect an increase in net sales by focusing further on developing
new products and introducing high-value-added productsthat meet market needs.

19Consolidated Statements of Income (Forecast)
FY2020(Million yen)

FY2021YoY Change

1H

FY

1H

FY

Results

Results

Results

Forecasts

57,817

121,885

71,568(Pharmaceuticals)(50,296)(106,478)(Functional Food)(7,521)

Amt%

135,000+13,115+10.8%(63,430)(119,300)(+12,822)(+12.0%)(15,406)(8,138)(15,700)(+294)(+1.9%)

24,994

49,954

24,58948,700-1,254-2.5%(43.2%)(41.0%)(34.4%)(36.1%)

SG&A expenses

14,02329,691

15,46831,800+2,109+7.1%

R&D expenses

7,659

16,10410,00526,500+10,396+64.5%

Operating profit

11,14026,13421,50428,000+1,866+7.1%

Non-operating income

600

1,326

1,127

1,000

-326

-24.6%

Non-operating expenses

492

701

380

500

-201

-28.7%

Ordinary profit

11,24826,76022,25028,500+1,740+6.5%

Income taxes, etc

3,174

8,056

5,689

7,500

-556

-6.9%

8,073

20,702

16,561

21,000

+298

+1.4%

Net sales

Cost of sales(Cost of sales ratio)

Profit attributable toowners of parent

20

Dividends Forecast
FY2020

FY2021

Interim¥49¥51

Annual

¥99

¥103

EPS

¥307.37¥311.79

Dividends payout ratio

32.2 ％

33.0 ％

Dividends per share

(Yen)

99

103

86
70

AnnualInterim52

21

R&D Pipeline

R&D pipeline (Domestic)
Code No.
(Generic name)<Origin>Application
type

Indications

development

PI

PI

PII

PI

NS-065/NCNP-01(viltolarsen)

Preparation for Preparation for

NME

Duchenne muscular dystrophy

NME

Iron deficiency anemia

PIII

NDA

LaunchPIIPIII
in progress<in-house>
NS-32(ferric derisomaltose)<in-license>
ZX008(fenfluraminehydrochloride)

NME

Dravet syndrome
Lennox-Gastaut syndrome<in-license>
PIII

Arteriosclerosis obliteransNew
NS-304indication

Lumbar spinal stenosis(selexipag)<in-house>

New dose

NS-580<in-house>

NME

Pediatric pulmonary arterial

PIII

hypertension
Endometriosis

NS-87（daunorubicin /Newcytarabine）

combination

Secondary acute myeloid leukemia<in-license>
NS-229<in-house>

NME

NS-917(radgocitabine)

NME<in-license>
NS-401(tagraxofusp)<in-license>

NME

Inflammatory diseases
Relapsed/refractory acute myeloid
leukemia
Blastic plasmacytoid dendritic cell
neoplasm

23

R&D pipeline (Overseas)
Code No.
(Generic name)<Origin>Application
type

Indications

NS-065/NCNP-01(viltolarsen)

NME

Duchenne muscular dystrophy

New

Chronic thromboembolic pulmonary

indication

hypertension

NME

Myelofibrosis

Preparation fordevelopment

PI

Preparation for
PⅡ

PII

PIII

LaunchPIIIin progress<in-house>
NS-304(selexipag)<in-house>
NS-018(ilginatinib)<in-house>

24Reference Materials

Consolidated Balance Sheet
End of

End of 1H

YoY Change

End of

End of 1H

YoY Change

FY2020

FY2021Amt

FY2020

FY2021

Amt(Million yen)

Assets197,028204,052+7,024Current assets

139,090

146,788+7,697

Non-current assets

57,93757,264-673

Liabilities

34,48529,162-5,322Current liabilities

31,514

26,155-5,358Non-current liabilities

2,970

3,006

+35

162,543

174,890+12,346

197,028204,052+7,024

Net assets
Total assets

197,028204,052+7,024

Total liabilities
and net assets＝Liabilities and Net assets＝＝Assets＝Cash and deposits+6,529Notes and accounts payable

Securities+1,070Accounts payable-843

Investments and other assets-1,093

Income taxes payable-637

Retained earnings-2,369+13,19326

Consolidated Statements of Cash Flows（Million yen）

1H FY2020

1H FY2021

YoY Change

Results

Results

Amt

Operating activities

8,127

13,333+5,206

Investing activities-2,071-1,954+116

Financing activities-2,899-3,365-466

47,49465,883+18,388

Cash and cash equivalents
at end of year
1H FY2020

1H FY2021+ 13,333- 1,954- 3,365+ 8,127- 2,071- 2,899

57,88344,29865,88347,49427

NS-065/NCNP-01 (viltolarsen)- Treatment for Duchenne muscular dystrophy Development Phase

・Japan : Launch・USA : Launch・China : NDA filing・Global : PIII

OriginCo-development: National Center of Neurology andPsychiatry

Development

Nippon Shinyaku

Mechanism ofactionExon 53 Skipping

Indication

Duchenne muscular dystrophy

Dosage form

Injection

Feature・Improvement in symptoms and prevention of the diseaseprogression by recovery of dystrophin protein expression・Morpholino based oligonucleotide with possible highsafety profile and maximized activity28

NS-32 (ferric derisomaltose)- Treatment for iron deficiency anemia Development Phase

Japan: NDA filing

Origin[Dec. 2016]
Licensed-in from: Pharmacosmos A/SDevelopment

Nippon Shinyaku

Mechanism of action

Iron

Indication

Iron deficiency anemia

Dosage form

IV bolus injection or IV drip infusion

Feature・Can be administered in high doses allowing full ironcorrection in the majority of patients・Good safety profile with no dose dependent ADRs
・Minimal potential toxicity from release of labile iron due
to tight iron binding in a matrix structure ofinterchanging isomaltoside and iron・No profound hypophosphatemia29

ZX008 (fenfluramine hydrochloride)- Treatment for rare intractable epilepsy Development Phase

Japan: PIII

Origin[March. 2019]
Commercial rights from: Zogenix, Inc.

Development

Zogenix, Inc.

Mechanism of action

Serotonin agonist

Indication

Dravet syndrome and Lennox-Gastaut syndrome

Dosage form

Oral liquid agent

Feature・Effective for Dravet syndrome and Lennox-Gastautsyndrome patients refractory to existing treatment
options・ZX008 can be used in combination with other drugs, asstandard of care for intractable epilepsy based on
combination therapy
30

NS-304 (selexipag)-Treatment for pulmonary hypertension, arteriosclerosis obliterans, lumbar spinal stenosis-

Development Phase<CTEPH> Japan: LaunchOverseas: PIII
<ASO>
Japan: PIIb
<LSS>Japan: PIIa
<Pediatric PAH> Japan: PII

OriginNippon Shinyaku

Development

・Co-development in Japan:
Janssen Pharmaceutical K.K. (CTEPH / Pediatric PAH)
・Overseas: Johnson & Johnson (CTEPH / Pediatric PAH)
・Nippon Shinyaku (ASO)
・Nippon Shinyaku (LSS)

Mechanism of action

Selective IP receptor agonist

Indication

・Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
・Arteriosclerosis obliterans (ASO)
・Lumbar spinal stenosis (LSS)・Pediatric pulmonary arterial hypertension(Pediatric PAH)

Dosage form

TabletFeatureLong-acting oral drug31NS-580- Treatment for endometriosis Development Phase

Japan: PIIa

OriginNippon Shinyaku

Development

Nippon Shinyaku

Mechanism of action

Inhibition of membrane-associated prostaglandin E
synthase-1

Indication

Endometriosis

Dosage form

Oral agent

FeatureTreatment for endometriosis without hormonal effectand with possible analgesic potency32

NS-018 (ilginatinib)- Treatment for myelofibrosis Development Phase

Overseas (USA): Preparation for PII

OriginNippon Shinyaku

Development

Nippon Shinyaku

Mechanism of action

JAK2 inhibitor

Indication

Myelofibrosis

Dosage form

TabletFeature・Potent and highly selective JAK2 inhibitor
・High efficacy and safety are expected for
myelofibrosis (MF) patients with low platelet count,
for whom QOL improvement can’t be obtained
because no treatment is available

33

NS-87 (daunorubicin / cytarabine)- Treatment for secondary acute myeloid leukemia Development Phase

Japan: PI/II

Origin[Mar. 2017]
Licensed-in from: Jazz Pharmaceuticals plc

Development

Nippon Shinyaku

Mechanism of action

Liposomal combination of cytarabine and daunorubicin

Indication

Secondary acute myeloid leukemia (secondary AML)

Dosage form

Injection

Feature・NS-87 is the first therapy for the treatment of
secondary AML in Japan・The enhancement of antitumor activity and reducing
adverse events are expected by NS-87 accumulated inbone marrow34NS-229- Treatment for inflammatory diseases Development Phase

Japan: PI

OriginNippon Shinyaku

Development

Nippon Shinyaku

Mechanism of action

JAK1 inhibitor

Indication

Inflammatory diseases (to be determined)

Dosage form

Oral agent

Feature・Potent and highly selective JAK1 inhibitor
・High efficacy and good safety profiles are expected in
the treatment for inflammatory diseases35

NS-917 (radgocitabine)- Treatment for relapsed or refractory acute myeloid leukemia -Development Phase

Japan: Preparation for PI

Origin[Mar. 2017]
Licensed-in from: Delta-Fly Pharma, Inc.

Development

Nippon Shinyaku

Mechanism of action

DNA strand-break by incorporating itself into DNA

Indication

Relapsed or refractory (r/r) acute myeloid leukemia (AML)

Dosage form

Injection

Feature・Significant anti-leukemic activity with uniquemechanism of action from other nucleoside analogs at
low dose continuous infusion
・Tolerable safety profile available to elderly patients with
r/r AML36

NS-401 (tagraxofusp)- Treatment for blastic plasmacytoid dendritic cell neoplasm Development Phase

Japan: Preparation for Clinical Development

Origin[Mar. 2021]
Licensed-in from: The Menarini Group

Development

Nippon Shinyaku

Mechanism of action

Induction apoptosis of cells by inhibiting protein
synthesis by specifically targeting cancer cells
expressing CD123

Indication

Blastic plasmacytoid dendritic cell neoplasm (BPDCN)

Dosage form

Injection

Feature・Composed of diphtheria toxin (DT) fusion protein andrecombinant human IL-3
・Novel targeted therapy directed to CD123 on tumor
cells・IL-3 binds to CD123-expressing tumor cells anddelivers the cytotoxic diphtheria toxin to the cells,
resulting in the blockage of protein synthesis in thecell and causing cell death in CD123-expressing cells
37Safe Harbor Statement• Materials and information provided during this presentation may contain so-called “forward-looking
statements.” These statements are based on current expectations, forecasts and assumptions thatare subject to risks and uncertainties which could cause actual outcomes and results to differ materiallyfrom these statements.
• Risks and uncertainties include general industry and market conditions, and general domestic andinternational economic conditions such as interest rate and currency exchange fluctuations. Risks anduncertainties particularly apply with respect to product-related forward-looking statements. Product risks
and uncertainties include, but are not limited to, technological advances and patents attained bycompetitors; challenges inherent in new product development, including completion or failure of clinical
trials; claims and concerns about product safety and efficacy; regulatory agency’s examination, obtaining
regulatory approvals; domestic and foreign social security reforms; trends toward healthcare cost
containment; and governmental laws and regulations affecting domestic and foreign operations.

• Also, for products that are approved, there are manufacturing and marketing risks and uncertainties, which
include, but are not limited to, inability to build production capacity to meet demand, unavailability of raw
materials, and competition with others.
• The Company disclaims any intention or obligation to update or revise any forward-looking statements
whether as a result of new information, future events or otherwise.
• This English presentation was translated from the original Japanese version.
In the event of any inconsistency between the statements in the two versions, the statements in theJapanese version shall prevail.