Outline of Consolidated Financial Results
for the 2nd Quarter Ended September 30, 2022
November 14, 2022
NIPPON SHINYAKU CO., LTD.

2Q FY2022 Summary◆ Revenue：

71,136 million yen （ - 0.6% ）◆ Operating profit：

19,161 million yen （ - 12.1% ）◆ Profit before tax：

19,398 million yen （ - 11.9% ）Profit attributable：

15,222 million yen （ - 7.9% ）◆ to owners of parent
Revenue

Operating profit

Profit before tax

Profit attributable toowners of parent（Million yen）

2

Segmental Review - Pharmaceuticals 63,430+3,022
Revenues from the licensingof industrial property rights-6,495+541
60,499（Million yen）
Ethical drugs
Revenues from the licensing ofindustrial property rights

Profit in co-promotion
Revenue

1H FY2021-2,931YoY Change

1H FY2022

Results

Ratio

Results

Ratio

Amt

%

38,001

59.9%

41,02367.8%+3,022+8.0%

20,96433.1%

14,46923.9%-6,495-31.0%

4,464

7.0%

5,005

8.3%

+541

+12.1%

63,430100.0%

60,499100.0%-2,931-4.6%

Sales of Ethical drugs including “Viltepso” and “Uptravi”, Revenues from the licensing of industrial
property rights containing royalty revenue from Uptravi’s overseas sales grew.
However, due to backlash from the loss of sales revenue from the priority review voucher booked in
1Q FY2021, Revenue of consolidated pharmaceuticals segment decreased by 4.6%.
3

Segmental Review - Functional Food 8,103+2,068
+40

-15
+447
+2,534

10,637（Million yen）

1H FY2021

1H FY2022

YoY Change

Results

Ratio

Results

Ratio

Amt

%

Protein preparations

5,292

65.3%

7,360

69.2%

+2,068+39.1%

Preservatives

1,384

17.1%

1,424

13.4%

+40

+2.9%

Health food ingredients

527

6.5%

511

4.8%

-15

-3.0%

Others

893

11.1%

1,340

12.6%

+447

+50.1%

Revenue

8,103

100.0%

10,637100.0%+2,534+31.3%

Revenue of consolidated functional food segment increased by 31.3% throughsales from Protein preparations including milk proteins and supplements suchas “WINZONE Protein”.
4Operating profit
21,808-397-3,736-902＋426＋2,436-473-2,647

19,161（Million yen）

1H FY2021

1H FY2022

YoY Change

Results

Ratio

Results

Ratio

71,533100.0%

71,136100.0%

-397

-0.6%

(Pharmaceuticals)(63,430)(88.7%)(60,499)(85.0%)(-2,931)(-4.6%)(Functional Food)(8,103)(11.3%)(10,637)(15.0%)(+2,534)(+31.3%)

Cost of sales

24,25433.9%

27,991

39.3%+3,736+15.4%

SG&A expenses

15,381

21.5%

16,284

22.9%

+902

+5.9%

R&D expenses

10,117

14.1%

9,691

13.6%

-426

-4.2%

Other income

369

0.5%

2,805

3.9%

+2,436+660.1%Other expenses

340

0.5%

813

1.2%

+473

+139.0%Operating profit

21,80830.5%

19,16126.9%-2,647-12.1%

RevenueAmt%

5Profit attributable to owners of parent
16,519-2,647+51-12＋1,310-1,297

15,222（Million yen）

1H FY2021

1H FY2022

Results

Results

YoY Change
Amt%

Operating profit

21,808

19,161-2,647-12.1%

Finance income

246

297

+51

+20.9%

Finance costs

48

60

+12

+26.0%

Profit before tax

22,006

19,398-2,608-11.9%

Income tax expense, etc

5,487

4,176

-1,310-23.9%

16,51915,222-1,297-7.9%

Profit attributable toowners of parent

6

Business Forecast for FY2022
FY2021(Million yen)

FY2022YoY Change

1H

FY

1H

FY

Results

Results

Results

Forecasts

71,533

137,484

71,136(Pharmaceuticals)(63,430)(120,650)(Functional Food)(8,103)Operating profit
Profit before tax

Revenue

Profit attributable toowners of parent

Amt％

141,000+3,516+2.6%(60,499)(119,500)-1,150-1.0%(16,834)(10,637)(21,500)+4,666+27.7%

21,80832,948

19,161

30,000-2,948-8.9%

22,00633,301

19,39830,400-2,901-8.7%

16,51924,986

15,222

24,000

-986

-3.9%

We expect sales of functional food, Viltepso in the U.S., domestic
pharmaceutical products, etc. to exceed the previous projection.
Therefore, we have revised our annual forecasts of Revenue, Operating
profit, Profit before tax, and Profit attributable to owners of parent.

7Segmental Forecast - Pharmaceuticals FY2021(Million yen)

Ethical drugs
Revenues from the licensing ofindustrial property rights

Profit in co-promotion
Revenue

FY2022YoY Change

1H

FY

1H

FY

Results

Results

Results

Forecasts

38,00178,50841,023

20,96433,207

4,464
63,430Amt%

79,800+1,292+1.6%

14,46930,200-3,007

-9.1%

8,934

5,005

9,500

+566

+6.3%

120,650

60,499119,500-1,150-1.0%

We look for sales of new products such as “Viltepso” and “Upravi, royalty
revenue from Uptravi’s overseas sales included in the Revenues from thelicensing of industrial property rights, and Profit in co-promotion to grow.
However, due to backlash from the loss of sales revenue from the priority
review voucher booked in FY2021, we predict Revenue of consolidated
pharmaceuticals segment to decrease.

8

Segmental Forecast - Functional Food FY2021(Million yen)

FY2022YoY Change

1H

FY

1H

FY

Results

Results

Results

Forecasts

Protein preparations

5,292

10,8707,360

Preservatives

1,384

2,788

Health food ingredients

527

Others
RevenueAmt%

15,000+4,130+38.0%

1,424

2,900

+112

+4.0%

1,078

511

1,100

+22

+2.0%

899

2,096

1,340

2,500

+404

+19.2%

8,103

16,834

10,63721,500+4,666+27.7%

We predict Revenue of consolidated functional food segment
to increase from the growth of sales of Protein preparations,
supplements, etc.

9Forecast of Consolidated Statements of Income
FY2021(Million yen)

FY2022YoY Change

1H

FY

1H

FY

Results

Results

Results

Forecasts

71,533

137,484

71,136(Pharmaceuticals)(63,430)(120,650)(Functional Food)(8,103)

Cost of sales

Amt%

141,000+3,516+2.6%(60,499)(119,500)(-1,150)(-1.0%)(16,834)(10,637)(21,500)(+4,666)(+27.7%)

24,25450,191

27,991

54,900+4,709+9.4%

SG&A expenses

15,381

32,173

16,28434,000+1,827+5.7%

R&D expenses

10,11722,863

9,691

24,000+1,137+5.0%

Other income

369

1,573

2,805

3,000

+1,427+90.6%

Other expenses

340

882

813

1,100

+218

+24.7%

Operating profit

21,80832,948

19,161

30,000-2,948-8.9%

Finance income

246

472

297

500

+28

+5.8%

Finance costs

48

119

60

100

-19

-16.0%

Profit before tax

22,00633,301

19,39830,400-2,901-8.7%

Income tax expense, etc

5,487

8,315

4,176

6,400

-1,915-23.0%

16,51924,986

15,222

24,000

-986

-3.9%

Revenue

Profit attributable toowners of parent

10Dividends Forecast
FY2021

FY2022(JGAAP)(IFRS)

Interim¥51¥57

Annual¥110¥114

Basic earnings per share¥342.14¥356.33

Payout ratio (consolidated)32.2 ％

32.0 ％

Dividends per share*Interim dividend for FY2022 is raised from 56 yen to 57 yen per share*Annual dividend for FY2022 is raised from 112 yen to 114 yen per share

(Yen)

110

114

99
86
Annual70

Interim11(estimated)

R&D Pipeline

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

Indications

for PI

PI/II

PI

PI

NS-065/NCNP-01(viltolarsen)

Preparation

NME

Duchenne muscular dystrophy

NME

Iron deficiency anemia

NME

Dravet syndrome

NME

Lennox-Gastaut syndrome<in-house>

PIII

PII

NDA

Preparation

Launchfor launch

PII

PIII
in progress

NS-32(ferric
derisomaltose)<in-license>
ZX008(fenfluraminehydrochloride)<in-license>
ZX008(fenfluraminehydrochloride)<in-license>
GA101（obinutuzumab）<in-license>

New
indicationNew
NS-304indication

Lupus nephritis

Arteriosclerosis obliterans(selexipag)<in-house>
New dose

Pediatric pulmonary arterial
hypertension： Changes from 1st Quarter FY2022

13

R&D Pipeline (Domestic)
Code No.
(Generic name)<Origin>
NS-580<in-house>
NS-87（daunorubicin /cytarabine）<in-license>

Application
type

NME

New
combination

NS-401(tagraxofusp)

NME<in-license>

NS-229<in-house>

NME

NS-917(radgocitabine)

NME<in-license>

NS-161<in-house>

NS-025<in-house>

Indications

Preparation
for PI

PI

PI/II

PII

PIII

NDA

Preparation

Launchfor launch

Endometriosis

Secondary acute myeloid
leukemia

Blastic plasmacytoid dendritic
cell neoplasm

Inflammatory diseases

Relapsed/refractory acute
myeloid leukemia

NME

Inflammatory diseases

NME

Urological diseases： Changes from 1st Quarter FY2022

14

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

Indications

NS-065/NCNP-01(viltolarsen)

NME

Duchenne muscular dystrophy

NME

Duchenne muscular dystrophy

NME

Myelofibrosis<in-house>

CAP-1002<partnership>PI

Preparation
for PⅡ

PII

PIII

LaunchPIIIin progress

NS-018(ilginatinib)<in-house>

15Reference Materials

Consolidated Balance Sheet
End of

End of 1H

YoY Change

End of

End of 1H

YoY Change

FY2021

FY2022Amt

FY2021

FY2022Amt(Million yen)

Assets219,943

225,374+5,430Current assets

149,724

155,339+5,614Non-current assets

70,21970,035-183

Liabilities

39,05733,870-5,186

Current liabilities

32,02927,365-4,663

Non-current liabilities

7,027

6,504

-522

180,886

191,503+10,617219,943

225,374+5,430EquityTotal assets

219,943

225,374+5,430Total liabilities
and equity＝Liabilities and Equity＝＝Assets＝Cash and cash equivalents+2,883

Trade and other payables

Other current assets+1,752Retained earnings-5,892＋11,26417

Consolidated Statements of Cash Flows（Million yen）

1H FY2021

1H FY2022

YoY Change

Results

Results

Amt

Operating activities

14,113

12,817-1,295Investing activities-1,935-5,882-3,947

Financing activities-4,161-4,911-749

65,88363,449-2,433

Cash and cash equivalents
at end of period+ 12,817+14,113- 1,935- 5,882- 4,161- 4,91165,88357,883

Cash andcash equivalents
at beginning of period

OperatingCF

InvestingCF

FinancingCF

Cash andcash equivalents
at end of period

60,566

Cash andcash equivalents
at beginning of period

63,449OperatingCF

InvestingCF

FinancingCF

Cash andcash equivalents
at end of period18NS-065/NCNP-01 (viltolarsen)- Treatment for Duchenne muscular dystrophy Development Phase

・Japan : Launch・USA : Launch・Global : PIII in progress

OriginCo-development :National Center of Neurology and Psychiatry

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 activity

19

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

Japan : Preparation for launch

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 of interchanging
isomaltoside and iron・No profound hypophosphatemia20

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

Japan : Preparation for launch (Dravet syndrome)
Japan : PⅢ （Lennox-Gastaut syndrome)

Origin[Mar. 2019]
Commercial rights from : UCB S.A. (former Zogenix, Inc.)

Development

UCB S.A. (former Zogenix, Inc.)

Mechanism of action

5-HT (serotonin) releaser with agonist activity at several
5-HT receptors

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
21

CAP-1002- Treatment for Duchenne muscular dystrophy Development Phase

USA : PIII

Origin[Jan. 2022]
Partnership for commercialization :Capricor Therapeutics, Inc.

Development

Capricor Therapeutics, Inc.

Mechanism ofactionExosomes released from cardiosphere-derived cells

Indication

Duchenne muscular dystrophy

Dosage form

Injection

Feature・Exosomes released from this drug are expected to reduce
oxidative stress, inflammation, fibrosis, and increase cell
energy and myocyte generation, resulting in improvementof motor and cardiac functions
・Its broad applicability makes it suitable for patients
regardless of the type of genetic mutation
22

GA101 (Obinutuzumab)- Treatment for lupus nephritis Development Phase

Japan : PIII

Origin[Nov. 2012]
Licensed-in from : Chugai Pharmaceutical Co., Ltd.

Development

Co-development : Chugai Pharmaceutical Co., Ltd.

Mechanism ofactionAnti-CD20 monoclonal antibody

Indication

Lupus nephritis

Dosage form

Injection

FeatureAnti-CD20 monoclonal antibody, increased antibodydependent cellular cytotoxicity (ADCC) activity and direct
cytotoxicity23

NS-304 (selexipag)
for pulmonary
hypertension,-- Treatment
Treatment
for Luhritis- arteriosclerosis obliterans Development Phase

Japan : PIIb（ASO）
Japan : PII （Pediatric PAH）

OriginNippon Shinyaku

Development

・Nippon Shinyaku（ASO）
・Co-development :Janssen Pharmaceutical K.K. (Pediatric PAH)

Mechanism of action

Selective IP receptor agonist

Indication

・Arteriosclerosis obliterans (ASO)
・Pediatric pulmonary arterial hypertension (Pediatric
PAH)

Dosage form

TabletFeatureLong-acting oral drug24

NS-580- Treatment for endometriosis Development Phase

Japan : PIIb

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 effect andwith possible analgesic potency25

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

Overseas : 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 count26

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 daunorubicin and cytarabine

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 marrow27

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

Japan : PI/II

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

Development

Nippon Shinyaku

Mechanism of action

Induction apoptosis of cells by inhibiting protein synthesisby 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 and delivers
the cytotoxic diphtheria toxin to the cells, resulting in
the blockage of protein synthesis in the cell and causing
cell death in CD123-expressing cells
28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 diseases29NS-917 (radgocitabine)- Treatment for relapsed or refractory acute myeloid leukemia Development Phase

Japan : 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 AML30

NS-161- Treatment for inflammatory diseases Development Phase

Japan : Preparation for PI

OriginNippon Shinyaku

Development

Nippon Shinyaku

Mechanism of action－

Indication

Inflammatory diseases (to be determined)

Dosage form

Oral agent

Feature－31

NS-025- Treatment for urological diseases Development Phase

Japan : Preparation for PI

OriginNippon Shinyaku

Development

Nippon Shinyaku

Mechanism of action－

Indication

Urological diseases (to be determined)

Dosage form

Oral agent

Feature－32

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.

Nippon Shinyaku Co., Ltd.
Financial Results for the Second Quarter Ended September 30, 2022
November 14, 2022

Nakai: I am Toru Nakai, President of Nippon Shinyaku Co., Ltd.
Thank you very much for participating in today’s financial results briefing for FY2022Q2. I would like toexpress my greatest gratitude.
Today, I will explain our financial results for Q2 of FY2022 and the forecast for the full year of FY2022. Aftermy presentation, Mr. Takagaki will discuss the progress of our R&D compounds.

I would like to begin with an overview of our performance in Q2 of FY2022. We reported consolidated
revenue of JPY71,136 million, operating profit of JPY19,161 million, profit before tax of JPY19,398 million,
and profit attributable to owners of parent of JPY15,222 million for the quarter.

1

In the pharmaceuticals business, although sales of Viltepso, a treatment for Duchenne muscular dystrophy,
and sales of Uptravi, a treatment for pulmonary arterial hypertension and chronic thromboembolic
pulmonary hypertension, as well as royalty income from overseas sales of Uptravi grew, consolidated
revenue of Pharmaceuticals business declined 4.6% YoY to JPY60,499 million, due to the loss of sales
revenue of the priority review voucher booked in 1H FY2021.

2

In the functional food business, sales of protein products including milk protein and supplements such as
WINZONE Protein for general consumers increased, resulting in consolidated revenue of JPY10,637 million,
up 31.3% YoY.

3

Next, in terms of operating expenses, the cost-of-sales ratio deteriorated 5.4 percentage point YoY to 39.3%,
due to factors such as the sales mix, including a decrease in the licensing of industrial property rights.
SG&A expenses increased by 5.9% YoY to JPY16,284 million, mainly due to expenses related to the US
subsidiary and an increase in sales promotion fees in line with increased domestic sales of Uptravi.
R&D expenses totaled JPY9,691 million, down 4.2%YoY, mainly due to a decrease in manufacturing costs forinvestigational nucleic acid drugs.
Other income totaled JPY28,05 million, mainly due to foreign exchange gains.
As a result, operating profit was JPY19,161 million, down 12.1%YoY.

4

Profit before tax was JPY19,398 million, down 11.9% YoY, and profit attributable to owners of parent was
JPY15,222 million, down 7.9% YoY.

5

I will now explain our full-year business forecast for FY2022.
As announced on November 10, we have revised our previous forecast announced in May.
Consolidated revenue was revised from JPY134,000 million to JPY141,000 million, as the performance of thefunctional food business, Viltepso in the US, and domestic pharmaceuticals are expected to exceed theinitial forecast.
As for consolidated profit, we revised operating profit from JPY27,000 million to JPY30,000 million, profit
before tax from JPY27,500 million to JPY30,400 million, and profit attributable to owners of parent from
JPY21,500 million to JPY24,000 million.
The exchange rate assumption was changed from JPY122 to USD1 at the beginning of the period to JPY140
to USD1.

6

In the pharmaceuticals business, although sales of new products such as Viltepso and Uptravi, royalty
revenue from overseas sales of Uptravi and profit in co-promotion are expected to grow. However, due tothe loss of sales revenue from the priority review voucher booked in the previous fiscal year, revenue is
expected to decrease by 1% YoY to JPY119,500 million.

7

In the functional food business, revenue is expected to be JPY21,500 million, an increase of 27.7% YoY, due
to growth in protein preparations and supplements.

8

Next, regarding operating expenses, the cost-of-sales ratio is expected to be 38.9%, a deterioration of 2.4
percentage points YoY. SG&A expenses are expected to be JPY34,000 million, and R&D expenses are
expected to be JPY24,000 million.
As a result, operating profit is expected to be JPY30,000 million, profit before tax JPY30,400 million, andprofit attributable to owners of parent JPY24,000 million.

9

As for dividends, we plan to link dividends to business performance during the Sixth Mid-term Management
Plan, with a consolidated payout ratio of around 35%.
In accordance with this dividend policy, we plan to increase the interim dividend by JPY1 per share to JPY57
per share, and the annual dividend forecast by JPY2 per share to JPY114 per share for FY2022, as profit
attributable to owners of parent is expected to exceed the initial forecast.
This concludes my presentation of the financial results for Q2 of FY2022 and the forecast for FY2022.

10

Takagaki: I will continue with an explanation of the progress of R&D compounds.
First, I will explain the development situation in Japan.
A treatment for Duchenne muscular dystrophy, NS-065/NCNP-01, Viltepso, was launched in May 2020. A
global Phase III study is currently underway.
In March this year, we obtained manufacturing and marketing approval for NS-32, MonoVer, a treatment foriron deficiency anemia, and are currently preparing for its launch.
As for ZX008, a treatment for intractable epilepsy, UCB Japan Co., Ltd. obtained manufacturing andmarketing approval for Dravet syndrome under the name of Fintepla in September 2022. We are currently
preparing for its launch. In addition, UCB S.A. is conducting a Phase III study for Lennox-Gastaut syndrome.
In June of this year, we initiated a Phase III study of GA101 for lupus nephritis in collaboration with Chugai
Pharmaceutical Co.
Nippon Shinyaku has been independently conducting Phase IIb study of NS-304 for arteriosclerosis
obliterans since February of this year. In addition, Phase II study for pediatric pulmonary arterial
hypertension is ongoing in collaboration with Janssen Pharmaceutical K.K.

11

As for NS-580, a treatment for endometriosis, we completed Phase IIa study and started Phase IIb study inJune this year.
As for NS-87, a treatment for secondary acute myeloid leukemia, Phase I/II study is ongoing.
In July of this year, we initiated a Phase I/II study for NS-401, a treatment for blastic plasmacytoid dendritic
cell neoplasm.
A Phase I study is ongoing for JAK1 inhibitor, NS-229, for inflammatory diseases.
As for NS-917, a treatment for relapsed/refractory acute myeloid leukemia, we have been conducting Phase
I study since February this year.
We have newly added two in-house discovered compounds to our pipeline.
We are currently preparing Phase I study of NS-161 for inflammatory diseases and Phase I study of NS-025
for urological diseases.

12I will continue with an explanation of the overseas R&D situation.
A treatment for Duchenne muscular dystrophy, NS-065/NCNP-01, Viltepso, was launched in the U.S. in
August 2020 and global Phase III study is currently underway. It received Orphan Drug Designation in Europe
in June 2020.
With regard to CAP-1002, a treatment for Duchenne muscular dystrophy, we concluded a sales anddistribution partnership agreement with Capricor Therapeutics Inc. for the territory of U.S. in January 2022.
Currently, Capricor Therapeutics Inc. is conducting Phase III study in the U.S.
We are currently preparing for Phase 2 study in the U.S. for NS-018, a treatment for myelofibrosis.
Procedures with investigational sites and screening of eligible patients are underway.
That is all for an overview of our research and development activities.

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