GlobeNewswire Inc | Aug 11, 2021 06:00AM EDT

August 11, 2021

- Received Orphan Drug Designation from the European Medicines Agency for INZ-701 for the treatment of ABCC6 Deficiency

Clinical Trial Application in Europe accepted for Phase 1/2 trial of INZ-701 in ABCC6 Deficiency

Investigational New Drug application in U.S. accepted for Phase 1/2 trial of INZ-701 in ABCC6 Deficiency

- Expect to enroll patients in Phase 1/2 clinical trials in ENPP1 Deficiency and ABCC6 Deficiency in Q4 2021 and report preliminary biomarker and safety data in the first half of 2022

- Cash, cash equivalents, and investments expected to support continued operations into the fourth quarter of2022

BOSTON, Aug. 11, 2021 (GLOBE NEWSWIRE) -- Inozyme Pharma, Inc. (Nasdaq: INZY), a rare disease biopharmaceutical company developing novel therapeutics for the treatment of abnormal mineralization, today reported financial results for the second quarter ended June 30, 2021, and provided recent business highlights.

We achieved several important milestones during the second quarter of 2021. We expect to initiate our clinical trials in ENPP1 Deficiency and ABCC6 Deficiency in the fourth quarter of this year and then to report preliminary biomarker and safety data in the first half of 2022, said Axel Bolte, MSc, MBA, Inozymes co-founder, president, and chief executive officer. We continue to make progress towards our mission of bringing hope to patients and their families who are living with ENPP1 Deficiency and ABCC6 Deficiency. In addition, we added several talented members to our leadership team who will contribute to this next phase of our growth.

Recent Business Highlights

-- Clinical Trial Application (CTA) in Europe and Investigational New Drug (IND) application in U.S. for ABCC6 Deficiency accepted The Company expects to enroll patients in a Phase 1/2 clinical trial in the fourth quarter of 2021 and report preliminary biomarker and safety data in the first half of 2022. -- Published data from Natural History Study of ENPP1 and ABCC6 Deficiencies Peer-reviewed article in the Journal of Bone and Mineral Research shows early mortality risk in GACI patients despite attempts to treat with bisphosphonates, high prevalence of rickets almost exclusive to ENPP1 Deficiency, and a spectrum of heterogenous calcification and multiple organ complications with both ENPP1 and ABCC6 variants, which suggests an overlapping pathology. -- Appointed Gayle Gironda as senior vice president, human resources Ms. Gironda is a human resources leader with more than 20 years of experience in organizational design, talent recruitment, performance culture, planning and leadership development. -- Appointed David Thompson, M.A., M.S., Ph.D. as chief development officer Dr. Thompson has more than 30 years of experience designing and leading research and development programs focused on bone disorders and phosphate regulation. He previously served as Inozymes senior vice president and chief scientific officer from 2018 to 2020 and was responsible for scientific research as the Company built its proprietary pipeline of investigational therapies, beginning with INZ-701.

Upcoming Anticipated Milestones

The Company also announced the following anticipated milestones for the INZ-701 clinical development program, subject to COVID-19-related restrictions:

-- ENPP1 DeficiencyQ4 2021: Start enrollment for Phase 1/2 clinical trialQ1 2022: Initiate prospective natural history studyH1 2022: Report preliminary safety and biomarker data from Phase 1/2 clinical trial -- ABCC6 DeficiencyQ4 2021: Start enrollment for Phase 1/2 clinical trialH1 2022: Report preliminary safety and biomarker data from Phase 1/2 clinical trial

Financial Results for the Quarter Ended Jun 30, 2021

-- Cash Position and Financial Guidance Cash, cash equivalents, and investments were$137.5 millionas of June 30, 2021. Based on its current plans, the Company expects that its existing cash, cash equivalents, and investments will be sufficient to enable funding of its operating expenses and capital expenditure requirements into the fourth quarter of 2022. -- Research and Development (R&D) Expenses R&D expenses were$8.2 millionfor the quarter ended June 30, 2021, compared to$7.9 millionfor the quarter ended June 30, 2020. The increase was primarily due to increased salaries, employee-related costs, and stock-based compensation expense due to the growth in the number of R&D employees. These increases were partially offset by a decrease in preclinical toxicology studies in support of our IND filing for INZ-701, and lower manufacturing costs based on the timing of production runs. -- General and Administrative (G&A) Expenses G&A expenses were$4.4 millionfor the quarter ended June 30, 2021, compared to$1.7 millionfor the quarter ended June 30, 2020. The increase was primarily due to the growth in the number of G&A employees, an increase in legal fees related to new contracts and operations as a public company and generally higher fees in areas such as audit, tax, and information technology to support the Companys growth. -- Net Loss Net loss was$12.5 million, or$0.53loss per share, for the quarter ended June 30, 2021, compared to$9.5 million, or$7.57loss per share, for the quarter ended June 30, 2020.

AboutInozyme Pharma

Inozyme Pharma, Inc. (Nasdaq: INZY), is a rare disease biopharmaceutical company developing novel therapeutics for the treatment of diseases of abnormal mineralization impacting the vasculature, soft tissue, and skeleton. Through our in-depth understanding of the biological pathways involved in mineralization, we are pursuing the development of therapeutics to address the underlying causes of these debilitating diseases. It is well established that two genes, ENPP1 and ABCC6, play key roles in a critical mineralization pathway and that defects in these genes lead to abnormal mineralization. We are initially focused on developing a novel therapy to treat the rare genetic diseases of ENPP1 and ABCC6 Deficiencies.

Inozyme Pharmawas founded in 2017 byJoseph Schlessinger, Ph.D.,Demetrios Braddock, M.D., Ph.D., andAxel Bolte, MSc, MBA, with technology developed byDr. Braddockand licensed fromYale University. For more information, please visitwww.inozyme.com.

Cautionary Note Regarding Forward-Looking Statements

Statements in this press release about future expectations, plans, and prospects, as well as any other statements regarding matters that are not historical facts, may constitute forward-looking statements within the meaning of The Private Securities Litigation Reform Act of 1995. These statements include, but are not limited to, statements relating to the initiation and timing of our clinical trials, the initiation and timing of our natural history study, our research and development programs, the availability of preclinical study and clinical trial data, the timing of our regulatory applications and the period over which we believe that our existing cash, cash equivalents and investments will be sufficient to fund our operating expenses. The words anticipate, believe, continue, could, estimate, expect, intend, may, plan, potential, predict, project, should, target, will, would and similar expressions are intended to identify forward-looking statements, although not all forward-looking statements contain these identifying words. Any forward-looking statements are based on managements current expectations of future events and are subject to a number of risks and uncertainties that could cause actual results to differ materially and adversely from those set forth in, or implied by, such forward-looking statements. These risks and uncertainties include, but are not limited to, risks associated with the Companys ability to initiate its planned Phase 1/2 clinical trials of INZ-701 for ENPP1 Deficiency and ABCC6 Deficiency; obtain and maintain necessary approvals from the FDA and other regulatory authorities; continue to advance its product candidates in preclinical studies and clinical trials; replicate in later clinical trials positive results found in preclinical studies and early-stage clinical trials of its product candidates; advance the development of its product candidates under the timelines it anticipates in planned and future clinical trials; obtain, maintain and protect intellectual property rights related to its product candidates; manage expenses; and raise the substantial additional capital needed to achieve its business objectives. For a discussion of other risks and uncertainties, and other important factors, any of which could cause the Companys actual results to differ from those contained in the forward-looking statements, see the Risk Factors section in the Companys most recent Annual Report on Form 10-K filed with the Securities and Exchange Commission, as well as discussions of potential risks, uncertainties and other important factors, in the Companys most recent filings with theSecurities and Exchange Commission. In addition, the forward-looking statements included in this press release represent the Companys views as of the date hereof and should not be relied upon as representing the Companys views as of any date subsequent to the date hereof. The Company anticipates that subsequent events and developments will cause the Companys views to change. However, while the Company may elect to update these forward-looking statements at some point in the future, the Company specifically disclaims any obligation to do so.

Condensed Consolidated Balance Sheet Data(Unaudited)

(in thousands)

June 30, December 31, 2021 2020Cash, cash equivalents and investments $ 137,464 $ 159,896 Total assets 148,259 169,363 Total liabilities 9,985 11,260 Additional paid-in-capital 252,920 249,175 Accumulated deficit (114,666 ) (91,076 )Total stockholders' equity 138,274 158,103

Condensed Consolidated Statements of Operations and Comprehensive Loss(Unaudited)

(in thousands, except share and per share data)

ThreeMonthsEndedJune 30, 2021 2020 Operating expenses: Research and development $ 8,220 $ 7,877 General and administrative 4,435 1,671 Total operating expenses 12,655 9,548 Loss from operations (12,655 ) (9,548 )Other income (expense): Interest income 58 71 Other income (expense) 57 4 Other income (expense), net 115 75 Net loss $ (12,540 ) $ (9,473 )Other comprehensive income (loss): Unrealized gains (losses) on available-for-sale 6 (15 )securitiesTotal other comprehensive income (loss) 6 (15 )Comprehensive loss $ (12,534 ) $ (9,488 )Net loss attributable to common $ (12,540 ) $ (9,473 )stockholders?basicand dilutedNet loss per share attributable to $ (0.53 ) $ (7.57 )commonstockholders?basic and dilutedWeighted-average common shares outstanding?basic 23,490,591 1,251,244 and diluted

Six MonthsEndedJune 30, 2021 2020 Operating expenses: Research and development $ 14,823 $ 14,283 General and administrative 8,804 3,171 Total operating expenses 23,627 17,454 Loss from operations (23,627 ) (17,454 )Other income (expense): Interest income 121 242 Other income (expense) (84 ) 1 Other income (expense), net 37 243 Net loss $ (23,590 ) $ (17,211 )Other comprehensive income (loss): Unrealized gains (losses) on available-for-sale 16 8 securitiesTotal other comprehensive income (loss) 16 8 Comprehensive loss $ (23,574 ) $ (17,203 )Net loss attributable to common $ (23,590 ) $ (17,211 )stockholders?basicand dilutedNet loss per share attributable to $ (1.01 ) $ (14.01 )commonstockholders?basic and dilutedWeighted-average common shares 23,460,218 1,228,296 outstanding?basicand diluted

Contacts

Investors:Inozyme PharmaStefan Riley, Director of Investor RelationsStefan.riley@inozyme.com

Media:Alex Van Rees, SmithSolve(973) 442-1555 ext. 111Alex.vanrees@smithsolve.com