GlobeNewswire Inc | May 12, 2021 06:00AM EDT

May 12, 2021

Filed Clinical Trial Application for INZ-701 for ABCC6 Deficiency in Europe; on track to initiate Phase 1/2 clinical trial in mid-2021

Published peer-reviewed preclinical data supporting INZ-701 as a potential treatment for ENPP1 Deficiency in Journal of Bone and Mineral Research

Presented data from first-ever Burden of Illness Study for ENPP1 and ABCC6 Deficiencies at multiple medical conferences

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

BOSTON, May 12, 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 first quarter ended March 31, 2021 and provided recent business highlights.

We closed 2020 with the clearance of our IND for INZ-701 by the FDA and our CTA by the MHRA of the UK, both for ENPP1 Deficiency. Since then, we have continued to execute on our plan to deliver a potential therapeutic option for patients with ENPP1 and ABCC6 Deficiencies, said Axel Bolte, MSc, MBA, co-founder, president, and chief executive officer of Inozyme Pharma. We recently reported key findings from the Burden of Illness Study in patients with both ENPP1 Deficiency and ABCC6 Deficiency. Notably, we found patients in different age groups are impacted by these deficiencies in distinct ways, illuminating an age-based shift that reflects the progression of these debilitating genetic diseases. We expect site activation in the United States for our Phase 1/2 clinical trial site for ENPP1 Deficiency in June 2021 and enrollment of the first patient shortly thereafter. Subject to receiving regulatory clearance, we also expect to initiate our planned Phase 1/2 clinical trial of INZ-701 for ABCC6 Deficiency in mid-2021.

Mr. Bolte continued, Our current cash position allows us to appropriately resource each of the key functions necessary to execute on our goals, and is expected to enable our operations into the fourth quarter of 2022.

Recent Business Highlights

-- Filed Clinical Trial Application for INZ-701 for ABCC6 Deficiency in Europe The Company expects to initiate a Phase 1/2 clinical trial in mid-2021 and to provide preliminary safety and biomarker data by the end of 2021. -- Published data supporting INZ-701 as a potential treatment for ENPP1 Deficiency Peer-reviewed article in the Journal of Bone and Mineral Research shows INZ-701 increased plasma pyrophosphate (PPi) levels, improved disease markers, and decreased mortality in an Enpp1-deficient mouse model. Preclinical findings support increase of PPi levels as a predictive marker of therapeutic benefit. -- Data presented on utility of INZ-701 as a potential treatment for ABCC6 Deficiency The Company and research collaborators from Thomas Jefferson University presented data from a preclinical study examining INZ-701 for the potential treatment for ABCC6 Deficiency/Pseudoxanthoma elasticum (PXE) at multiple medical conferences. -- Data presented from Burden of Illness Study for ENPP1 and ABCC6 Deficiencies The Company presented data from the first-ever burden of illness study in patients with ENPP1 and ABCC6 Deficiencies at multiple medical conferences.

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 DeficiencyJune 2021: Site activation for Phase 1/2 clinical trialMid-2021: Enrollment for Phase 1/2 clinical trialMid-2021: Initiate prospective natural history studyH2 2021: Report preliminary safety and biomarker data from Phase 1/2 clinical trial -- ABCC6 DeficiencyMid-2021: Site activation and enrollment for Phase 1/2 clinical trialBy the End of 2021: Report preliminary safety and biomarker data from Phase 1/2 clinical trial

Financial Results for the Quarter Ended March 31, 2021

-- Cash Position and Financial Guidance Cash, cash equivalents, and investments were$147.6 millionas ofMarch 31, 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$6.6 millionfor the quarter ended March 31, 2021, compared to$6.4 millionfor the quarter ended March 31, 2020. The increase was primarily due to costs associated with preclinical studies and clinical preparation activities with the Companys contract research organization and increased salaries and employee-related costs due to the growth in the number of R&D employees. -- General and Administrative (G&A) Expenses G&A expenses were$4.4 millionfor the quarter ended March 31, 2021, compared to$1.5 millionfor the quarter ended March 31, 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$11.1 million, or$0.47loss per share, for the quarter endedMarch 31, 2021, compared to$7.7 million, or$6.42loss per share, for the quarter ended March 31, 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)

March 31, 2021 December 31, 2020Cash, cash equivalents and investments $ 147,634 $ 159,896 Total assets 158,915 169,363 Total liabilities 10,026 11,260 Additional paid-in-capital 251,001 249,175 Accumulated deficit (102,126 ) (91,076 )Total stockholders' equity 148,889 158,103

Condensed Consolidated Statements of Operations and Comprehensive Loss(Unaudited)

(in thousands, except share and per share data)

Three Months Ended March 31, 2021 2020 Operating expenses: Research and development $ 6,603 $ 6,406 General and administrative 4,369 1,500 Total operating expenses 10,972 7,906 Loss from operations (10,972 ) (7,906 )Other income (expense): Interest income 63 171 Other expenses (141 ) (3 )Other income (expense), net (78 ) 168 Net loss $ (11,050 ) $ (7,738 )Other comprehensive income: Unrealized gains on available-for-sale 10 23 securitiesTotal other comprehensive income 10 23 Comprehensive loss $ (11,040 ) $ (7,715 )Net loss attributable to common $ (11,050 ) $ (7,738 )stockholders?basicand dilutedNet loss per share attributable to $ (0.47 ) $ (6.42 )commonstockholders?basic and dilutedWeighted-average common shares 23,429,507 1,205,346 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