Exhibit 99.1

Sio Gene Therapies Announces Fiscal Year 2020 Year-End Financial Results and Expected Fiscal Year 2021 Key Milestones

Multiple GM1 gangliosidosis program milestones expected in FY2021, including 12-month data updates from the ongoing dose-escalation study and meeting with FDA to align on registrational pathway

Strong cash position with $119 million of cash and cash equivalents as of March 31, 2021, and cash runway expected into Q4 2022

NEW YORK, NY AND RESEARCH TRIANGLE PARK, NC, June 9, 2021 (GLOBE NEWSWIRE) – Sio Gene Therapies Inc. (NASDAQ: SIOX), a clinical-stage company focused on developing gene therapies to radically transform the lives of patients with neurodegenerative diseases, today provided financial results for its fiscal year ended March 31, 2021.

“At Sio we remain committed to bringing urgently needed treatments to patients with rare pediatric disorders and adult neurodegenerative diseases. We are grateful for the steadfast dedication of patients, caregivers, investigators, and patient advocacy groups who have supported our mission in the midst of a global pandemic, and we are proud of our team’s efforts in advancing the development of Sio’s three clinical-stage product candidates through an extraordinary year,” said Pavan Cheruvu, M.D., Chief Executive Officer of Sio Gene Therapies.

“The positive data recently shared from our Phase 1/2 study of AXO-AAV-GM1, together with our industry-leading position in clinical enrollment and investment in earlier diagnosis, give us renewed momentum as we aim to advance this investigational therapy towards commercialization. In the second half of 2021, we will present 12-month follow-up data on safety, biomarkers, and clinical efficacy from the low-dose cohort of the GM1 gangliosidosis program. We expect these data, as well as a planned regulatory meeting with the FDA expected in the first half of 2022, will help define our registrational pathway in GM1 gangliosidosis. We have confidence in AXO-AAV-GM1 as well as our two other clinical-stage candidates in Tay-Sachs/Sandhoff disease and Parkinson’s disease, and look forward to several major milestones across our three programs during this fiscal year.”

Dr. Cheruvu continued, “In parallel, we are continuing to build our preclinical and analytical development capabilities in our growing laboratory in Research Triangle Park, and are conducting a search and evaluation process for promising genetic medicines that may expand our pipeline with new product candidates in the years ahead. Our strengthened balance sheet leaves us well-positioned to develop first-in-class and best-in-class gene therapy product candidates in our current pipeline, while also investing in exciting new, biology-driven opportunities in areas of significant unmet patient need.”

Key Highlights and Development Updates

AXO-AAV-GM1 gene therapy for GM1 gangliosidosis
Reductions in CSF GM1 ganglioside were observed in 4 out of 5 children treated with the lowest dose of AXO-AAV-GM1 at 6-months follow up, providing the first clear evidence of a biochemical effect in the CNS following intravenous delivery (data presented at the American Society of Cell and Gene Therapy conference in May 2021)
Announced six-month follow-up data from 5 patients in the low-dose cohort (1.5×1013 vg/kg) of AXO-AAV-GM1, demonstrating that the investigational gene therapy had a favorable safety profile with evidence of clinical disease stability and biomarker improvement
Based on the favorable safety profile at the low dose, the independent Data Safety Monitoring Committee (DSMC) concurred with progressing to the high-dose cohort (4.5×1013 vg/kg). Thus far two patients have received the high dose without complications.
Upcoming milestones:
12-month topline safety, biomarker, and efficacy data from the low-dose cohort in the second half of 2021
12-month topline data from the first two patients dosed in the high-dose cohort (4.5×1013 vg/kg) in Q1 2022
Meeting with the U.S. Food and Drug Administration (FDA) to discuss the registrational pathway for AXO-AAV-GM1 in the first half of 2022


The following information was filed by Sio Gene Therapies Inc. (SIOX) on Wednesday, June 9, 2021 as an 8K 2.02 statement, which is an earnings press release pertaining to results of operations and financial condition. It may be helpful to assess the quality of management by comparing the information in the press release to the information in the accompanying 10-K Annual Report statement of earnings and operation as management may choose to highlight particular information in the press release.

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