Aeglea Biotherapeutics, Inc. (AGLE) SEC Filing 10-K Annual report for the fiscal year ending Monday, December 31, 2018

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Completed dosing of Phase 1/2 trial in February 2019, with 14 out of 16 enrolled patients completing eight weeks of repeat dosing. Clinical data from this trial, which informed the design of the Phase 3 PEACE (Pegzilarginase Effect on Arginase 1 Deficiency Clinical Endpoints) trial protocol design, included the demonstration that pegzilarginase is effective in reducing plasma arginine levels with accompanying improvements in important disease-related abnormalities. Insights from standardized clinical assessments and feedback from physicians and caregivers also established the value of mobility and adaptive behavior assessments in capturing the clinical benefits of pegzilarginase. Additionally, pegzilarginase appeared safe and well tolerated. Aeglea expects to report Phase 1/2 data at an oral presentation at the Society for Inherited Metabolic Disorders (SIMD) annual meeting being held April 6-9 in Seattle, Washington.

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In December 2018, the Company announced the design of its single, global pivotal Phase 3 PEACE trial based on input from the U.S. Food and Drug Administration (FDA) and the European Medicine Agency (EMA). Pegzilarginase is believed to be the first-ever investigative therapy for patients with ARG1-D that addresses the high arginine levels that are the key drivers of this devastating disease. PEACE is a global, randomized, double-blind, placebo-controlled trial designed to assess the effects of treatment with pegzilarginase versus placebo over 24 weeks with a primary endpoint of plasma arginine reduction. Secondary endpoints will include mobility and adaptive behavior as assessments of clinically meaningful effects, in addition to safety and pharmacokinetics. Aeglea expects to dose the first patient in the PEACE trial in the second quarter of 2019 and anticipates topline data will be available in the first quarter of 2021.

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Pediatric review voucher (PRV) eligible given receipt of rare pediatric disease designation from FDA for treatment of ARG1-D.

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Completed the Phase 1b safety evaluation and dose selection for pegzilarginase in combination with KEYTRUDA® (pembrolizumab), Merck’s anti-PD-1 therapy. This trial is designed to assess the safety and efficacy of pegzilarginase with KEYTRUDA in patients with extensive disease small cell lung cancer (SCLC) who have relapsed or progressive disease after platinum-based chemotherapy.

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Initiated enrollment in the open label Phase 2 study of pegzilarginase in combination with KEYTRUDA in December 2018. This trial is designed to assess the safety and efficacy of pegzilarginase with KEYTRUDA in