A Phase 1/2 Study of AEB1102 in Patients With Arginase I Deficiency

  • Research type

    Research Study

  • Full title

    A Phase 1/2 Open-label Study in Patients with Arginase I Deficiency to Investigate the Safety, Pharmacokinetics, and Pharmacodynamics of Intravenous AEB1102

  • IRAS ID

    236714

  • Contact name

    Suresh Vijay (Vijayaraghavan), Dr

  • Contact email

    Suresh.Vijay1@nhs.net

  • Sponsor organisation

    Aeglea BioTherapeutics, Inc.

  • Eudract number

    2017-003851-45

  • Clinicaltrials.gov Identifier

    NCT02488044

  • Clinicaltrials.gov Identifier

    127774, IND Number

  • Duration of Study in the UK

    0 years, 8 months, 0 days

  • Research summary

    The study sponsor, Aeglea BioTherapeutics, Inc., is developing AEB1102 (pegzilarginase) a recombinant human arginase enzyme to treat excess arginase levels in patients with Arginase 1 Deficiency, a urea cycle disorder caused by a mutation in the arginase 1 gene that results in the inability to degrade the amino acid arginine.

    Pegzilarginase is an engineered human arginase 1 enzyme and is intended for use as enzyme replacement therapy to reduce the elevated blood arginine levels to the normal physiological range.

    Arginase 1 Deficiency is a debilitating, progressive disease with significant morbidity and early mortality. This Phase 1/2, multicentre, single arm, open label trial is designed to evaluate the safety and tolerability of intravenous administration of pegzilarginase in patients with the deficiency. It will also evaluate the pharmacokinetic and pharmacodynamic effects of repeated doses of pegzilarginase on plasma arginine levels.

    The study will enrol approximately 10 patients, adult and paediatric, with Arginase 1 Deficiency in the United States, Canada, and Europe.
    It will be conducted in 2 parts: Part 1 (Single Ascending Dose Escalation) and Part 2 (Repeated Dosing). Each part will be preceded by a baseline assessment of arginine levels. All patients who participate in Part 1 may continue AEB1102 dosing in Part 2 if they qualify for continued dosing.

  • REC name

    Yorkshire & The Humber - Leeds East Research Ethics Committee

  • REC reference

    18/YH/0046

  • Date of REC Opinion

    12 Mar 2018

  • REC opinion

    Further Information Favourable Opinion