A Study of AEB1102 (Pegzilarginase) in Patients With Arginase I Deficiency

Inclusion Criteria:

1. Complete treatment in Study CAEB1102-101A without experiencing any clinically
significant adverse event or other unmanageable drug toxicity that would preclude
continued dosing

2. Confirmation by the Investigator and the Sponsor determine that it is acceptable for
the patient to continue dosing with AEB1102

3. If female and of child-bearing potential, has a negative serum pregnancy test within 7
days before enrollment

4. If sexually active (male or female), must be surgically sterile, post-menopausal
(female), or must agree to use a physician-approved method of birth control during the
study and for a minimum of 30 days after the last study drug administration

5. Patient or legal guardian is able and willing to provide written informed consent and
where required assent, and to comply with all requirements of study participation
(including all study procedures and continuation of prescribed diet without
modification), prior to any screening procedures

Exclusion Criteria:

1. Clinically significant concurrent disease, serious intercurrent illness, or other
extenuating circumstances