Open Label Extension Study of AMX0035 in Patients With ALS

The Centaur Open Label Extension Study (CENTAUR-OLE) is designed to provide longer term
access to AMX0035 for patients with ALS who participated in the CENTAUR study. The study will
assess longer term safety and therapeutic potential of AMX0035.

Inclusion Criteria:

1. Completion of all visits in the randomized, double blind AMX0035 study. Subjects that
receive tracheostomy or PAV during the course of the main study will still be followed
as ITT until the week 24 visit before enrollment in the OLE.

2. Must enroll in the OLE within 28 days of the Week 24 visit of the main study.

3. Signed informed consent to enter the open label extension phase.

Exclusion Criteria:

1. Discontinued study drug prematurely in the double-blind phase of the study for reasons
other than tracheostomy or PAV.

2. Exposure to or anticipated requirement for any disallowed medication listed below.

3. Any ongoing adverse events that in the opinion of the Site Investigator are clear
contraindications to the study drug.

4. Unstable cardiac or other life-threatening disease emergent during the randomized,
double blind study

5. Any major medical condition that in the opinion of the Site Investigator would
interfere with the study and place the subject at increased risk.