Study of Ozanezumab (GSK1223249) Versus Placebo in the Treatment of Amyotrophic Lateral Sclerosis

Inclusion Criteria:

- Patients with diagnosis of familial or sporadic ALS

- Onset of muscle weakness no more than 30 months before screening visit.

- Slow Vital Capacity (SVC) of at least 65% predicted for gender, age, ethnicity and
height at Screening.

- If on riluzole, the dose must have been stable for at least 28 days prior to Baseline
visit.

- Age 18 - 80 years inclusive.

- Female subjects may participate if they are of non-child-bearing potential or if they
are of child-bearing potential they must agree to use the approved contraceptive
methods

- Aspartate aminotransferase (AST) and alanine aminotransferase (ALT) <=2x upper limit
of normal (ULN); alkaline phosphatase and bilirubin <=1.5xULN.

- QTc (both QTcB and QTcF) <450 milliseconds (msec) or <480 msec for subjects with
Bundle Branch Block at Screening and Baseline (average from triplicate ECGs).

Exclusion Criteria:

- Patients with other neuromuscular disorders (including a history of polio) which in
the opinion of the investigator could have contributed to the muscular atrophy or
weakness caused by ALS

- Patients with primary lateral sclerosis, monomelic ALS, ALS Parkinsonism dementia
complex.

- Patients requiring non-invasive or mechanical ventilation (non-invasive ventilation
for sleep apnoea is allowed subject to discussion with Medical Monitor)

- Patients on diaphragmatic pacing.

- Presence of any of the following clinical conditions: Drug abuse or alcoholism,
uncontrolled hypertension, active major infectious disease, unstable psychiatric
illness within 90 days of the Screening visit

- Subjects, who in the investigator's judgement, pose a significant suicide risk. -
Current or chronic history of liver disease, known hepatic or biliary abnormalities
(with the exception of Gilbert's syndrome or asymptomatic gallstones), positive
Hepatitis B surface antigen or Hepatitis C antibody test.

- Subjects who have participated in a clinical trial involving receipt of a
biopharmaceutical product within 6 months prior to the first dosing day.

- Exposure to non-biological experimental agents 1 month or 5 half-lives prior to
Baseline visit (whichever is longer).

- History of sensitivity to ozanezumab, or components thereof, or a history of other
allergies that, in the opinion of the investigator, contraindicates participation in
the study.