Safety and Efficacy Study of NP001 in Patients With Amyotrophic Lateral Sclerosis (ALS) and Systemic Inflammation

This is a randomized, double-blind, placebo-controlled study of NP001 in subjects with ALS
and evidence of elevated systemic inflammation. Subjects will be allocated (1:1) to NP001 and
placebo. Drug or placebo will be given intravenously.

Key Inclusion Criteria:

- Diagnosis of clinically possible, clinically probable (with or without laboratory
support), or clinically definite ALS (using the revised El Escorial Criteria)

- Forced vital capacity greater than or equal to 65% of that predicted for age and
height

- Onset of ALS-related weakness less than 3 years prior to first dose of study drug

- Plasma high sensitivity C-reactive protein (hs-CRP) concentration of greater than or
equal to 0.113 mg/dL at pre-screening/screening

- Stable dose (greater than 30 days) of riluzole if undergoing treatment with this agent

- For females: Not be of childbearing potential or agree to use adequate birth control
during the study

Key Exclusion Criteria:

- Life expectancy of less than 6 months

- Tracheotomy or be using ventilatory assistance, including Bi-level Positive Airway
Pressure (BiPAP) or Continuous Positive Airway Pressure (CPAP)

- Active pulmonary disease

- Gastrostomy

- Stem cell therapy

- Immune modulator therapy or participation in studies of other agents within 12 weeks
of pre-screening/screening

- Unstable medical condition other than ALS