• Diagnosis of ALS; the diagnosis of ALS defined by revised El Escorial criteria as follows:

∙ Evidence of lower motor neuron (LMN) degeneration by clinical, electrophysiological, or neuropathological examination.

‣ Evidence of upper motor neuron (UMN) degeneration by clinical examination.

‣ Progressive spread of symptoms or signs within a region or to other regions, as determined by clinical examination or the history of disease progression.

‣ Absence of electrophysiological, neuroimaging, or pathological evidence of other diseases that might explain the UMN or LMN degeneration and exclusion of other causes.

• Male or female subjects aged 18 to 75 years inclusive.

• Must provide written informed consent for study-related procedures.

• Must be capable of completing all study-related procedures, assessments, and visits in the judgment of Investigator.

• Disease duration from ALS symptom onset of motor weakness ≤24 months.

• ALSFRS-R total score ≥38 at screening visit.

• ALSFRS-R Breathing subscore should be ≥9 at the time of screening.

• ALSFRS-R Bulbar subscore should be ≥9 at the time of screening.

• Forced vital capacity \>70% of predicted value.

⁃ PIFR ≥100 L/minute.

⁃ Must be receiving a stable dose of standard-of-care treatment Riluzole for 4-weeks before signing informed consent.

⁃ Female subjects who are of childbearing potential must agree to use of highly effective methods of contraception consistent with local regulations during the study, and for 3 months after the study drug administration. Examples include the following, but not limited to:

• Combined (estrogen and progestogen containing) or progestogen-only hormonal contraceptives;

∙ Intrauterine device or intrauterine hormone-releasing system; OR

∙ Post-menopausal status must have experienced their last menstrual period minimum of 1 year prior to study drug administration; OR

∙ Surgically sterilized. Female subject should be willing to not donate egg during the trial and for 3 months after the last dose of the study drug.

⁃ Male subjects who are sexually active with a female of childbearing potential must agree to use highly effective contraception as described above, or a combination of 2 acceptable methods of contraception (e.g., a barrier method along with a female partner using a hormonal contraceptive method), in accordance with local regulations, throughout the duration of the study, and for 3 months after the last dose of the study drug.

⁃ (Male subject should be willing to not donate sperm during the trial and for 3 months after the last dose of the study drug.)