Inclusion Criteria:
Diagnosis of familial or sporadic ALS ≤ 24 months prior to screening
Upright Slow Vital Capacity (SVC) ≥ 65% of predicted for age, height and sex at screening
Able to swallow tablets
A caregiver (if one is needed)
Able to perform reproducible pulmonary function tests
Pre-study clinical laboratory findings within the normal range or, if outside the normal range, deemed not clinically significant by the Investigator
Male patients who have not had a vasectomy and confirmed zero sperm count must agree to either use acceptable methods of contraception or abstain from sex
Female patients must be post-menopausal or sterilized or must not be breastfeeding, have a negative pregnancy test, have no intention to become pregnant during the study and use acceptable methods of contraception
Patients must be either on riluzole for at least 30 days prior to screening or have not taken riluzole for at least 30 days prior to screening and not planning to start riluzole during the course of the study.
Exclusion Criteria:
At the time of screening, any use of non-invasive ventilation (NIV), e.g. continuous positive airway pressure [CPAP], noninvasive bi-level positive airway pressure [NPPV] or noninvasive volume ventilation [NVV]for any portion of the day, or mechanical ventilation via tracheostomy, or on any form of oxygen supplementation
Neurological impairment due to a condition other than ALS
Presence at screening of any medically significant cardiac, pulmonary, GI, musculoskeletal, or psychiatric illness that might interfere with the patient's ability to comply with study procedures or that might confound the interpretation of clinical safety or efficacy data
Has taken any investigational study drug within 30 days or five half-lives of the prior agent, whichever is longer, prior to dosing
Known to have received CK-2127107 or tirasemtiv in any previous clinical trial
Has received or is considering receiving during the course of the study any form of stem cell therapy for the treatment of ALS
Has received or is considering receiving during the course of the study any form of gene therapy for the treatment of ALS
Has received or is considering obtaining during the course of the study a diaphragmatic pacing system
History of substance abuse within the past 2 years
Use of certain medications
