The purpose of this study is to assess the effect of CK-2127107 versus placebo on respiratory
function and other measures of skeletal muscle function in patients with ALS.

The COMMEND Study will assess the safety and effectiveness of FLX-787 in men and women with
Motor Neuron Disease [including Amyotrophic Lateral Sclerosis (ALS), Primary Lateral
Sclerosis (PLS) or Progressive Muscular Atrophy (PMA)] experiencing muscle cramps.
Participants will be asked to take two study products during the course of the study. One of
these study products will be a placebo.

Approximately 120 participants in approximately 30 study centers across the United States are
expected to take part. Participants will be in the study for approximately 3 months and visit
the study clinic 3 times.

The purpose of this study is to determine the muscle strength of a muscle in the thigh after
12 weeks of home exercise.

This research study is being done to find out if tocilizumab, also known as Actemra™, can
help with Amyotrophic Lateral Sclerosis (ALS). The investigators also want to find out if
tocilizumab is safe to take without causing too many side effects.

Currently ALS has no cure and only 1 modestly effective treatment to slow the progression of
the disease. Although not the initial cause of ALS, the immune system plays a role in the
death of motor neurons. The immune cells that participate in this process are stimulated by
a substance called interleukin-6 (IL-6) whose effect is blocked by tocilizumab and thus, may
slow the death of motor neurons and slow the disease.

The goals of this study are: (1) to better understand the relationship between the phenotype
and genotype of amyotrophic lateral sclerosis (ALS) and related diseases, including primary
lateral sclerosis (PLS), hereditary spastic paraplegia (HSP), progressive muscular atrophy
(PMA), and frontotemporal dementia (FTD); and (2) to develop biomarkers that might be useful
in aiding therapy development for this group of disorders.