By isolating cells from patients’ spinal tissue within a few days after death, researchers funded by the National Institutes of Health have developed a new model of the paralyzing disease amyotrophic lateral sclerosis (ALS). They found that during the disease, cells called astrocytes become toxic to nerve cells — a result previously found in animal models but not in humans. The new model could be used to investigate many more questions about ALS, also known as Lou Gehrig’s disease.
Spinal muscular atrophy legislation, “The SMA Treatment Acceleration Act”, will soon be introduced in both the U.S House of Representatives and the U.S. Senate.
What is spinal muscular atrophy?
Spinal muscular atrophy (SMA) is an autosomal recessive (meaning the disorder is inherited by receiving one gene from both mother and father) neuromuscluar disorder that affects motor neurons of the spinal cord and brainstem. Motor neurons are responsible for supplying electrical and chemical messages to muscle cells. Without proper input from motor neurons, muscle cells don’t function properly and become much smaller (atrophy), producing symptoms of muscle weakness and affecting the ability to swallow, breath and move limbs.