ALS tagged posts

A soft, gel-like substance called myelin envelopes the axons of neurons to shield them from damage. In ALS, the protective sheath is gradually stripped off, leaving axons bare, exposed and vulnerable to damage. Harvard Medical School researchers have identified a key instigator of this process.

Scientists have identified a key instigator of nerve cell damage in people with amyotrophic lateral sclerosis, or ALS. Researchers say the findings of their study may lead to new therapies to halt the progression of the uniformly fatal disease that affects more than 30,000 Americans. One such treatment is already under development for testing in humans after the current study showed it stopped nerve cell damage in mice with ALS.

The HMS study reveals that the aberrant behavior of an enzyme called RI...

The dentate gyrus of the mouse hippocampal formation which contributes to the formation of new episodic memories stained for neurons (green) and stem cells (red). Credit: Andrew L Bashford and Vasanta Subramanian University of Bath

Scientists identify 2 regions of mouse brains where C9orf72 is expressed. For the first time novel expression sites in the brain have been identified for a gene which is associated with Motor Neuron Disease and Frontotemporal Dementia. Many people who develop Motor Neuron Disease, also called Amyotropic Lateral Sclerosis (ALS), and/or Frontotemporal Dementia (FTD) have abnormal repeats of nucleotides within a gene called C9orf72 which causes neurons to die.

A team from the Department of Biology & Biochemistry at the University of Bath discovered for the first ti...

Skeletal myotubes grown for three weeks on gelatin hydrogel. Credit: Archana Bettadapur, Gio Suh, Evelyn Wang, Holly Huber, Alyssa Viscio and Megan McCain

Muscles-on-a-chip could be used to study muscle development and disease, as well as provide a relevant testing ground for new potential drugs. During normal embryonic development, skeletal muscles form when myoblasts fuse to form muscle fibers, myotubes. In past experiments, mouse myotubes have detached or delaminated from protein-coated plastic scaffolds after approximately one week and failed to thrive.

In this experiment, the researchers fabricated a gel scaffold from gelatin, a derivative of the naturally occurring muscle protein collagen, and achieved much better results...

Copper, zinc superoxide dismutase is essential to life, but when damaged can become toxic. Credit: Photo courtesy of Oregon State University

OSU researchers have announced that they have essentially stopped the progression of amyotrophic lateral sclerosis (ALS), or Lou Gehrig’s disease, for nearly 2 years in one type of mouse model used to study the disease – allowing the mice to approach their normal lifespan. In decades of work, no treatment can do anything but prolong human survival less than a month in ALS. This mouse model is one that may more closely resemble the human reaction to this treatment, using copper-ATSM. Researchers are moving as quickly as possible toward human clinical trials, testing first for safety and then efficacy.

ALS is known to be caused by the death and deterior...