Novel Human Stem Cell-based Model of Amyotrophic Lateral Sclerosis Opens Doors for Rapid Drug Screening

piggy submitted, created time 1 month 2 days (www.sciencedaily.com)

Long thought of as mere bystanders, astrocytes are crucial for the survival and well-being of motor neurons, which control voluntary muscle movements. In fact, defective astrocytes can lay waste to motor neurons and are the main suspects in the muscle-wasting disease amyotrophic lateral sclerosis (ALS), commonly known as Lou Gherig's disease.

To get to the root of this complicated relationship, researchers from the Salk Institute for Biological Studies for the very first time established a human embryonic stem cell (hESC)-based system for modeling ALS

Submit comment | Blog It | Email It | Keywords: stem cells stem cell research Lou Gherig's disease ALS drug screening SOD1 motor neurons neurons ALS amyotrophic lateral sclerosis astrocytes

Neurodegenerative disease: Giving survival a boost

sea-maid submitted, created time 1 month 2 weeks (www.nature.com)

Although it is the selective death of motor neurons that ultimately causes the symptoms of amyotrophic lateral sclerosis (ALS), the disease also renders other spinal cord cells, including astrocytes, dysfunctional. Maragakis and colleagues have now shown that the replacement of damaged astrocytes through precursor cell transplantation might be a useful therapeutic strategy for ALS.

The authors transplanted glial restricted precursors (GRPs) into the grey matter of the spinal cord in a transgenic rat model of ALS

1 comment | Blog It | Email It | Keywords: neurons neuroscience astrocytes Lou Gherig's disease motor neurone disease ALS transplants amyotrophic lateral sclerosis

Gene silencing made practical: siRNA used to treat neurogenerative disorders in the lab

Darkfrog submitted, created time 7 months 1 day (www.jbc.org)

This abstract is about as easy to read as sixteenth-century tax law, but here's the gist: Human neurogenerative disorders, like Huntington's, Parkinson's, Alzheimer's and amyotrophic lateral sclerosis (Lou Gherig's disease) are all caused or exacerbated by the expression of mutant genes. If we switch off the genes--or at least keep them from producing proteins--then the patients might get better. One way to switch off a gene is to inject the cell with a siRNA (short interfering RNA) that complements the gene's product mRNA. This is called gene silencing

2 comments | Blog It | Email It | Keywords: gene silencing Huntingtons disease Alzheimers disease Parkinsons alzheimers huntingtons amyotrophic lateral sclerosis small interfering RNA (siRNA) lou gherig's disease ALS

New Gene Nailed for ALS

Eric wu submitted, created time 10 months 1 week (sciencenow.sciencemag.org)

Even with the entire human genome in hand, scientists can still have trouble rooting out the genes behind a disease. Consider amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease. One ALS-related gene mutation was found in 1993, but it affects only 1% of all ALS patients. Now, after a long dry spell, researchers have finally hit on a second. It's just as rare as the first, but it seems to be more closely related to aspects of ALS pathology found in all patients.

Submit comment | Blog It | Email It | Keywords: disease genes human genome genes amyotrophic lateral sclerosis ALS Lou Gherig's disease ALS

Mutation in one gene tied to Lou Gehrig's disease

Eric wu submitted, created time 1 year 3 weeks (www.reuters.com)

A mutation in a single gene may raise one's risk of getting amyotrophic lateral sclerosis (ALS), or Lou Gehrig's disease, by as much as 30 percent, offering a potential new target for drug research, Dutch scientists said on Sunday.

Submit comment | Blog It | Email It | Keywords: Lou Gherig's Disease ALS genetic research mutations amyotrophic lateral sclerosis