Neurodegenerative disease: Giving survival a boost

sea-maid submitted, created time 3 days 21 hours (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

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Induced stem cell lines may soon be available from Harvard

Darkfrog submitted, created time 3 months 2 weeks (sciencenow.sciencemag.org)

A few weeks ago, we talked about how researchers had been able to take a cell from an ALS patient and develop a working, research-quality pluripotent cell line. Well the next step has been taken.

I've been saying that induced pluripotent stem cells might become the preferred research model (over embryonic stem cells), but only if they became easier to obtain than embryonic stem cells. It looks as though that might happen soon. The Harvard Stem Cell Institute is dedicating an iPS core lab

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

Darkfrog submitted, created time 5 months 2 weeks (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

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New Gene Nailed for ALS

Eric wu submitted, created time 8 months 3 weeks (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.

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Mutation in one gene tied to Lou Gehrig's disease

Eric wu submitted, created time 11 months 6 days (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.

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http://content.nejm.org/cgi/content/short/357/8/775

jiangyun submitted, created time 1 year 2 months (content.nejm.org)

Approximately 90% of persons with amyotrophic lateral sclerosis (ALS) have the sporadic form, which may be caused by the interaction of multiple environmental factors and previously unknown genes.

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Mutation of SOD1 in ALS: a Gain of a Loss of Function

cappuccion submitted, created time 1 year 6 months (hmg.oxfordjournals.org)

Amyotrophic lateral sclerosis is a neurodegenerative disease caused by motoneuron loss. Some familial cases (fALS) are linked to mutations of Superoxide-Dismutase type-1 (SOD1), an antioxidant enzyme whose activity is preserved in most mutant forms. Due to the similarities in sporadic and fALS forms, mutant SOD1 animal and cellular models are a useful tool to study the disease. In transgenic mice expressing either wild type (wt) human SOD1 or mutant G93A-SOD1, we found that wtSOD1 was present in cytoplasm and in nuclei of motoneurons, while mutant SOD1 was mainly cytoplasmic.

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Stem cell study zeroes in on ALS cause

alpha submitted, created time 1 year 7 months (www.reuters.com)

"Cells that are supposed to nourish and support other nerve cells instead secrete the poisons that cause amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, researchers reported on Sunday."

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