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May 17, 2007
Loved one DX
Blood-Spinal Cord Barrier Breaks Early in ALS- April 8, 2008
A research team that included MDA grantees M. Kerry O’Banion at the University of Rochester (N.Y.) Medical Center and Severine Boillee at the University of California-San Diego has found that leaks in the blood vessels of the spinal cord precede damage to the nervous system in ALS (amyotrophic lateral sclerosis, or Lou Gehrig’s disease) in mice with a genetic form of this paralyzing disorder.

Berislav Zlokovic at the University of Rochester and colleagues, who published their findings in the April issue of Nature Neuroscience, found that mice with mutated SOD1 genes, one of the known causes of the familial (inherited) form of ALS, had disruptions in a barrier between the spinal cord and the bloodstream that normally protects the central nervous system from toxins and injury.

In these mice, the blood-spinal cord barrier was disrupted because of reduced levels of three of the so-called “tight junction” proteins that normally keep it together, the investigators say.

The disruptions resulted in inflammation, as well as reductions in blood flow and, in some areas, small hemorrhages with release of toxic products, the researchers say.

They say their study demonstrates that an increase in the permeability of the spinal-cord blood vessels probably makes an important contribution to degeneration of the muscle-controlling nerve cells (motor neurons), which is the hallmark of ALS.

They also note that damage to these blood vessels is among the earliest events in a “toxic cascade” set in motion by SOD1 gene mutations. (Research published last year showed damage to these blood vessels in mice with SOD1-related ALS, but the investigators couldn’t determine whether the damage came before or after damage to nerve cells.)

“Genetic or pharmacologic interventions targeted specifically to the endothelium [lining of blood vessels] will help to determine both the causality between the blood-spinal cord barrier leakage and motor neuron degeneration and how such damage may delay disease onset and/or progression,” the researchers note. (The University of Rochester Medical Center says the team is currently testing a compound that may help seal leaky blood vessels.)

A caveat is that these experiments were conducted in mice with SOD1-related ALS. Their meaning for ALS resulting from other causes isn’t known.

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