New ALS Biomarkers Potential New Drug Targets

Increasing evidence shows that neuroinflammation mediating; by activating glia and infiltrating immune cells is involving in potential biomarkers the pathogenesis of sporadic amyotrophic lateral sclerosis. The disease has what is knowing a focal onset; where paralysis starts with an arm or a leg and spreads throughout the body as motor neurons in the spinal cord and brain die off.

Amyotrophic lateral sclerosis, or ALS, is an adult-onset neurodegenerative disease that causes paralysis and ultimately death when the nerves enervating the lungs cease to carry the signals needed for breathing. Early diagnosis of the disease has not been possible because of a lack of known biomarkers indicative of ALS; but scientists believe that cellular changes within spinal neurons occur before symptoms are detectable; and these changes could serve as useful biomarkers that can aid in earlier diagnosis.

Potential biomarkers

When symptoms of ALS begin with the paralysis of an arm or a leg, it means; that the disease has affected the motor neurons; that enervate that arm or leg and which originate in a specific region along the spinal cord. For example, neurons that innervate the arm originate in the upper part of the spinal cord.

In ALS, where symptoms first appear in the arm, the motor neurons in the upper spine region die off. Motor neurons above and below that region begin to die off next as the disease spreads up and down the spine; causing paralysis of other parts of the body. Patients with focal-onset ALS had different types of neurons in areas of the spinal cord that are less affecting by the disease comparing with patients without neurological disorders.

Effective treatment

The debilitating disease has no effective treatment to stop the disease progression and there are only two medications that can prolong patient survival by a few months. So, new drug targets; especially ones that could be given in the earlier stages of the disease, are very much needed.

They also finding that spinal neurons in less affecting regions of patients; who died of focal-onset ALS were associati9ng with cells called microglia and astrocytes. Since there must be cellular changes occurring in spinal cord regions adjacent to areas; where the disease has clearly affected motor neurons in the spine; we wanted to look at neurons from these adjacent areas to determine if they are different from healthy tissue.