Research Unveils a Most Valuable Player in Lou Gehrig's Disease

A look into cells known as oligodendrocytes provides insight into how amyotrophic lateral sclerosis (ALS) develops.

Researchers at the Solomon H. Snyder Department of Neuroscience at the Johns Hopkins University School of Medicine have discovered how NG2+ cells (immature cells that eventually morph into mature nervous-system cells known as oligodendrocytes or oligos) proliferate in ALS patients.

These NG2+ cells (which normally divide in adult tissues at a steady pace, sometimes replacing themselves and other times forming new oligos) undergo explosive division and transform into abnormal-appearing oligodendrocytes, then quickly die in mice models with the gene that causes ALS.

Dwight Bergles, Ph.D., associate professor in the Department of Neuroscience at Johns Hopkins School of Medicine said the brakes that normally hold these cells in check look to be non-existent in ALS. He explained that oligodendrocytes die before anything else degenerates, "which identifies a new and important player in the progression of this disease."

The researchers said this new observation about the overgrowth of NG2+ cells "could lead to an entirely new understanding of ALS."

ALS, or Lou Gehrig's disease, is a progressive, incurable neurodegenerative disease characterized by muscle weakness and atrophy caused by the death of motor neurons. The relatively rare disease usually strikes those over age 50 and results in paralysis and finally death, usually within five years of diagnosis.