Brain cell death in ALS, dementia tied to loss of key biochemical transport structure in nucleus

Brain cell death in ALS, dementia tied to loss of key biochemical transport structure in nucleus
(Top) The nuclear pore protein Pom121 in healthy brain cells. (Bottom) Brain cells with an ALS/dementia genetic mutation have less of the nuclear pore protein Pom121 than healthy brain cells, which leads to disrupted transport routes and ultimately cell death. Credit: Alyssa Coyne

Researchers have long sought to explain precisely how the most common genetic mutation linked to both amyotrophic lateral sclerosis (ALS) and frontotemporal dementia causes the death of nerve cells. Now, Johns Hopkins Medicine researchers report new evidence that the mutation slowly disrupts the vital transport system of proteins, enzymes and other material in and out of the cell’s control center, a process that eventually kills neurons in the brain and spinal cord.

Read more…