Lou Gehrig's Breakthrough: Protein trash not being taken out. Update.

"Nature" reports that all three types of ALS (Lou Gehrig's Disease) have a malfunctioning janitor protein called ubiquilin2.  Its job is to recycle damaged or misfolded protein in the brain and spinal cord but in ALS patients, it is found embedded in tangled skeins of off-grade protein. These yarn-like skeins gradually disable the neurons they cling to.  Recent research has focused on which neurons were failing and identified about ten genes predictive of the disease. An exciting part of the news is the implication for Alzheimer's and Parkinson's.  These diseases also show protein clumping at nerves.  The same line of study may help fix all three conditions.

The lead author, Han-Xiang Deng summaries the results:  “This study provides robust evidence showing a defect in the protein degradation pathway causes neurodegenerative disease.”

h/t Instapundit linking to Neuro Science News which refers to the original article in the August issue of Nature.

UPDATE   Confirming this report, a second lazy janitor has been found in the same pathway, a gene named Sequestosome1.

This screen clip of the Nature abstract shows why much scientific reporting needs to be rewritten so it is useful not just to insiders: