go israelis, big ALS breakthru

[id10t]

Israeli researchers announced Tuesday that they have uncovered a key mechanism that causes the neurodegenerative disease ALS, which may unlock a way to delay or reverse the condition which affects thousands of people annually.

The team from Tel Aviv University, along with researchers from Sheba Medical Center and from institutes in Germany, France, the UK and US, found that a buildup of a protein called TDP-43 near neuromuscular junctions, which translates neural signals into motor activity, causes motor neurons to degenerate and die by inhibiting mitochondrial production.

“This consequently leads to the degeneration of the nerve and the death of motor neurons in the spinal cord, however until now we could not understand the basic biological mechanism causing the initial damage behind this vicious cascade.”

https://www.timesofisrael.com/tel-aviv-university-breakthrough-touted-as-key-to-reversing-als/

[Steve Carlton]

That’s awesome news!

[LWJ]

Wow. One of my least desired ways to die. Great news.

[stealthn]

Nice to hear some good news for a change. Now the real work begins

[beatnavy]

Originally Posted by LWJ

Wow. One of my least desired ways to die. Great news.

ALS is a really cruel way to go. College buddy died from it when he should have been in his prime.

Beating it would be awesome.

[Sooner or later]

Great news!

[vash]

i love me some smart ass people. especially Scientist..

lots of those heros lately.

[flatbutt]

My former Mrs died of ALS at 60 yo. 18 months from diagnosis to death was a mercy in my opinion but our daughter and son get very somber at this time of year.

This is wonderful news. Now we have a chance at fighting back.

[oldE]

Good news! A long time buddy was diagnosed in '05. His funeral was two years later.

One day......

Best
Les

[Bob Kontak]

From an October 2020 article:

TAR DNA-binding protein 43 (TDP-43) aggregates are frequently observed in multiple diseases, such ALS, FTD, AD, and LATE

-Amyotrophic lateral sclerosis
-Frontotemporal dementia
-Alzheimer’s disease
-Limbic predominant age-related TDP-43 encephalopathy

So the TDP-43 is not new news but what it specifically does must be new? The article I pulled the above sentence from is not written for a layman but I can't see what it specifically does. Is it the new molecule that breaks down the TDP-43 the breakthrough?

https://www.nature.com/articles/s12276-020-00513-7

[Geneman]

Bob you are right in spirit. This info has been around a long long time in the neuro-degenerative literature. The "rediscovery" of TDP protein at sites where ALS pathology occurs is slightly novel. but mostly tangential and incremental. I really hate when science journalists use the "B" word: Breakthru...in a context like this. It raises false hopes and in fact , in the end does a disservice to both scientists and patients by overpromising. Moreover in this specific case, there is virtually no evidence that a drug directed at the or reverse TDP protein could control ALS pathology or symptoms. Even if it is a "correct " target , such a drug is 20 years off.

[flatbutt]

My reading says that they have determined that it is the TDP 43 that causes the damage to the motor neurons. If so then they can investigate either preventing the TDP accumulating or figure out a way to remove it.

[Jim Bremner]

I have hope that they might cure MS.