TDP-43 protein aggregates

The National Institutes of Health (NIH) has awarded a $3.3 million grant to researchers at two universities to advance studies of TDP-43, a protein that builds to form toxic clumps in people with amyotrophic lateral sclerosis (ALS) and Alzheimer’s disease. Their goal is to better understand how…

The clear, colorless liquid that fills and surrounds the brain and spinal cord may spread toxic protein aggregates from one nerve cell to others among people with amyotrophic lateral sclerosis (ALS), a study in mice suggests. When repeatedly infused with the cerebrospinal fluid (CSF) from ALS patients, animals…

A newly discovered self-destructive mechanism in mitochondria, the cells’  powerhouses, may be one of the first deficits leading to motor neuron degeneration associated with toxic TDP-43 clumps — a hallmark of amyotrophic lateral sclerosis (ALS). That evidence from a preclinical study suggests available therapies against mitochondrial degeneration might help halt neurodegeneration…

A new study identified a group of sporadic amyotrophic lateral sclerosis (ALS) patients whose disease may arise from a group of “jumping genes” unleashed when TDP-43 protein accumulates in clumps in the brain and spinal cord. These “jumping genes” — which can randomly hop from one location on…

Biopharma AC Immune has entered a research collaboration with scientists at the Perelman School of Medicine at the University of Pennsylvania (UPenn) to uncover why TDP-43 misshapes and accumulates in the nervous system of some people, like those with amyotrophic lateral sclerosis (ALS).

High glucose levels might compensate for the toxic effects of an accumulation of TDP-43 — a widely expressed nuclear protein that binds both DNA and RNA — and protect neurons in amyotrophic lateral sclerosis (ALS), a study in a fly model of the disease suggests. The study,…

While TDP-43 aggregates are associated with amyotrophic lateral sclerosis (ALS) and other neurodegenerative diseases, researchers have found that these protein clumps are also involved in the regeneration of  healthy skeletal muscle. This finding — that abnormal TDP-43 aggregates temporarily form to repair damaged muscle — may open new lines of research into…

An exploratory anti-cancer therapy may halt the characteristic toxic accumulation of TDP-43 in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), researchers suggest. The preclinical study, “Poly(ADP-Ribose) Prevents Pathological Phase Separation of TDP-43 by Promoting Liquid Demixing and Stress Granule Localization,” was published in the journal…

Researchers have discovered a natural mechanism that can help prevent the formation of harmful protein clumps that contribute to the development of amyotrophic lateral sclerosis (ALS), according to results of a new study. They also gained new insights into the formation of the protein clumps known as TDP-43 protein aggregates that…

Suppressing the expression of a protein called ataxin-2 may prolong survival in patients with amyotrophic lateral sclerosis (ALS), according to a recent mouse study. The study, “Therapeutic Reduction Of Ataxin-2 Extends Lifespan And Reduces Pathology In TDP-43 Mice,” was published in the journal Nature. According to researchers, nearly…