TDP 43 News and Research

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The Complex Link Between DNA Modification and ALS

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease which remains incurable. The disease is characterized by the selective degeneration of upper motor neurons in the motor cortex as well as the lower motor neurons in the brainstem and spinal cord. The cause of ALS remains unknown in 90 percent of cases, which are referred to as sporadic ALS because there is no family history for the disease. Cumulative evidence suggests that sporadic ALS may result from complex interactions between genetic susceptibility and aging.

23 Aug 2024

New Biomarker Offers Early Detection of ALS and FTD

Two progressively degenerative diseases, amyotrophic lateral sclerosis (ALS, commonly known as Lou Gehrig's disease) and frontotemporal dementia (FTD, recently in the news with the diagnoses of actor Bruce Willis and talk show host Wendy Williams), are linked by more than the fact that they both damage nerve cells critical to normal functioning -; the former affecting nerves in the brain and spinal cord leading to loss of movement, the latter eroding the brain regions controlling personality, behavior and language.

19 Mar 2024

Breaking Down the Complex Mechanisms of Neurodegeneration

By using a novel neuronal cell culture model, researchers at the University of Zurich have illuminated the complex mechanisms behind neurodegeneration.

23 Feb 2024

Misfolded Proteins: How They Damage Neurons and Cause Neurodegeneration

Neurodegenerative diseases are characterized by the deposition of clumped proteins in the brain and progressive neuronal cell death.

5 Dec 2023

Abnormal Immune Activity in Astrocytes Causes Dementia-Related Cognitive Deficits

People with dementia have protein build-up in astrocytes that may trigger abnormal antiviral activity and memory loss, according to a preclinical study by a team of Weill Cornell Medicine investigators.

21 Apr 2023

DNA drugs help restore the normal processing of protein-encoding RNA

A protein named TDP-43 has been lost from its normal location in the nucleus of the cell in virtually all persons having amyotrophic lateral sclerosis (ALS) and in up to half of all cases of frontotemporal dementia and Alzheimer’s disease (AD).

From UC San Diego 20 Mar 2023

Delaying paralysis in ALS by using designer DNA drug

In virtually all persons with amyotrophic lateral sclerosis (ALS) and in up to half of all cases of Alzheimer's disease (AD) and frontotemporal dementia, a protein called TDP-43 is lost from its normal location in the nucleus of the cell.

20 Mar 2023

RNA modification plays a vital role in TDP-43-related neurodegeneration in ALS

Nearly 100% of cases of amyotrophic lateral sclerosis-the progressive, fatal neurodegenerative disease known as ALS or Lou Gherig's disease-involve the buildup of a protein called TDP-43.

28 Feb 2023

Scientists discover two ways to preserve diseased upper motor neurons in ALS

Northwestern Medicine scientists have discovered two ways to preserve diseased upper motor neurons that would normally be destroyed in ALS, based on a study in mice. Upper motor neurons initiate movement, and they degenerate in ALS.

24 Feb 2023

Potential approach for mitigating ALS pathogenesis

Every year, 5,000 people in the United States are diagnosed with ALS, an incurable neurodegenerative disease that will destroy them within two to five years.

8 Feb 2023

Little-known protein may play a role in frontotemporal lobar degeneration

Scientists have known for years that amyloid fibrils -; fibrous, ropelike structures formed by closely linked protein molecules -; are present in the brains of patients with Alzheimer's and Parkinson's diseases and likely play a role in the progression of these disorders.

21 Apr 2022

Simple lab model helps investigate the properties of muscle cells from sIBM patients

A team of researchers at Tohoku University have developed a simple lab-based system for growing human muscle cells that are capable of vigorously contracting.

3 Mar 2022

Novel molecular pathway shared by ALS and frontotemporal dementia identified

Scientists from two independent research teams have discovered how the mislocalization of a protein, known as TDP-43, alters the genetic instructions for UNC13A, providing a possible therapeutic target that could also have implications in treating amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD), and other forms of dementia.

1 Mar 2022