The molecular mechanisms of TDP-43 aggregate formation is not well understood; TDP-43 contains an N-terminal oligomerization domain and an intrinsically 

2020. 8. 15. · Since its discovery as a primary component in cytoplasmic aggregates in post-mortem tissue of patients with Amyotrophic Lateral Sclerosis (ALS), TAR DNA Binding Protein 43 kDa (TDP-43) has remained a central focus to understand the disease. TDP-43 links both familial and sporadic forms of ALS as mutations are causative for disease and cytoplasmic aggregates

Key words amyotrophic lateral sclerosis, TDP-43. Accepted for publication 16 November 2012. Correspondence. Osamu Onodera, Department of Molecular.

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disorder involving loss of upper and lower motor neurons, with most cases ending in death within 3-5 years of onset. Several molecular and cellular pathways have been identified to cause ALS; however, treatments to stop or reverse disease progression are yet to be found. Riluzole, a neuroprotective agent offering

Mutations in the gene encoding the TAR DNA-binding protein 43 (TDP-43) are a well-recognized genetic cause of ALS, and an imbalance in energy 

TDP-43 was identified as a primary component of ubiquitin-positive cytosolic inclusion bodies seen in remnant motor neurons in both sporadic and familial ALS ( 

FIGURE 5 | Liquid-liquid phase separation (LLPS) and liquid-solid phase separation (LSPS) of TDP-43. (A) Proteins containing low complexity/prion-like domains undergo phase-separation into membrane-less, spherical compartments, often aided by the presence of salt, pH changes or temperature changes. Persistent stress, mutations and droplet-aging, might induce irreversible

Amyotrophic lateral sclerosis (ALS) is a fatal neurological disease that is defined by the progressive degradation of both upper and lower motor neurons. The disease ultimately results in paralysis and death between three and five years after it is diagnosed. In spite of the fact that ALS is primarily a disease of the motor neurons, nearly half of ALS patients show cognitive or behavioral

2014. 12. 9. · Amyotrophic lateral sclerosis These mechanisms could be a plausible molecular basis for this disease continuum and, This misfolded conformation then recruits native monomers of TDP-43 to induce pathological misfolding on to the native form in what is known as ‘templated conformational change’.

2018. 11. 16. · Aggregates of the TAR DNA binding protein 43 (TDP-43), are hallmark features of the neurodegenerative diseases Amyotrophic Lateral Sclerosis (ALS) and frontotemporal dementia (FTD), with overlapping clinical, genetic and pathological features. TDP-43 and Neurodegeneration: From Bench to Bedside summarizes new findings in TDP-43 pathobiology

Summary: TDP-43 pathology is a disease hallmark that characterizes both amyotrophic lateral sclerosis (ALS) and frontotemporal lobar