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 …

Amyotrophic lateral sclerosis (ALS) is characterized by the degeneration of upper and lower
motor neurons (MNs) leading to paralysis and, ultimately, death by respiratory failure 3–5 …

Cytoplasmic accumulation of TDP-43 is a disease hallmark for many cases of amyotrophic
lateral sclerosis (ALS), associated with a neuroinflammatory cytokine profile related to …

Mutations in or dys-regulation of the TDP-43 gene have been associated with TDP-43
proteinopathy, a spectrum of neurodegenerative diseases including Frontotemporal Lobar …

Genetic mutations in TAR DNA-binding protein 43 (TDP-43) cause amyotrophic lateral
sclerosis (ALS) and frontotemporal dementia (FTD). Importantly, TDP-43 proteinopathy …

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease
characterized by selective, early degeneration of motor neurons in the brain and spinal cord …

Mitochondrial defects result in dysregulation of metabolomics and energy homeostasis that
are detected in upper motor neurons (UMNs) with TDP-43 pathology, a pathology that is …

The disturbance in mitochondrial functions and homeostasis are the major features of
neuron degenerative conditions, like Parkinson's disease, Amyotrophic Lateral Sclerosis …

Background and Purpose Amyotrophic lateral sclerosis (ALS), a neurodegenerative disease
characterized by the degeneration of upper and lower motor neurons, progressive wasting …

Patients with ALS show, in addition to the loss of motor neurons in the spinal cord,
brainstem, and cerebral cortex, an abnormal depletion of energy stores alongside …