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Full-Text Articles in Medicine and Health Sciences
Granulins Modulate Liquid–Liquid Phase Separation And Aggregation Of The Prion-Like C-Terminal Domain Of The Neurodegeneration-Associated Protein Tdp-43, Anukool A. Bhopatkar, Vladimir N. Uversky, Vijayaraghavan Rangachari
Granulins Modulate Liquid–Liquid Phase Separation And Aggregation Of The Prion-Like C-Terminal Domain Of The Neurodegeneration-Associated Protein Tdp-43, Anukool A. Bhopatkar, Vladimir N. Uversky, Vijayaraghavan Rangachari
Molecular Medicine Faculty Publications
TAR DNA-binding protein 43 (TDP-43) has emerged as a key player in many neurodegenerative pathologies, including frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). Hallmarks of both FTLD and ALS are the toxic cytoplasmic inclusions of the prion-like C-terminal fragments of TDP-43 CTD (TDP-43 C-terminal domain), formed upon proteolytic cleavage of full-length TDP-43 in the nucleus and subsequent transport to the cytoplasm. Both full-length TDP-43 and its CTD are also known to form stress granules by coacervating with RNA in the cytoplasm during stress and may be involved in these pathologies. Furthermore, mutations in the PGRN gene, leading to …
Advancing Stem Cell Therapy For Repair Of Damaged Lung Microvasculature In Amyotrophic Lateral Sclerosis, Svitlana Garbuzova-Davis, Robert Shell, Hilmi Mustafa, Surafuale Hailu, Alison E. Willing, Paul R. Sanberg, Cesar V. Borlongan
Advancing Stem Cell Therapy For Repair Of Damaged Lung Microvasculature In Amyotrophic Lateral Sclerosis, Svitlana Garbuzova-Davis, Robert Shell, Hilmi Mustafa, Surafuale Hailu, Alison E. Willing, Paul R. Sanberg, Cesar V. Borlongan
Neurosurgery and Brain Repair Faculty Publications
Amyotrophic lateral sclerosis (ALS) is a fatal disease of motor neuron degeneration in the brain and spinal cord. Progressive paralysis of the diaphragm and other respiratory muscles leading to respiratory dysfunction and failure is the most common cause of death in ALS patients. Respiratory impairment has also been shown in animal models of ALS. Vascular pathology is another recently recognized hallmark of ALS pathogenesis. Central nervous system (CNS) capillary damage is a shared disease element in ALS rodent models and ALS patients. Microvascular impairment outside of the CNS, such as in the lungs, may occur in ALS, triggering lung damage …