Amyotrophic lateral sclerosis: a neurodegenerative disorder poised for successful therapeutic translation
Amyotrophic lateral sclerosis (ALS) is a devastating disease caused by degeneration of
motor neurons. As with all major neurodegenerative disorders, development of disease …
motor neurons. As with all major neurodegenerative disorders, development of disease …
Amyotrophic lateral sclerosis: translating genetic discoveries into therapies
Recent advances in sequencing technologies and collaborative efforts have led to
substantial progress in identifying the genetic causes of amyotrophic lateral sclerosis (ALS) …
substantial progress in identifying the genetic causes of amyotrophic lateral sclerosis (ALS) …
Liquid-liquid phase separation in biology: mechanisms, physiological functions and human diseases
Cells are compartmentalized by numerous membrane-enclosed organelles and
membraneless compartments to ensure that a wide variety of cellular activities occur in a …
membraneless compartments to ensure that a wide variety of cellular activities occur in a …
Heat-shock chaperone HSPB1 regulates cytoplasmic TDP-43 phase separation and liquid-to-gel transition
S Lu, J Hu, OA Arogundade, A Goginashvili… - Nature cell …, 2022 - nature.com
While acetylated, RNA-binding-deficient TDP-43 reversibly phase separates within nuclei
into complex droplets (anisosomes) comprised of TDP-43-containing liquid outer shells and …
into complex droplets (anisosomes) comprised of TDP-43-containing liquid outer shells and …
Single-cell transcriptomic landscape of the developing human spinal cord
J Andersen, N Thom, JL Shadrach, X Chen… - Nature …, 2023 - nature.com
Understanding spinal cord assembly is essential to elucidate how motor behavior is
controlled and how disorders arise. The human spinal cord is exquisitely organized, and this …
controlled and how disorders arise. The human spinal cord is exquisitely organized, and this …
Genome-wide identification of the genetic basis of amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS) is a complex disease that leads to motor neuron death.
Despite heritability estimates of 52%, genome-wide association studies (GWASs) have …
Despite heritability estimates of 52%, genome-wide association studies (GWASs) have …
Constitutively active SARM1 variants that induce neuropathy are enriched in ALS patients
AJ Bloom, X Mao, A Strickland, Y Sasaki… - Molecular …, 2022 - Springer
Background In response to injury, neurons activate a program of organized axon self-
destruction initiated by the NAD+ hydrolase, SARM1. In healthy neurons SARM1 is …
destruction initiated by the NAD+ hydrolase, SARM1. In healthy neurons SARM1 is …
Hsp40 proteins phase separate to chaperone the assembly and maintenance of membraneless organelles
Membraneless organelles contain a wide spectrum of molecular chaperones, indicating
their important roles in modulating the metastable conformation and biological function of …
their important roles in modulating the metastable conformation and biological function of …
TDP-43 pathology: from noxious assembly to therapeutic removal
Our understanding of amyotrophic lateral sclerosis and frontotemporal dementia has
advanced dramatically since the discovery of cytoplasmic TAR DNA-binding protein 43 (TDP …
advanced dramatically since the discovery of cytoplasmic TAR DNA-binding protein 43 (TDP …
Recent updates on the genetics of amyotrophic lateral sclerosis and frontotemporal dementia
L Kirola, A Mukherjee, M Mutsuddi - Molecular Neurobiology, 2022 - Springer
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) primarily affect the
motor and frontotemporal areas of the brain, respectively. These disorders share clinical …
motor and frontotemporal areas of the brain, respectively. These disorders share clinical …