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 …
Sources, resolution and physiological relevance of R-loops and RNA–DNA hybrids
RNA–DNA hybrids are generated during transcription, DNA replication and DNA repair and
are crucial intermediates in these processes. When RNA–DNA hybrids are stably formed in …
are crucial intermediates in these processes. When RNA–DNA hybrids are stably formed in …
Cellular functions of the protein kinase ATM and their relevance to human disease
The protein kinase ataxia telangiectasia mutated (ATM) is a master regulator of double-
strand DNA break (DSB) signalling and stress responses. For three decades, ATM has been …
strand DNA break (DSB) signalling and stress responses. For three decades, ATM has been …
C9orf72-mediated ALS and FTD: multiple pathways to disease
R Balendra, AM Isaacs - Nature Reviews Neurology, 2018 - nature.com
The discovery that repeat expansions in the C9orf72 gene are a frequent cause of
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) has revolutionized …
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) has revolutionized …
Amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease, is characterized
by the degeneration of both upper and lower motor neurons, which leads to muscle …
by the degeneration of both upper and lower motor neurons, which leads to muscle …
Human ALS/FTD brain organoid slice cultures display distinct early astrocyte and targetable neuronal pathology
Amyotrophic lateral sclerosis overlap** with frontotemporal dementia (ALS/FTD) is a fatal
and currently untreatable disease characterized by rapid cognitive decline and paralysis …
and currently untreatable disease characterized by rapid cognitive decline and paralysis …
Genetics of amyotrophic lateral sclerosis: seeking therapeutic targets in the era of gene therapy
N Suzuki, A Nishiyama, H Warita, M Aoki - Journal of human genetics, 2023 - nature.com
Amyotrophic lateral sclerosis (ALS) is an intractable disease that causes respiratory failure
leading to mortality. The main locus of ALS is motor neurons. The success of antisense …
leading to mortality. The main locus of ALS is motor neurons. The success of antisense …
Redox dysregulation as a driver for DNA damage and its relationship to neurodegenerative diseases
Redox homeostasis refers to the balance between the production of reactive oxygen species
(ROS) as well as reactive nitrogen species (RNS), and their elimination by antioxidants. It is …
(ROS) as well as reactive nitrogen species (RNS), and their elimination by antioxidants. It is …
Integrated transcriptome landscape of ALS identifies genome instability linked to TDP-43 pathology
Abstract Amyotrophic Lateral Sclerosis (ALS) causes motor neuron degeneration, with 97%
of cases exhibiting TDP-43 proteinopathy. Elucidating pathomechanisms has been …
of cases exhibiting TDP-43 proteinopathy. Elucidating pathomechanisms has been …
Motor neuron susceptibility in ALS/FTD
AMG Ragagnin, S Shadfar, M Vidal… - Frontiers in …, 2019 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the
death of both upper and lower motor neurons (MNs) in the brain, brainstem and spinal cord …
death of both upper and lower motor neurons (MNs) in the brain, brainstem and spinal cord …