[HTML][HTML] ALS genetics, mechanisms, and therapeutics: where are we now?
The scientific landscape surrounding amyotrophic lateral sclerosis (ALS) continues to shift
as the number of genes associated with the disease risk and pathogenesis, and the cellular …
as the number of genes associated with the disease risk and pathogenesis, and the cellular …
Amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis is characterised by the progressive loss of motor neurons in
the brain and spinal cord. This neurodegenerative syndrome shares pathobiological …
the brain and spinal cord. This neurodegenerative syndrome shares pathobiological …
[HTML][HTML] Generation of functional human 3D cortico-motor assembloids
Neurons in the cerebral cortex connect through descending pathways to hindbrain and
spinal cord to activate muscle and generate movement. Although components of this …
spinal cord to activate muscle and generate movement. Although components of this …
[HTML][HTML] Self-organizing 3D human trunk neuromuscular organoids
JMF Martins, C Fischer, A Urzi, R Vidal, S Kunz… - Cell stem cell, 2020 - cell.com
Neuromuscular networks assemble during early human embryonic development and are
essential for the control of body movement. Previous neuromuscular junction modeling …
essential for the control of body movement. Previous neuromuscular junction modeling …
[HTML][HTML] Multi-lineage human iPSC-derived platforms for disease modeling and drug discovery
Human induced pluripotent stem cells (hiPSCs) provide a powerful platform for disease
modeling and have unlocked new possibilities for understanding the mechanisms governing …
modeling and have unlocked new possibilities for understanding the mechanisms governing …
[HTML][HTML] SOD1, more than just an antioxidant
ECA Eleutherio, RSS Magalhães… - Archives of Biochemistry …, 2021 - Elsevier
During cellular respiration, radicals, such as superoxide, are produced, and in a large
concentration, they may cause cell damage. To combat this threat, the cell employs the …
concentration, they may cause cell damage. To combat this threat, the cell employs the …
[HTML][HTML] Large-scale differentiation of iPSC-derived motor neurons from ALS and control subjects
Using induced pluripotent stem cells (iPSCs) to understand the mechanisms of neurological
disease holds great promise; however, there is a lack of well-curated lines from a large array …
disease holds great promise; however, there is a lack of well-curated lines from a large array …
Nuclear accumulation of CHMP7 initiates nuclear pore complex injury and subsequent TDP-43 dysfunction in sporadic and familial ALS
AN Coyne, V Baskerville, BL Zaepfel… - Science translational …, 2021 - science.org
Alterations in the components [nucleoporins (Nups)] and function of the nuclear pore
complex (NPC) have been implicated as contributors to the pathogenesis of genetic forms of …
complex (NPC) have been implicated as contributors to the pathogenesis of genetic forms of …
[HTML][HTML] A 3D culture model of innervated human skeletal muscle enables studies of the adult neuromuscular junction
Two-dimensional (2D) human skeletal muscle fiber cultures are ill-equipped to support the
contractile properties of maturing muscle fibers. This limits their application to the study of …
contractile properties of maturing muscle fibers. This limits their application to the study of …
[HTML][HTML] Mitochondrial bioenergetic deficits in C9orf72 amyotrophic lateral sclerosis motor neurons cause dysfunctional axonal homeostasis
Axonal dysfunction is a common phenotype in neurodegenerative disorders, including in
amyotrophic lateral sclerosis (ALS), where the key pathological cell-type, the motor neuron …
amyotrophic lateral sclerosis (ALS), where the key pathological cell-type, the motor neuron …