Amyotrophic lateral sclerosis: Current perspectives from basic research to the clinic
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by
progressive degeneration of upper and lower motoneurons, leading to muscle weakness …
progressive degeneration of upper and lower motoneurons, leading to muscle weakness …
Characterization of the contribution of genetic background and gender to disease progression in the SOD1 G93A mouse model of amyotrophic lateral sclerosis: a …
Background: The SOD1 G93A mouse model of amyotrophic lateral sclerosis (ALS) is the
most frequently used model to examine ALS pathophysiology. There is a lack of …
most frequently used model to examine ALS pathophysiology. There is a lack of …
Targeting mi R‐155 restores abnormal microglia and attenuates disease in SOD 1 mice
Objective To investigate miR‐155 in the SOD1 mouse model and human sporadic and
familial amyotrophic lateral sclerosis (ALS). Methods NanoString microRNA, microglia and …
familial amyotrophic lateral sclerosis (ALS). Methods NanoString microRNA, microglia and …
Neuroprotective effects of the mitochondria-targeted antioxidant MitoQ in a model of inherited amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by
motor neuron degeneration that ultimately results in progressive paralysis and death …
motor neuron degeneration that ultimately results in progressive paralysis and death …
Adult spinal motoneurones are not hyperexcitable in a mouse model of inherited amyotrophic lateral sclerosis
Key points Intrinsic hyperexcitability of spinal motoneurones is thought to contribute to
excitotoxicity during amyotrophic lateral sclerosis (ALS), but it has never been demonstrated …
excitotoxicity during amyotrophic lateral sclerosis (ALS), but it has never been demonstrated …
The vulnerability of spinal motoneurons and soma size plasticity in a mouse model of amyotrophic lateral sclerosis
SS Dukkipati, TL Garrett… - The Journal of …, 2018 - Wiley Online Library
Key points Motoneuron soma size is a largely plastic property that is altered during
amyotrophic lateral sclerosis (ALS) progression. We report evidence of systematic spinal …
amyotrophic lateral sclerosis (ALS) progression. We report evidence of systematic spinal …
Activity-dependent neuroprotective protein deficiency models synaptic and developmental phenotypes of autism-like syndrome
G Hacohen-Kleiman, S Sragovich… - The Journal of …, 2018 - Am Soc Clin Investig
Previous findings showed that in mice, complete knockout of activity-dependent
neuroprotective protein (ADNP) abolishes brain formation, while haploinsufficiency (Adnp+ …
neuroprotective protein (ADNP) abolishes brain formation, while haploinsufficiency (Adnp+ …
Modulation of astrocytic mitochondrial function by dichloroacetate improves survival and motor performance in inherited amyotrophic lateral sclerosis
Mitochondrial dysfunction is one of the pathogenic mechanisms that lead to
neurodegeneration in Amyotrophic Lateral Sclerosis (ALS). Astrocytes expressing the ALS …
neurodegeneration in Amyotrophic Lateral Sclerosis (ALS). Astrocytes expressing the ALS …
C57BL/6J congenic Prp-TDP43A315T mice develop progressive neurodegeneration in the myenteric plexus of the colon without exhibiting key features of ALS
ALS therapy development has been hindered by the lack of rodent animal models. The
discovery of TDP-43, a transcription factor that accumulates in the cytoplasm of motor …
discovery of TDP-43, a transcription factor that accumulates in the cytoplasm of motor …
Lack of GDAP1 induces neuronal calcium and mitochondrial defects in a knockout mouse model of charcot-marie-tooth neuropathy
M Barneo-Muñoz, P Juárez, A Civera-Tregón… - PLoS …, 2015 - journals.plos.org
Mutations in GDAP1, which encodes protein located in the mitochondrial outer membrane,
cause axonal recessive (AR-CMT2), axonal dominant (CMT2K) and demyelinating …
cause axonal recessive (AR-CMT2), axonal dominant (CMT2K) and demyelinating …