Now scientists have used CRISPR to learn more about the cellular dysfunction that underlies the neurodegenerative disorder ALS (amyotrophic lateral sclerosis). The findings have linked the disease to ...

Using CRISPR gene editing and stem cell-derived motor neurons, scientists from Stockholm University and UK Dementia Research Institute uncovered that mitochondrial dysfunction occurs early in ALS, ...

Motor neurone disease (MND) is a rare but progressive neurological disorder characterised by the degeneration of motor ...

In addition to the mitochondrial defects, a key feature of both sporadic and familial ALS is the unexplained loss or dysfunction of glutamate transporters localized on astrocytes.

Miller noted that in ALS, “You see a lot wrong with the muscle, too,” and suggested there is a renewed appreciation for the role of muscle in the ALS disease process. Magrané was excited to see that ...

Manfredi added that the ALS-FTD spectrum has been expanding, and this kind of “hybrid” mitochondrial myopathy/motor neuron disease is not entirely novel. After all, the multisystem proteinopathy due ...

Also, studies in Alzheimer's disease have linked changes in mitochondrial function to interactions between an abnormal form of tau, which accumulates in the brains of patients with Alzheimer's ...

Mitochondrial dysfunction characterizes a range of neurodegenerative disorders, including Alzheimer's, Parkinson's, and Huntington's diseases, ALS, and multiple sclerosis. In such conditions ...

“Knockout of GSDME in SOD1 G93A ALS mice prolonged survival, ameliorated motor dysfunction, rescued motor neuron loss, and reduced neuroinflammation,” they stated.

In Brazil, few epidemiological studies of ALS exist. The prevalence of the disease is estimated at 0.9-1.5 cases per 100,000 inhabitants per year.

Mitochondria are crucial organelles for cellular homeostasis, regulating processes such as ATP production, reactive oxygen ...

Reference: Schweingruber C, Nijssen J, Mechtersheimer J, et al. Single-cell RNA-sequencing reveals early mitochondrial dysfunction unique to motor neurons shared across FUS- and TARDBP-ALS. Nat Commun ...