Motor neuron disease

Motor neuron disease usually refers to amyotrophic lateral sclerosis, but it can also refer to other kind of neurodegenerative disease that affect the motor neurons, such as progressive primary lateral sclerosis, progressive muscular atrophy and progressive bulpar palsy.

Latest Research and Reviews

Research
11 January 2023 | Open Access

NeuroLINCS Proteomics: Defining human-derived iPSC proteomes and protein signatures of pluripotency
- Andrea D. Matlock
- , Vineet Vaibhav
- & Jennifer E. Van Eyk
Scientific Data 10, 24
Reviews | 21 December 2022

Amyotrophic lateral sclerosis: a neurodegenerative disorder poised for successful therapeutic translation

Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease affecting motor neurons. In their Review, Shaw and colleagues provide a comprehensive picture of the various pathological mechanisms involved in this complex disease, and discuss the deep and diverse pipeline under development to tackle these processes. They highlight advances in ALS translational research that might be broadly applicable to other neurodegenerative disorders.
- Richard J. Mead
- , Ning Shan
- & Pamela J. Shaw
Nature Reviews Drug Discovery, 1-28
Research
21 December 2022 | Open Access

Proof of principle for the clinical use of a CE-certified automatic imaging analysis tool in rare diseases studying hereditary spastic paraplegia type 4 (SPG4)
- Tobias Lindig
- , Benjamin Bender
- & Tim W. Rattay
Scientific Reports 12, 22075
Research
09 December 2022 | Open Access

Molecular basis of C9orf72 poly-PR interference with the β-karyopherin family of nuclear transport receptors
- Hamidreza Jafarinia
- , Erik Van der Giessen
- & Patrick R. Onck
Scientific Reports 12, 21324
Reviews | 08 December 2022

Prospects for gene replacement therapies in amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis (ALS) is a devastating, incurable disease characterized by progressive loss of upper and lower motor neurons. Here, the authors describe the current landscape of genetic therapies for ALS and discuss new opportunities for gene replacement therapy, focusing on loss-of-function mutations.
- Ilaria Giovannelli
- , Adrian Higginbottom
- & Pamela J. Shaw
Nature Reviews Neurology 19, 39-52
Research
07 December 2022 | Open Access

Fatty acids derived from the probiotic Lacticaseibacillus rhamnosus HA-114 suppress age-dependent neurodegeneration

A probiotic bacterial strain (Lacticaseibacillus rhamnosus) delays neurodegeneration phenotypes in C. elegans models of amyotrophic lateral sclerosis and Huntington’s disease, with fatty acid extracts from the bacteria able to reproduce the protective effect.
- Audrey Labarre
- , Ericka Guitard
- & J. Alex Parker
Communications Biology 5, 1340

All Research & Reviews

News and Comment

Research Highlights | 03 January 2023

Spinal cord transcriptome provides ALS insights
- Heather Wood
Nature Reviews Neurology 19, 67
Research Highlights | 31 October 2022

FTD–ALS risk factors converge on the endolysosomal pathway

New research shows that variants in two FTD–ALS risk genes impair the functioning of the endolysosomal pathway, leading to pathological intracellular accumulation of TDP43 protein.
- Heather Wood
Nature Reviews Neurology 18, 699
Research Highlights | 03 October 2022

ALS therapy hits molecular mark but misses clinical one

Despite reducing the amount of toxic SOD1 protein, tofersen treatment did not lead to clinical improvements in patients with ALS.
- Karen O’Leary
Nature Medicine
News & Views | 08 September 2022

Machine learning approach finds nonlinear patterns of neurodegenerative disease progression

We developed a machine learning method that consistently and accurately identified dominant patterns of disease progression in amyotrophic later sclerosis (ALS), Alzheimer’s disease and Parkinson’s disease. Of note, the model was able to identify nonlinear progression trajectories in ALS, a finding that has clinical implications for patient stratification and clinical trial design.

Nature Computational Science 2, 565-566
News & Views | 15 July 2022

Early treatment is a lifeline for infants with SMA

In the phase 3 SPR1NT trial, pre-symptomatic gene therapy demonstrated impressive clinical outcomes in infants with a genetic diagnosis of spinal muscular atrophy (SMA); long-term safety follow-up of these patients must now be a key priority.
- Charlotte J. Sumner
- & Thomas O. Crawford
Nature Medicine 28, 1348-1349
News & Views | 12 July 2022

Monitoring disease onset in amyotrophic lateral sclerosis

A new longitudinal study involving carriers of amyotrophic lateral sclerosis (ALS)-associated mutations has identified a prodromal phase of ALS characterized by mild motor impairment. The findings could help us to define a time window during which neuroprotective interventions might be effective in patients with genetic forms of ALS.
- Mamede de Carvalho
Nature Reviews Neurology 18, 513-514

All News & Comment

Motor neuron disease

Latest Research and Reviews

NeuroLINCS Proteomics: Defining human-derived iPSC proteomes and protein signatures of pluripotency

Amyotrophic lateral sclerosis: a neurodegenerative disorder poised for successful therapeutic translation

Proof of principle for the clinical use of a CE-certified automatic imaging analysis tool in rare diseases studying hereditary spastic paraplegia type 4 (SPG4)

Molecular basis of C9orf72 poly-PR interference with the β-karyopherin family of nuclear transport receptors

Prospects for gene replacement therapies in amyotrophic lateral sclerosis

Fatty acids derived from the probiotic Lacticaseibacillus rhamnosus HA-114 suppress age-dependent neurodegeneration

News and Comment

Spinal cord transcriptome provides ALS insights

FTD–ALS risk factors converge on the endolysosomal pathway

ALS therapy hits molecular mark but misses clinical one

Machine learning approach finds nonlinear patterns of neurodegenerative disease progression

Early treatment is a lifeline for infants with SMA

Monitoring disease onset in amyotrophic lateral sclerosis

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