ArticlePloS one2012
Plasma neurofilament heavy chain levels correlate to markers of late stage disease progression and treatment response in SOD1(G93A) mice that model ALS.
Article in PloS one, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers.
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Who cites it
28 citing papers in PubMed.
- Decreased antioxidant-related superoxide dismutase 1 expression in peripheral immune cells indicates early ethanol exposure.Scientific reports · 2024Article
- Review
- Efficacy and safety of a SOD1-targeting artificial miRNA delivered by AAV9 in mice are impacted by miRNA scaffold selection.Molecular therapy. Nucleic acids · 2023Article
- In vivo genome editing using novel AAV-PHP variants rescues motor function deficits and extends survival in a SOD1-ALS mouse model.Gene therapy · 2023Article
- Immunology of amyotrophic lateral sclerosis - role of the innate and adaptive immunity.Frontiers in neuroscience · 2023Review
- Humoral response to neurofilaments and dipeptide repeats in ALS progression.Annals of clinical and translational neurology · 2021Article
- The altered expression of neurofilament in mouse models and patients with spinal muscular atrophy.Annals of clinical and translational neurology · 2021Article
- Neurofilament Proteins as Biomarkers to Monitor Neurological Diseases and the Efficacy of Therapies.Frontiers in neuroscience · 2021Review
- Analysis of circulating protein aggregates as a route of investigation into neurodegenerative disorders.Brain communications · 2021Article
- Antibodies to neurofilament light as potential biomarkers in multiple sclerosis.BMJ neurology open · 2021Article
- Validation of serum neurofilaments as prognostic and potential pharmacodynamic biomarkers for ALS.Neurology · 2020Observational
- Muscle and not neuronal biomarkers correlate with severity in spinal and bulbar muscular atrophy.Neurology · 2019Article
- A motor neuron strategy to save time and energy in neurodegeneration: adaptive protein stoichiometry.Journal of neurochemistry · 2018Article
- Antisense oligonucleotides extend survival and reverse decrement in muscle response in ALS models.The Journal of clinical investigation · 2018Article
- Dysregulation of MicroRNAs and Target Genes Networks in Peripheral Blood of Patients With Sporadic Amyotrophic Lateral Sclerosis.Frontiers in molecular neuroscience · 2018Article
- Diagnostic and Prognostic Performance of Neurofilaments in ALS.Frontiers in neurology · 2018Review
- Fluid-Based Biomarkers for Amyotrophic Lateral Sclerosis.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2017Review
- Neurofilament depletion improves microtubule dynamics via modulation of Stat3/stathmin signaling.Acta neuropathologica · 2016Article
- New Insights on the Mechanisms of Disease Course Variability in ALS from Mutant SOD1 Mouse Models.Brain pathology (Zurich, Switzerland) · 2016Review
- Article
Corrections and comments
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Authors and funding
6 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundAmyotrophic lateral sclerosis (ALS) is an incurable neurodegenerative disorder characterised by progressive degeneration of motor neurons leading to death, typically within 3-5 years of symptom onset. The diagnosis of ALS is largely reliant on clinical assessment and electrophysiological findings. Neither specific investigative tools nor reliable biomarkers are currently available to enable an early diagnosis or monitoring of disease progression, hindering the design of treatment trials. METHODOLOGY/PRINCIPAL
findingsIn this study, using the well-established SOD1(G93A) mouse model of ALS and a new in-house ELISA method, we have validated that plasma neurofilament heavy chain protein (NfH) levels correlate with both functional markers of late stage disease progression and treatment response. We detected a significant increase in plasma levels of phosphorylated NfH during disease progression in SOD1(G93A) mice from 105 days onwards. Moreover, increased plasma NfH levels correlated with the decline in muscle force, motor unit survival and, more significantly, with the loss of spinal motor neurons in SOD1 mice during this critical period of decline. Importantly, mice treated with the disease modifying compound arimoclomol had lower plasma NfH levels, suggesting plasma NfH levels could be validated as an outcome measure for treatment trials. CONCLUSIONS/SIGNIFICANCE: These results show that plasma NfH levels closely reflect later stages of disease progression and therapeutic response in the SOD1(G93A) mouse model of ALS and may potentially be a valuable biomarker of later disease progression in ALS.
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