ReviewProgress in neurobiology2011
On the development of markers for pathological TDP-43 in amyotrophic lateral sclerosis with and without dementia.
Review in Progress in neurobiology, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 34 papers, 4 of them syntheses that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
34 citing papers in PubMed, 4 syntheses or guidelines pooled it, 53 citations in OpenAlex.
- Muscle MRI and Muscle Ultrasound Applications in MND/ALS: Academic Insights and Clinical Opportunities.European journal of neurology · 2026Pooled it
- Premorbid de novo artistic creativity in frontotemporal dementia (FTD) syndromes.Journal of neural transmission (Vienna, Austria : 1996) · 2021Pooled it
- The imaging signature of C9orf72 hexanucleotide repeat expansions: implications for clinical trials and therapy development.Brain imaging and behavior · 2021Pooled it
- Prevalence of transactive response DNA-binding protein 43 (TDP-43) proteinopathy in cognitively normal older adults: systematic review and meta-analysis.Neuropathology and applied neurobiology · 2018Pooled it
- Article
- Neurotransmitter-defined cerebral vulnerability patterns in frontotemporal lobar degeneration (FTLD).Journal of neurology · 2026Article
- Driving with Motor Neuron Disease: Disease-Specific Considerations, Multi-Domain Assessments and Support Strategies.Brain sciences · 2026Review
- Putative mitochondrial components of frontotemporal lobar degeneration: topological correlations between mitochondrial density and atrophy in FTLD/FTD phenotypes.Journal of neurology · 2025Article
- New developments in imaging in ALS.Journal of neurology · 2025Review
- Cumulative incidence of motor and cognitive features in the amyotrophic lateral sclerosis-frontotemporal degeneration spectrum.Brain communications · 2025Article
- Clinical, Cortical, Subcortical, and White Matter Features of Right Temporal Variant FTD.Brain sciences · 2024Article
- Hippocampal Metabolic Alterations in Amyotrophic Lateral Sclerosis: A Magnetic Resonance Spectroscopy Study.Life (Basel, Switzerland) · 2023Article
- White matter microstructure alterations in frontotemporal dementia: Phenotype-associated signatures and single-subject interpretation.Brain and behavior · 2022Article
- Emergent creativity in frontotemporal dementia.Journal of neural transmission (Vienna, Austria : 1996) · 2021Review
- TDP-43 Vasculopathy in the Spinal Cord in Sporadic Amyotrophic Lateral Sclerosis (sALS) and Frontal Cortex in sALS/FTLD-TDP.Journal of neuropathology and experimental neurology · 2021Article
- TDP-43 and Phosphorylated TDP-43 Levels in Paired Plasma and CSF Samples in Amyotrophic Lateral Sclerosis.Frontiers in neurology · 2021Article
- Development of neurodegeneration in amyotrophic lateral sclerosis: from up or down?Journal of neural transmission (Vienna, Austria : 1996) · 2020Review
- Editorial: Biomarkers and Clinical Indicators in Motor Neuron Disease.Frontiers in neurology · 2019Article
- Widespread subcortical grey matter degeneration in primary lateral sclerosis: a multimodal imaging study with genetic profiling.NeuroImage. Clinical · 2019Article
- Brainstem pathology in amyotrophic lateral sclerosis and primary lateral sclerosis: A longitudinal neuroimaging study.NeuroImage. Clinical · 2019Article
Corrections and comments
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Authors and funding
8 authors at 4 institutions in 3 countries.
Funding
Abstract
Pathological 43-kDa transactive response sequence DNA-binding protein (TDP-43) has been recognized as the major disease protein in amyotrophic lateral sclerosis (ALS), frontotemporal lobar degeneration with ubiquitin positive, tau and α-synuclein negative inclusions (FTLD-U) and the transitional forms between these multisystem conditions. In order to develop TDP-43 into a successful ALS biomarker, the natural history of TDP-43 pathology needs to be characterized and the underlying pathophysiology established. Here we propose a spatial and temporal "two-axes" model of central nervous system vulnerability for TDP-43 linked degeneration and review recent studies on potential biomarkers related to pathological TDP-43 in the cerebrospinal fluid (CSF), blood, and skeletal muscle. The model includes the following two arms: Firstly, a "motor neuron disease" or "spinal cord/brainstem to motor cortex" axis (with degeneration possibly ascending from the lower motor neurons to the upper motor neurons); and secondly, a "dementia" or "corticoid/allocortex to neocortex" axis (with a probable spread of TDP-43 linked degeneration from the mediotemporal lobe to wider mesocortical and neocortical brain areas). At the cellular level, there is a gradual disappearance of normal TDP-43 in the nucleus in combination with the formation of pathological aggregates in the cell body and cellular processes, which can also be used to identify the stage of the disease process. Moreover, TDP-43 lesions in subpial/subependymal or perivascular localizations have been noted, and this might account for increased CSF and blood TDP-43 levels through mechanisms that remain to be elucidated.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.