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Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis
Ling SC, Polymenidou M, Cleveland DW.. Neuron, 2013-08-01
Topic: Brain proteins, microglia and lab models
Breakthrough discoveries identifying common genetic causes for amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) have transformed our view of these disorders. They share unexpectedly similar signatures, including dysregulation in common molecular players including TDP-43, FUS/TLS, ubiquilin-2, VCP, and expanded hexanucleotide repeats within the C9ORF72 gene. Dysfunction in RNA processing and protein homeostasis is an emerging theme. We present the case here that these two processes are intimately linked, with disease-initiated perturbation of either leading to further deviation of both protein and RNA homeostasis through a feedforward loop including cell-to-cell prion-like spread that may represent the mechanism for relentless disease progression.
Cite this study APA, Vancouver, BibTeX · download .bib or .ris
APA 7th
Ling, S. C., Polymenidou, M., & Cleveland, D. W. (2013). Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis. Neuron. https://doi.org/10.1016/j.neuron.2013.07.033
Vancouver
Ling SC, Polymenidou M, Cleveland DW. Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis. Neuron. 2013. doi:10.1016/j.neuron.2013.07.033. PMID: 23931993.
BibTeX
@article{ling2013-23931993,
author = {Ling, S. C. and Polymenidou, M. and Cleveland, D. W.},
title = {{Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis}},
journal = {Neuron},
year = {2013},
doi = {10.1016/j.neuron.2013.07.033},
pmid = {23931993},
url = {https://doi.org/10.1016/j.neuron.2013.07.033}
}
Built from the record alzhub holds for this paper, not looked up. Volume, issue and page numbers are not held in the record. Check the citation against the original before you use it.
Key findings
- Breakthrough discoveries identifying common genetic causes for amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) have transformed our view of these disorders.
- They share unexpectedly similar signatures, including dysregulation in common molecular players including TDP-43, FUS/TLS, ubiquilin-2, VCP, and expanded hexanucleotide repeats within the C9ORF72 gene.
- Dysfunction in RNA processing and protein homeostasis is an emerging theme.
- We present the case here that these two processes are intimately linked, with disease-initiated perturbation of either leading to further deviation of both protein and RNA homeostasis through a feedforward loop including cell-to-cell prion-like spread that may represent the mechanism for relentless disease progression.
Why it matters
This review argues that amyotrophic lateral sclerosis and frontotemporal dementia share disrupted RNA and protein homeostasis, with a possible feedforward loop including cell-to-cell prion-like spread driving progression. It is a mechanistic proposal about these two diseases, not a treatment, cure or prevention for Alzheimer's disease.
Study details
- Evidence strengthLow
How this study scored 58/100
Published in a peer-reviewed journal. Scored zero for study design, sample size, effect reported, studied in people and randomised / blinded because the record does not state it.
| Component | Points | Why |
|---|---|---|
| Journal tier | 7 / 10 | Tier 3 — peer-reviewed journal (Neuron) |
| Journal's citation record | 4 / 4 | Neuron: journal h-index 689 (OpenAlex, 2026-10-29) |
| Study design | 0 / 12 | No study design recorded — scores zero, not guessed (not stated in the record — scored 0) |
| Strength of evidence | 1 / 5 | Rated “low” in our summary |
| Peer-review validation | 10 / 10 | Peer-reviewed. Published as an article in a named peer-reviewed journal. |
| How recent | 1 / 5 | 2013 — within 20 year(s) of the newest study here |
| Sample size | 0 / 4 | Participant count not stated — scores zero (not stated in the record — scored 0) |
| Effect reported | 0 / 3 | No effect size reported — scores zero (not stated in the record — scored 0) |
| Studied in people | 0 / 3 | Not stated whether this was studied in people — scores zero (not stated in the record — scored 0) |
| Randomised / blinded | 0 / 2 | No randomisation or blinding stated — scores zero (not stated in the record — scored 0) |
| Times cited | 7 / 7 | Cited 1,814 times (OpenAlex, 2026-10-10) |
| Citations per year | 5 / 5 | 120.9 citations a year over 15 year(s) since 2013 |
| Impact for its field and year | 6 / 6 | Cited 63.8× the average for papers of its field and year (OpenAlex FWCI) |
| Cited by studies here | 4 / 6 | Cited by 53 other studies in this library |
| Still being cited | 4 / 4 | Cited 422 times in 2024–2026 (2026 still in progress; OpenAlex) |
| Senior author's track record | 4 / 4 | Last-listed author Don W. Cleveland: h-index 165 (OpenAlex, whole career) |
| Institution's track record | 2 / 3 | University of California San Diego: institution h-index 1,852 (OpenAlex) |
| Multi-centre collaboration | 1 / 3 | OpenAlex lists 2 institution(s) in 1 country |
| Indexed in PubMed | 2 / 2 | Indexed in PubMed (PMID 23931993) |
| Free to read | 0 / 2 | Behind a paywall — scores zero |
| Total | 58 / 100 | — |
What each of these 20 components measures, and what this score is not →
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