#TDP43
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realfeedapp.com
VectorY Therapeutics expanded the PIONEER-ALS trial from 12 to 36 participants to test VTx-002, an antibody targeting TDP-43 protein aggregates found in 97% of patients.

#ALS #TDP43 #AntibodyTherapy
PIONEER-ALS enrollment expands before efficacy data
Source: Longevity.Technology
realfeedapp.com
October 19, 2026 at 6:00 PM
rridrobot.bsky.social
The authors included RRIDs in their in Biophysical Chemistry paper! RRIDs improve reproducibility in scientific research. #OpenScience #STMpublishing #ReproducibleResearch
Small heat shock protein HSPB8 interacts with a pre-fibrillar TDP43 low complexity domain species to delay fibril formation
Read the full paper: Small heat shock protein HSPB8 interacts with a pre-fibrillar TDP43 low complexity domain species to delay fibril formation
doi.org
October 9, 2026 at 7:12 AM
molneurodegen.bsky.social
Nucleoporin 50 rescues hallmarks of #TDP43 proteinopathy in #ALS/FTD disease models

Courtney L. Smith, Bilal Khalil, Alyssa C. Walker... Wilfried Rossoll @rossolllab.bsky.social @mayoclinic.org #FrontotemporalDementia

link.springer.com/article/10.1...
October 8, 2026 at 4:55 PM
molneurodegenadvnc.bsky.social
Monospecific antibodies reveal reduction of granulins in #FrontotemporalDementia caused by GRN mutations

Paola Merino, Ludmilla Troiano Araujo, Anarmaa Mendsaikhan...Thomas Kukar @kukarlab.bsky.social #TDP43 #Progranulin #LysosomalStorageDisorder

link.springer.com/article/10.1...
Monospecific antibodies reveal reduction of granulins in frontotemporal dementia caused by GRN mutations - Molecular Neurodegeneration Advances
Background Loss-of-function mutations in the progranulin (PGRN) gene (GRN) are a major cause of Frontotemporal dementia (FTD), also known as frontotemporal lobar degeneration (FTLD). PGRN is a lysosom...
link.springer.com
October 8, 2026 at 5:55 PM
molneurodegen.bsky.social
In vivo detection of pathologic α- #synuclein and #TDP43 in the skin and olfactory mucosa of a patient with #Parkinsons disease-like phenotype carrying the p.Thr272Serfs*10 GRN mutation

Paola Caroppo, Vittoria Aprea...Grazia Devigili

link.springer.com/article/10.1...
In vivo detection of pathologic α- synuclein and TDP-43 in the skin and olfactory mucosa of a patient with Parkinson’s disease-like phenotype carrying the p.Thr272Serfs*10 GRN mutation - Molecular Neu...
We describe a patient carrying the GRN p.Thr272Serfs*10 mutation, who presented with parkinsonism at onset and later developed prodromal frontotemporal dementia, in whom seed amplification assay on ol...
link.springer.com
October 5, 2026 at 2:16 PM
elevylab.bsky.social
Out of 10 IDRs, 8 colocalized indiscriminately, whereas
TDP43 and UBQ2 formed spatially distinct condensates. In TDP43 a segment forming a transient α-helix (see work by @nicolasluxfawzi.bsky.social) determined self-recognition specificity as a truncated mutant co-localized with all other IDRs.
September 25, 2026 at 12:25 PM
molneurodegenadvnc.bsky.social
As part of our inaugural review series!

Targeting protein aggregate co-pathologies in #neurodegeneration: a viable therapeutic strategy?

Silas A. Buck, Sidharth S. Madhavan, Laurie H. Sanders
@dukemedschool.bsky.social #TDP43 #tauopathy #amyloid #alphasynuclein

link.springer.com/article/10.1...
Targeting protein aggregate co-pathologies in neurodegeneration: a viable therapeutic strategy? - Molecular Neurodegeneration Advances
Many neurodegenerative diseases are characterized by pathological protein aggregation in the brain. Alzheimer’s disease displays amyloid-β and tau inclusions in the form of amyloid-β plaques and tau n...
link.springer.com
September 23, 2026 at 3:46 PM
traffic-journal.bsky.social
Just Published !

#TDP43 #ALS #neurodegeneration #yeast #endoplasmicreticulum

onlinelibrary.wiley.com/doi/10.1111/...
Investigating Translocation Mechanisms of <fc>TDP</fc>‐43 Into the Endoplasmic Reticulum Using Budding Yeast Models
The graphical abstract illustrates a model in which TDP-43 C-terminal fragments undergo Ste24-suppressed, Sec61/Sec66-dependent noncanonical ER entry in budding yeast. ER entry is facilitated by resi...
onlinelibrary.wiley.com
September 22, 2026 at 12:34 PM
rridrobot.bsky.social
Authors used pDuet TDP43 WT from @addgene.bsky.social in their study. Including #RRIDs will make this less ambiguous.

SciScore made a table with this resource, see “Automated Services” module (download as csv, xml or #jats) #OpenScience #reproducibility
www.biorxiv.org
September 8, 2026 at 12:00 PM
nsrhighlights.bsky.social
In mouse cortex, developmental TDP-43 expression disrupted long-range projection patterns; expression after birth shortened axons and reduced branching.

https://doi.org/10.1016/j.neures.2026.105079
#NeuroscienceResearch #Neuroscience #TDP43
Japanese painterly NSR cover showing a cortical neuron sending a long callosal axon; visibly shortened distal projection; and reduced branch complexity without depicting degeneration in a patient. The canonical explorer walks along the projection and records where branches become sparse. A small washi banner reads Neuroscience Research.
August 31, 2026 at 11:15 AM
nsrhighlights.bsky.social
ALSに関わるTDP-43の異常は、軸索を作る時期と、できた後で同じ影響を及ぼすのでしょうか。マウス皮質では、発達期には長距離投射の配置が乱れ、出生後には軸索が短くなって分岐が減りました。

https://doi.org/10.1016/j.neures.2026.105079
#NeuroscienceResearch #Neuroscience #TDP43
Japanese painterly NSR cover showing a cortical neuron sending a long callosal axon; visibly shortened distal projection; and reduced branch complexity without depicting degeneration in a patient. The canonical explorer walks along the projection and records where branches become sparse. A small washi banner reads Neuroscience Research.
August 31, 2026 at 11:15 AM
jamaneurology.com
This Review proposes #TDP43-associated neurodegenerative disease as a staging framework that links presymptomatic biology with later clinical phenotypes and could support earlier mechanism-based treatment development. ja.ma/4g487c8
August 24, 2026 at 3:00 PM
jamaneurology.com
This JAMA Neurology #Review proposes #TDP43-associated neurodegenerative disease as a staging framework that links presymptomatic biology with later clinical phenotypes and could support earlier mechanism-based treatment development. ja.ma/4g487c8
TDP-43–Associated Neurodegenerative Disease
This narrative review discusses an alternative approach to disease classification based on underlying pathobiology, specifically examining the role of transactive response DNA-binding protein, 43 kDa ...
ja.ma
August 24, 2026 at 4:42 PM
alicefrolov.bsky.social
Progressive cytoplasmic mislocalization of TDP-43 across neuronal aging stages

I am sharing a data visualization intended to communicate a well-documented trend in TDP-43 proteostasis.

#TDP43 #Proteostasis #Neurodegeneration #Nucleocytoplasmic #DataVisualization #Proteinopathy
Progressive cytoplasmic mislocalization of TDP-43 across neuronal aging stages I am sharing a data visualization intended to communicate a well-documented trend in TDP-43 proteostasis, rendered here as a ridgeline-style plot. Each row depicts the single-neuron distribution of the cytoplasmic-to-nuclear (C/N) TDP-43 ratio for a discrete neuronal aging stage, with distributions stacked vertically to facilitate comparison of both central tendency and dispersion across stages. I want to state explicitly at the outset that the data shown are simulated. The intent is to illustrate a literature-grounded trend rather than to report a specific empirical dataset. The simulated cohort comprises approximately 600 neurons per stage, with C/N ratios sampled to reproduce the qualitative behavior reported across the aging and neurodegeneration literature. The central feature is a rightward shift of the modal C/N ratio with advancing age, accompanied by progressive right-skew and heavier upper tails. Mechanistically, this pattern is consistent with age-associated attrition of nuclear import capacity, disruption of the RanGTPase gradient and nucleocytoplasmic transport machinery, and impaired clearance of cytoplasmic TDP-43 species. As nuclear TDP-43 becomes depleted, loss of autoregulatory splicing control and de-repression of cryptic exons are expected downstream consequences, while cytoplasmic accumulation predisposes to liquid-liquid phase separation and progression toward insoluble, phosphorylated inclusions. The widening dispersion in later stages is intended to capture stochastic single-neuron heterogeneity, reflecting the non-synchronous transition of individual neurons across a proteostatic threshold rather than a uniform population drift. I would welcome methodological critique, particularly regarding appropriate parameterizations for the tail behavior and suggestions for empirical datasets suitable for calibration and validation.
August 11, 2026 at 5:00 PM
timmyfromtheblock.bsky.social
interesting work on als mechs in *not* MNs. proprioceptive sensory degeneration in SOD1 and TDP43 transgenic mice. http://onlinelibrary.wiley.com/resolve/doi?DOI=10.1002/cne.23906
December 7, 2024 at 12:35 AM
zimmermanv66.bsky.social
www.sciencedaily.com/releases/202...
Scientists discover ALS protein that links DNA repair to cancer and dementia
A protein tied to ALS and dementia may have a much bigger role in disease than scientists realized. Researchers found that TDP43 controls a key DNA repair process, but when the protein becomes imbalan...
www.sciencedaily.com
March 22, 2026 at 3:24 PM
zimmermanv66.bsky.social
www.sciencedaily.com/releases/202...
Scientists discover ALS protein that links DNA repair to cancer and dementia
A protein tied to ALS and dementia may have a much bigger role in disease than scientists realized. Researchers found that TDP43 controls a key DNA repair process, but when the protein becomes imbalan...
www.sciencedaily.com
March 20, 2026 at 3:47 PM
zimmermanv66.bsky.social
www.sciencedaily.com/releases/202...
Scientists discover ALS protein that links DNA repair to cancer and dementia
A protein tied to ALS and dementia may have a much bigger role in disease than scientists realized. Researchers found that TDP43 controls a key DNA repair process, but when the protein becomes imbalan...
www.sciencedaily.com
March 18, 2026 at 3:53 PM
zimmermanv66.bsky.social
www.sciencedaily.com/releases/202...
Scientists discover ALS protein that links DNA repair to cancer and dementia
A protein tied to ALS and dementia may have a much bigger role in disease than scientists realized. Researchers found that TDP43 controls a key DNA repair process, but when the protein becomes imbalan...
www.sciencedaily.com
March 16, 2026 at 3:36 PM
zimmermanv66.bsky.social
www.sciencedaily.com/releases/202...
Scientists discover ALS protein that links DNA repair to cancer and dementia
A protein tied to ALS and dementia may have a much bigger role in disease than scientists realized. Researchers found that TDP43 controls a key DNA repair process, but when the protein becomes imbalan...
www.sciencedaily.com
March 15, 2026 at 3:10 PM
lorneproteins.bsky.social
APPA Joint Session- Yun-Ru (Ruby) Chen. Welcomes everyone to APPA in Singapore in 2028! Neurodegenerative Disease. TDP proteinopathies, not thioflavin T positive. Forms soluble aggregate. Conformational antibody to detect amyloid. Translational therapeutic development in progress. #LorneProteins2025
Ruby Chen standing behind a lectern. Speaking about neurodegenerative proteinopathies. Cross seeded with aB, prevents aB fibril growth but worsens neurodegeneration in models. 57% of AD patients have TDP43 amyloid. N-term and C-term of TDP43 determinants of Abeta fibril formation. Keeps Abeta in an aggregated toxic form and limits fibril formation. Hyperphosphorylation of TDP43 drives amyloid formation and possesses neuronal toxicity at the oligomeric stage. Application of monoclonal antibody against TDP43 monomer and oligomer state. Improvements in ALS symptoms in mouse model. C9ORF72 hexanucleotide expansions. Dipeptide repeats 20 to 1000 form toxic amyloids that can pass from cell to cell. Classic ThT positive amyloid. Toxicity from Arg-rich DPRs. Length and dose-dependent toxicity. SAXS shows helical conformatiok in a length dependent manner. Pro insertion breaks helices. PolyGR peptides damage the nuclear membrane. Can interact with DNA to induce DNA damage. HS disaccharides to rescue GR toxicity.
February 10, 2025 at 10:13 PM
gregoryai.bsky.social
https://gregory-ms.com/articles/280474/
Stimulating VAPB-PTPIP51 ER-mitochondria tethering corrects FTD/ALS mutant TDP43 linked Ca2+ and synaptic defects

#MSchat #MS #Neurology #MultipleSclerosis #NeuroSky #NeuroSkyence
June 2, 2024 at 4:10 PM
rthepple.bsky.social
I wonder how specific this may be to ALS? Aging also causes Tdp43 dysfunction (risk of ALS increases w age) www.nature.com/articles/s42... so perhaps this is more about importance of CoxIV to motoneuron survival than ALS per se?
Age-related demethylation of the TDP-43 autoregulatory region in the human motor cortex - Communications Biology
In order to assess the effects of aging on the autoregulation of TAR DNA-binding protein 43 (TDP-43) and the potential effects of this on the role of TDP-43 in Amyotrophic Lateral Sclerosis (ALS), Koi...
www.nature.com
March 15, 2025 at 7:06 PM
prudenciolab.bsky.social
Our manuscript got highlighted with a beautiful infographic!

#TDP43 #PerrySyndrome #Neurodegeneration #RNAsplicing #Biomarkers #Neuroscience #FTLD #ALS #PrudencioLab

DOI: lnkd.in/eXr8te8A
April 3, 2025 at 4:17 PM
wisconsinadrc.bsky.social
Join @uwmadison.bsky.social and #CLARiTI for "Advancing TDP-43 Biomarkers: From Discovery to Application," a virtual summit exploring cutting-edge biomarker research.
📅 June 12
✅ RSVP at go.wisc.edu/92qiel to gain insights from top researchers shaping the future of neurodegenerative disease research
2025 Summit Advancing TDP43 Biomarkers: From Discovery to Application | Alzheimer's Disease Research Center
RSVP today to join Drs. Henrik Zetterberg, Hartmuth Kolb and Sterling Johnson for the 2025 Summit Advancing TDP43 Biomarkers: From Discovery to Application, hosted by the University of Wisconsin–Madis...
go.wisc.edu
April 28, 2025 at 7:56 PM