Dataset Information

Protein-agnostic pulldown of brain-derived aggregates from Motor Neuron Disease

ABSTRACT: Characterisation of homognised BA tissue extracts from TDP-43 MND (Ã—5), SOD1-MND (Ã—2) and neurologically normal controls (Ã—3) using the protein-agnostic aggregate probe CAP1.

INSTRUMENT(S):

ORGANISM(S): Homo Sapiens (human)

TISSUE(S): Brain

DISEASE(S): Motor Neuron Disease

SUBMITTER: Dezerae Cox

LAB HEAD: Prof. Sir David Klenerman

PROVIDER: PXD061429 | Pride | 2026-02-03

REPOSITORIES: Pride

ACCESS DATA

Dataset's files

Source:

Items per page:

1 - 5 of 15

Publications

Quantitative Profiling of Nanoscopic Protein Aggregates Reveals Specific Fingerprint of TDP-43-Positive Assemblies in Motor Neuron Disease.

Cox Dezerae D Burke Melanie M Milani Sara S White Matthew A MA Waldron Fergal M FM Böken Dorothea D Lobanova Evgeniia E Sreedharan Jemeen J Gregory Jenna M JM Klenerman David D

Advanced science (Weinheim, Baden-Wurttemberg, Germany) 20250923 44

Abnormal aggregation of TAR DNA-binding protein 43 (TDP-43) is a pathological hallmark of motor neuron disease (MND), yet current methods for quantifying these aggregates in biological samples remain limited in sensitivity and resolution. Here, single-molecule fluorescence microscopy is applied to post-mortem brain extracts to quantitatively characterize aggregates containing TDP-43 at the individual particle level. The resulting aggregate fingerprints, consisting of morphological and compositio ...[more]

PMID: 40985163

Similar Datasets

Project description:In our original grant we proposed to use the NR3B-null mouse model to study the role of NR3B subunit in motor neuron function. We have now successfully generated NR3B null mice. Interestingly, NR3B-null mice invariably die at age P4-P8. Our preliminary examination indicates that the motor strength of these mice is severely impaired prior to death. As we continue to explore the cause of death in NR3B null mice, we propose to conduct gene profiling experiments to search for transcription changes in the brain related to ablation of the NR3B gene. We have used the facility provided by the NINDS/NIMH Microarray Consortium to identify genes that show abnormal expression patterns in these mice. We would like to compare these changes with that opccured in SOD1 mice, a mouse model of motor neuron diseases. Analysis of these genes will help to identify changes in networks and pathways that may cause the death of NR3B-null mice. These studies will further help to elucidate the functional role of NR3B in motor neurons. We will compare samples from motor neurons of wild type and SOD1 mice to identify genes that show abnormal expression patterns, which may be implicated in the death of SOD1 mice and shared with the same changes in NR3B-null mice. We hypothesize that genes with their transcription level changing significantly by ablation of NR3B will be associated with the molecular mechanism underlying the death of motor neurons in NR3B null mice. As NR3B is expressed primarily in the motor neurons of hindbrain and spinal cord, we have first collected and analyzed the spinal cord samples from NR3B null mice and wild-type controls in P4, an age of disease onset. We like to compare motor neuron smaples from SOD1 mice at the age around the disease onset. Total RNA from total 6 samples will be purified from ~200 motor neurons obtained by Laser Capture Microdissection. Extracted RNAs will be subjected to two rounds of amplification and the obtained cRNA will be biotinylated. The purified cRNA will be sent to the NINDS/NIMH Microarray Consortium be used to hybridize the GeneChip Mouse Genome 430 2.0 Array. The hybridization, scanning, and initial data analysis of these GeneChips will be conducted by the Consortium staff. We will analyze the collected data further after data collection. We will first identify genes that show significant changes between wild-type and SOD1 mice and then compare that with the result from NR3B null mice.

			Action	DRS
	08072023_DRIX061_322.raw	Raw
	08072023_DRIX061_323.raw	Raw
	08072023_DRIX061_324.raw	Raw
	08072023_DRIX061_325.raw	Raw
	08072023_DRIX061_326.raw	Raw