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Using immunoprecipitations from HEK293T cells of UBQLN2-HA constructs, sites of ubiquitination were identified. A variety of UBQLN2 variants were studied including lysineless proteins, deletions of the UBL domain and N-terminal fusions to ubiquitin.
ORGANISM(S): Homo sapiens (Human) 
2025-12-17 | PXD059465 | Pride
Familial neurodegenerative diseases commonly involve mutations that result in either aberrant proteins or dysfunctional components of the proteolytic machinery that acts on aberrant proteins. UBQLN2 is a ubiquitin receptor of the UBL/UBA family that binds the proteasome through its ubiquitin-like (...
ORGANISM(S): Homo sapiens (Human) Mus musculus (Mouse) 
2021-01-20 | PXD021991 | Pride
To address whether expression of ALS/FTD-associated UBQLN2 mutant variants changes the cellular proteome, we performed label-free quantification-based global protein abundance profiling of patient-derived LCLs and CRISPR engineered HeLa cells. UBQLN2 mutant in both cell lines carried the mutation T4...
ORGANISM(S): Homo sapiens (Human) 
2022-06-24 | PXD029747 | Pride
UBQLN2 Links Proteotoxicity with Lipid Metabolism in Neurodegeneration
Protein homeostasis and lipid metabolism are crucial cellular processes that are dysregulated in neurodegeneration. However, their connection in neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) remains unexplored. Ubiquilin 2 (UBQLN2) is a prot...
ORGANISM(S): Homo sapiens 
2025-07-01 | GSE272994 | GEO
Amyotrophic Lateral Sclerosis (ALS) is a rare neurodegenerative disease characterized by motor neuron dysfunction and loss, leading to progressive paralysis and death. A portion of ALS cases is caused by mutation of the proteasome shuttle factor Ubiquilin 2 (UBQLN2), but the molecular pathway leadin...
ORGANISM(S): Homo sapiens (Human) 
2023-03-24 | PXD031964 | Pride
In the project “Phosphoproteomic analysis of UBQLN2 mutant cells” by Laura Strohm, Zehan Hu, Jörn Dengjel, and Christian Behrends eight sets of SILAC experiments were performed, two times four biological replicates, comparing proteome and phosphoproteome of control and UBQLN2 mutant, patient-derived...
ORGANISM(S): Homo sapiens (Human) 
2022-06-28 | PXD029730 | Pride
The human genome is replete with sequences derived from foreign elements including endogenous retrovirus-like proteins of unknown function. The human genome is replete with sequences derived from foreign elements including endogenous retrovirus-like proteins of unknown function. Here we show that UB...
ORGANISM(S): Homo sapiens (Human) 
2025-06-04 | PXD061852 | Pride
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