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UBQLN2 encodes an ubiquitin-like protein (ubiquilin) that shares high degree of similarity with related products in yeast, rat and frog. Additionally we are shipping Ubiquilin 2 Antibodies (53) and Ubiquilin 2 Kits (9) and many more products for this protein.
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UBQLN2 is specifically expressed in the urine of urothelial carcinoma patients.
Results showed that UBQLN2 is selectively recruited to nuclear inclusions in Huntington's disease but not spinocerebellar ataxia (show USP14 Proteins) type 3
These findings provide a molecular basis for the development of ALS/FTD-associated proteinopathy and establish novel therapeutic targets for ALS.
Mutations in UBQLN2 gene cause dominant inheritance of amyotrophic lateral sclerosis due to Defective Proteasome Delivery.
UBQLN2 may be a new molecular target for chemotherapeutics and a useful clinicopathological marker in human osteosarcoma.
ALS (show IGFALS Proteins)-linked mutations in ubiquilin-2 or hnRNPA1 (show HNRNPA1 Proteins) reduce interaction between ubiquilin-2 and hnRNPA1 (show HNRNPA1 Proteins)
ALS (show IGFALS Proteins)-linked mutations in both OPTN (show OPTN Proteins) and UBQLN2 interfere with the constitution of specific endosomal vesicles, suggesting that the vesicles are involved in protein homeostasis and that these proteins function in common pathological processes.
A single putative mutation of UBQLN2 in a cohort of patients with front temporal lobar degeneration was found.
Data indicate cognitive deficits in mutant ubiquilin 2 protein UBQLN2P497H transgenic mice.
Causative mutation in the UBQLN2 gene is rare in Korean patients with either familial or sporadic ALS.
UBQLN2 most evidently bound to HSP70 (show HSP70 Proteins)-type chaperones, UBQLN1 (show UBQLN1 Proteins) and UBQLN4 (show UBQLN4 Proteins) and to a lesser extent to proteasomal subunits; UBQLN2 may be involved in the regulation of misfolded proteins. Mutations in UBQLN2, which lead to neurodegeneration in humans, are defective in chaperone binding, impair aggregate clearance, and cause cognitive deficits in mice.
UBQLN2 dysregulation in neurons can drive NF-kappaB (show NFKB1 Proteins) activation and cytosolic TDP-43 (show TARDBP Proteins) aggregation.
Transduction of UBQLN2 mutants (P497H, P497S, and P506T) induce proteinopathy and cause behavioral deficits, supporting a "toxic" gain-of-function amyotrophic lateral sclerosis model, in mice.
results were confirmed by similar findings for ubiquilin-1 (show UBQLN1 Proteins) and -2 in human brain tissue sections, where accumulation was observed in huntingtin (show HTT Proteins) inclusions
UBQLN2-transfected cells did not show increased starvation-induced cell death.
This gene encodes an ubiquitin-like protein (ubiquilin) that shares high degree of similarity with related products in yeast, rat and frog. Ubiquilins contain a N-terminal ubiquitin-like domain and a C-terminal ubiquitin-associated domain. They physically associate with both proteasomes and ubiquitin ligases\; and thus, are thought to functionally link the ubiquitination machinery to the proteasome to affect in vivo protein degradation. This ubiquilin has also been shown to bind the ATPase domain of the Hsp70-like Stch protein.
, Nedd4 binding protein 4
, protein linking IAP with cytoskeleton 2
, ubiquitin-like product Chap1/Dsk2
, Ubiquitin-related protein