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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 (60) and Ubiquilin 2 Proteins (5) and many more products for this protein.
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The structures of proteasome substrate receptor complexes with the shuttle factors that deliver ubiquitinated proteins to proteasomes have been solved, namely human Rpn13 (show Adrm1 ELISA Kits) complexed with PLIC2 and Saccharomyces cerevisiae Rpn1 (show PSMD2 ELISA Kits) with Rad23.
Ubiquilins are a family of chaperones for cytosolically exposed transmembrane domains and use ubiquitin to triage clients for degradation via coordinated intra- and intermolecular interactions.
We analyzed mutations in the UBQLN2 gene in a Chinese cohort with sporadic ALS (sALS). A novel missense mutation was detected in one sALS patient. The p.M392V mutation substitutes a highly conserved residue, has not been reported in the population databases, and previously, at the same residue, a missense mutation p.M392I was detected in two Turkey ALS patients and was considered to be pathogenic.
Frontotemporal dementia -linked mutations in gene ubiquilin 2 encoding autophagy adaptor proteins , indicate that impaired autophagy might cause Frontotemporal dementia.
excess UBQLN2 is toxic rather than protective to neurons and that uncontrolled enhancement of UBQLN2 function is involved in UBQLN2 pathogenesis
Ubiquilin-2 immunostaining - a new marker as a diagnostic supplement in urine cytology?
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 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 most evidently bound to HSP70 (show HSP70 ELISA Kits)-type chaperones, UBQLN1 (show UBQLN1 ELISA Kits) and UBQLN4 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 ELISA Kits) activation and cytosolic TDP-43 (show TARDBP ELISA Kits) 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 ELISA Kits) and -2 in human brain tissue sections, where accumulation was observed in huntingtin (show HTT ELISA Kits) 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