Ubiquilin-2 (UBQLN2) is a 624-amino acid multi-domain adaptor protein and a member of the ubiquilin family of proteins that regulate the degradation of ubiquitinated proteins by the ubiquitin-proteasome system (UPS), autophagy and the endoplasmic reticulum-associated protein degradation (ERAD) pathway. Ubiquilins are characterised by the presence of an N-terminal ubiquitin-like domain and a C-terminal ubiquitin-associated domain. The central portion is highly variable.
UBQLN2 Mediates the proteasomal targeting of misfolded or accumulated proteins for degradation by binding to their polyubiquitin chains, through the ubiquitin-associated domain (UBA) and by interacting with the subunits of the proteasome through the ubiquitin-like domain (ULD).
Mutations in UBQLN2 are associated with Amyotrophic Lateral Sclerosis with most ALS-linked mutations localised to the proline-rich repeat (Pxx) region that is unique to ubiquilin-2 and not present in the other members of the ubiquilin protein family. UBQLN2 has also been shown to bind the ATPase domain of the Hsp70-like Stch protein. Mutations in UBQLN2 are also observed in familial ALS (FALS) cases associated with aberrant TDP-43 inclusions.
Product Type:
NS Reagents Antibody
Antibody Type:
Polyclonal
Format:
100 µg in 100 µl PBS containing 0.02% sodium azide.
Rat, Golden hamster, Guinea Pig, Pig, Horse, Ord's kangaroo rat, Cat, Dog
If you would like us to check if this antibody is likely to bind to this protein from a different species please contact us. We are happy to check for you.
Immunogen:
Partial length recombinant human UBQLN2 from the N-terminal region
If you would like further information regarding the immunogen used in the productiion of this antibody or have a query about whether this antibody will bind to your protein/species please contact us and we can do the analysis for you.
Ubiquilin-2 (UBQLN2) is a 624-amino acid multi-domain adaptor protein and a member of the ubiquilin family of proteins that regulate the degradation of ubiquitinated proteins by the ubiquitin-proteasome system (UPS), autophagy and the endoplasmic reticulum-associated protein degradation (ERAD) pathway. Ubiquilins are characterised by the presence of an N-terminal ubiquitin-like domain and a C-terminal ubiquitin-associated domain. The central portion is highly variable.
UBQLN2 Mediates the proteasomal targeting of misfolded or accumulated proteins for degradation by binding to their polyubiquitin chains, through the ubiquitin-associated domain (UBA) and by interacting with the subunits of the proteasome through the ubiquitin-like domain (ULD).
Mutations in UBQLN2 are associated with Amyotrophic Lateral Sclerosis with most ALS-linked mutations localised to the proline-rich repeat (Pxx) region that is unique to ubiquilin-2 and not present in the other members of the ubiquilin protein family. UBQLN2 has also been shown to bind the ATPase domain of the Hsp70-like Stch protein. Mutations in UBQLN2 are also observed in familial ALS (FALS) cases associated with aberrant TDP-43 inclusions.
Rat, Golden hamster, Guinea Pig, Pig, Horse, Ord's kangaroo rat, Cat, Dog
If you would like us to check if this antibody is likely to bind to this protein from a different species please contact us. We are happy to check for you.
Immunogen:
Partial length recombinant human UBQLN2 from the N-terminal region
If you would like further information regarding the immunogen used in the productiion of this antibody or have a query about whether this antibody will bind to your protein/species please contact us and we can do the analysis for you.
Ubiquilin 2 (also known as PLIC2 and Chap1) is a member of the ubiquilin protein family, which regulate the degradation of cellular proteins through proteasome or autophage-like pathways (1, 2, 3). Humans have four ubiquilin genes, each encoding a separate protein referred to as Ubiquilin 1, 2, 3 and 4. All ubiquilins contain an N-terminal ubiquitin-like (UBL) domain and a C-terminal ubiquitin-associated (UBA) domain, while the central part of the molecules are highly variable. The UBL domains bind subunits of the proteasome, and the UBA domains binds to polyubiquitin chains that are typically conjugated onto proteins marked for proteosomal degradation (1). Ubiquilin 2 has a unique region close to the C terminus containing 12 PXX tandem collagen like repeats, where P is proline and X is most cases valine, glycine, isoleucine or threonine. Teepu Siddique and his collaborators have identified mutations in the ubiquilin 2 gene leading to protein point mutations which were important contributors to several forms of amyotrophic lateral sclerosis (ALS) and Frontotemporal lobar degeneration (FTLD). Interestingly, these mutations involved alterations in proline residues in the PXX repeat region (P497H, P497S, P506T, P509S and P525S, ref. 4). Recently, the Lee and Trojanowski group investigated C9orf72 hexanucleotide expansion and ubiquilin 2 pathology in patients with ALS and FTLD by genetic analysis and immunohistochemistry and found distinct ubiquilin 2 pathology in ALS and FTLD-TDP with C9orf72 expansion (5). C9orf72 hexonucleotide expansion is the most commmon cause to date of familial ALS and FTLD (6, 7). Ubiquilin 2 protein is of different molecular size in mouse and human, 638 and 624 amino acids respectively. As a result the mouse protein, endogenously expressed in rodent 3T3 cells, runs on SDS-PAGE and western blots slightly slower than the human protein.
Product Type:
Antibody
Antibody Type:
Monoclonal
Format:
Lyophilized from PBS containing 5% trehalose and 0.5% sodium azide.
Storage Temp:
Aliquot and store at -20 °C for up to six months after date of receipt. Avoid freeze-thaw cycles.
Host Animal:
Mouse
Species Reactivity:
Mouse
Immunogen:
Recombinant human ubiquilin 2 expressed and purified from E. coli.
Western Blotting (WB) and Immunocytochemistry (IC). A dilution of 1:1,000 - 1:2,000 is recommended for WB. A dilution of 1:500-1:1,000 is recommended for IC. Biosensis recommends optimal dilutions/concentrations should be determined by the end user.
Accession Number:
Q9UHD9
Reconstitution:
Reconstitute with 100 uL sterile water.
Shelf Life:
12 months after purchase unopened.
Specificity:
In primary mouse neuron and glia cell culture, endogenous ubiquilin 2 appears as a weak band at 68kDa in all tranduced and non-transduced cells, indicating low endogenous expression of mouse ubiquilin 2. Strong bands are seen in cells transduced with human wild type or mutant ubiquilin 2. Small proteins which run at 50 kDa in these cells are the fragments of ubiquilin 2. Note, ubiquilin 2 runs at ~66 kDa in human Hela cells and 68 kDa in rodent 3T3 cells. The antibody has also been used successfully for immunocytochemistry. |
Plays an important role in the regulation of different protein degradation mechanisms and pathways including ubiquitin-proteasome system (UPS), autophagy and the endoplasmic reticulum-associated protein degradation (ERAD) pathway. Mediates the proteasomal targeting of misfolded or accumulated proteins for degradation by binding (via UBA domain) to their polyubiquitin chains and by interacting (via ubiquitin-like domain) with the subunits of the proteasome (PubMed:10983987). Plays a role in the ERAD pathway via its interaction with ER-localized proteins FAF2/UBXD8 and HERPUD1 and may form a link between the polyubiquitinated ERAD substrates and the proteasome (PubMed:24215460, PubMed:18307982). Involved in the regulation of macroautophagy and autophagosome formation; required for maturation of autophagy-related protein LC3 from the cytosolic form LC3-I to the membrane-bound form LC3-II and may assist in the maturation of autophagosomes to autolysosomes by mediating autophagosome-lysosome fusion (PubMed:19148225, PubMed:20529957). Negatively regulates the endocytosis of GPCR receptors: AVPR2 and ADRB2, by specifically reducing the rate at which receptor-arrestin complexes concentrate in clathrin-coated pits (CCPs) (PubMed:18199683).
UBQLN2 Antibody detects endogenous levels of total UBQLN2
Immunogen:
A synthesized peptide derived from human UBQLN2
Cross Reactivity:
Human,Mouse,Rat
Conjugate:
Unconjugated
purification:
The antiserum was purified by peptide affinity chromatography using SulfoLink(TM) Coupling Resin (Thermo Fisher Scientific).
Concentration:
1mg/ml
Fragment:
Fab fragment
Buffer:
Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.Store at -20 °C.Stable for 12 months from date of receipt
Plays an important role in the regulation of different protein degradation mechanisms and pathways including ubiquitin-proteasome system (UPS), autophagy and the endoplasmic reticulum-associated protein degradation (ERAD) pathway. Mediates the proteasomal targeting of misfolded or accumulated proteins for degradation by binding (via UBA domain) to their polyubiquitin chains and by interacting (via ubiquitin-like domain) with the subunits of the proteasome (PubMed:10983987). Plays a role in the ERAD pathway via its interaction with ER-localized proteins FAF2/UBXD8 and HERPUD1 and may form a link between the polyubiquitinated ERAD substrates and the proteasome (PubMed:24215460, PubMed:18307982). Involved in the regulation of macroautophagy and autophagosome formation; required for maturation of autophagy-related protein LC3 from the cytosolic form LC3-I to the membrane-bound form LC3-II and may assist in the maturation of autophagosomes to autolysosomes by mediating autophagosome-lysosome fusion (PubMed:19148225, PubMed:20529957). Negatively regulates the endocytosis of GPCR receptors: AVPR2 and ADRB2, by specifically reducing the rate at which receptor-arrestin complexes concentrate in clathrin-coated pits (CCPs) (PubMed:18199683).
UBQLN2 Antibody detects endogenous levels of total UBQLN2
Immunogen:
A synthesized peptide derived from human UBQLN2
Cross Reactivity:
Human,Mouse,Rat
Conjugate:
Unconjugated
purification:
The antiserum was purified by peptide affinity chromatography using SulfoLink(TM) Coupling Resin (Thermo Fisher Scientific).
Concentration:
1mg/ml
Fragment:
Fab fragment
Buffer:
Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.Store at -20 °C.Stable for 12 months from date of receipt
Gene ID:
29978
Gene:
UBQLN2
Swiss-Prot:
Q9UHD9
Molecular Weight:
66 kDa
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