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 production 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.
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 production 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.
Mouse anti-Ubiquilin 2 Monoclonal Antibody (Unconjugated), suitable for WB, ICC.
Background Info:
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 0.05% sodium azide.
Host Animal:
Mouse
Species Reactivity:
Human,Mouse
Immunogen:
Recombinant human ubiquilin 2 expressed and purified from E. coli.
Applications:
ICC,WB
Clone number:
6H9
Antibody Isotype:
IgG1
Application Details:
Western Blotting (WB) and Immunocytochemistry (ICC). 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.
Biosensis Brand:
Biosensis®
Conjugate:
Unconjugated
Shelf Life:
12 months after date of receipt (unopened vial).
Use:
For research use only.
References:
Kleijnen MF, et al. The hPLIC proteins may provide a link between the ubiquitination machinery and the proteasome. Molec. Cell 6: 409-419 (2000). N'Diaye EN, et al. PLIC proteins or ubiquilins regulate autophagy-dependent cell survival during nutrient starvation. EMBO Rep. 10:173-9 (2009). Rothenberg C, Srinivasan D, Mah L, Kaushik S, Peterhoff CM, Ugolino J, Fang S, Cuervo AM, Nixon RA, Monteiro MJ. Ubiquilin functions in autophagy and is degraded by chaperone-mediated autophagy. Hum Mol Genet. Aug 15;19 (16): 3219-32. Epub Jun 7 (2010). Deng HX, et al. Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia. Nature. 2011 Aug 21;477(7363):211-5. Brettschneider J, et al. Pattern of ubiquilin pathology in ALS and FTLD indicates presence of c9orf72 hexanucleotide expansion. Acta Neuropathol. 2012 Jun;123(6):825-396. Renton AE, Majounie E, Waite AA, et al. Hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD. Neuron. 2011 Oct 20;72(2):257-687. DeJesus-Hernandez M, et al. Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Neuron. 2011 Oct 20;72(2):245-56
Specificity:
In primary mouse neuron and glia cell culture, endogenous ubiquilin 2 appears as a weak band at 68 kDa 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.
UBQLN2 Antibody detects endogenous levels of total UBQLN2.
Blocking Peptide:
A corresponding control peptide is available for this antibody (please email tech@nktscientific.com for details)
Conjugate:
Unconjugated
Uniprot:
Q9UHD9
Gene:
UBQLN2
Format:
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.
UBQLN2 Antibody detects endogenous levels of total UBQLN2.
Blocking Peptide:
A corresponding control peptide is available for this antibody (please email tech@nktscientific.com for details)
Conjugate:
Unconjugated
Uniprot:
Q9UHD9
Gene:
UBQLN2
Format:
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.
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