Product Details
Catalog Number: MO22131
Applications: WB, IF
Type: Mouse IgG
Dilutions: ICC 1:100,000; IHC 1:100,000 WB 1:100,000
Immunogen: Human ubiquilin 2 expressed in and purified from E. coli
Storage: Antibody can also be aliquotted and stored frozen at -20° C to -70° C in a manual defrost freezer for six months without detectable loss of activity. The antibody can be stored at 2° - 8° C for 1 month without detectable loss of activity. Avoid repeated freeze-thaw cycles
Format A: Affinity Purified
Format B: liquid
Species Reactivity: Human, Mouse
Downloads: Datasheet (pdf)
Product Sizes
SizeList PricePriceCart
100 ul$295.00$245.00Add to Cart

Ubiquilin 2, also known as UBQLN2, PLIC2 and Chap1, is a member of the ubiquilin protein family, which regulate the degradation of cellular proteins through proteasome or autophage-like pathways. 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.

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*). 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.** *Nature Aug 21;477(7363):211-5 2011). **Acta Neuropathol. Jun;123 (6):825-39 2012.

Images

HeLa cells stained with Ubiquilin 2 (green), and counterstained with chicken polyclonal Vimentin antibody (CH22108) to (red) and DNA (blue). The antibody reveals strong nuclear lamina staining, while the Vimentin antibody reveals cytoplasmic intermediate filaments. Protocol on datasheet.

HeLa cells stained with Ubiquilin 2 (green), and counterstained with chicken polyclonal Vimentin antibody (CH22108) to (red) and DNA (blue). The antibody reveals strong nuclear lamina staining, while the Vimentin antibody reveals cytoplasmic intermediate filaments. Protocol on datasheet.

Western blot analysis of untransfected primary mouse neuron and glia cell cultures (lane 1), the same cells transduced with human Ubiquilin 2 wild type (lane 2), with Ubiquilin 2 P506T mutant (lane 3), with Ubiquilin 2 P497S mutant (lane 4) and with enchanced GFP control (lane 5), all probed with this antibody. Protocol on datasheet.

Western blot analysis of untransfected primary mouse neuron and glia cell cultures (lane 1), the same cells transduced with human Ubiquilin 2 wild type (lane 2), with Ubiquilin 2 P506T mutant (lane 3), with Ubiquilin 2 P497S mutant (lane 4) and with enchanced GFP control (lane 5), all probed with this antibody. Protocol on datasheet.