Ubiquilin 2 (Ubiquilin-2, UBQLN2, Chap1, DSK2 Homolog, HRIHFB2157, N4BP4, Protein Linking IAP with Cytoskeleton 2, PLIC2, PLIC-2, hPLIC-2, Ubiquitin-like Product Chap1/Dsk2)

Size

£544.00

SKU:: 031434
Purity:: Purified by Protein G affinity chromatography.
Storage Conditions:: -20°C
Supplier:: United States Biological
Host:: Mouse
Immunogen:: Full length recombinant protein corresponding to human ubiquilin 2 expressed in E. coli.
Clone:: 6H9
Format:: Supplied as a liquid in PBS, 5mM sodium azide, 50% glycerol.
Extra Details:: 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. C9orf72 hexonucleotide expansion is the most commmon cause to date of familial ALS and FTLD. 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. The HGNC name for this protein is UBQLN2. Applications: Suitable for use in Immunofluorescence, Immunocytochemistry, Immunohistochemistry and Western Blot. Other applications not tested. Recommended Dilution: Immunofluorescence (IC): 1:1000 Immunohistochemistry: 1:1000 Western Blot: 1:1000-1:2000 Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Shipping Conditions:: Blue Ice