Mouse monoclonal antibody to Ubiquilin 2

ZZZZ_Status (Active, Disc) : Active

 

AA_Supplier Name : Biosensis

 

Alternative_Name (Other Names) :

 

Antibody_Isotype
(Antibody only) : IgG1

 

Application_Details :

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.

 

Background_Info :

 

Clone_Name
(AB only, monoclonals) : 6H9

 

Datasheet_Link : http://www.biosensis.com/pdf_datasheet.php?products_id=1234

 

Description (Long Description) : 

  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.

 

Expiry_Date : 

12 months after purchase unopened.

 

Format (Form) : 

Lyophilized from PBS containing 5% trehalose and 0.5% sodium azide.

 

Immunogen (Antigen) :Recombinant human ubiquilin 2 expressed and purified from E. coli.

 

Kit_Components :

 

MSDS (File name) :