Ubiquilin 2 antibody

Catalog No. ABIN1580461

Product Details anti-Ubiquilin 2 Antibody

Target: Ubiquilin 2 (UBQLN2)

Reactivity: Human, Mouse

Host: Mouse

Clonality: Monoclonal

Conjugate: This Ubiquilin 2 antibody is un-conjugated

Application: Western Blotting (WB), Immunohistochemistry (IHC), Immunofluorescence (IF), Immunocytochemistry (ICC)

Purification: affinity purified antibody

Clone: 6H9

Isotype: IgG1

Application Details

Application Notes: Try at dilutions of ~1:1,000 for immunofluorescence. For western blots try at 1:1,000-2,000. A suitable control tissue is HeLa cell lysates. The ubiqulin 2 protein runs at about ~66 kDa (human) and 68 kDa (mouse) on SDS-PAGE gels

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 mg/mL

Preservative: Sodium azide

Precaution of Use: This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Handling Advice: Avoid repeated freezing and thawing.

Storage: 4 °C/-20 °C

Storage Comment: Store at 4°C short term or -20°C long term.

Target Details for Ubiquilin 2

Target: Ubiquilin 2 (UBQLN2)

Alternative Name: Ubiquilin 2 (UBQLN2 Products)

Synonyms: ALS15 antibody, CHAP1 antibody, DSK2 antibody, N4BP4 antibody, PLIC2 antibody, Chap1 antibody, Dsk2 antibody, HRIHFB2157 antibody, Plic-2 antibody, Plic2 antibody, RGD1563566 antibody, UBQLN2 antibody, ubiquilin 2 antibody, UBQLN2 antibody, Ubqln2 antibody

Background: 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. 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. 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. C9orf72 hexonucleotide expansion is the most common 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. Our antibody MCA-6H9 was raised against human ubiquilin 2 expressed in and purified from E. coli. The HGNC name for this protein is UBQLN2.