Ubiquilin 2 Antikörper

Produktnummer ABIN1580461

Dieses Maus Monoklonal-Antikörper erkennt spezifisch Ubiquilin 2 in WB, IHC, IF und ICC. Er zeigt eine Reaktivität gegenüber Human und Maus.

Kurzübersicht für Ubiquilin 2 Antikörper (ABIN1580461)

Target: Ubiquilin 2 (UBQLN2)

Reaktivität: Human, Maus

Wirt: Maus

Klonalität: Monoklonal

Konjugat: Dieser Ubiquilin 2 Antikörper ist unkonjugiert

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

Klon: 6H9

Produktdetails anti-Ubiquilin 2 Antikörper

Aufreinigung: affinity purified antibody

Isotyp: IgG1

Anwendungsinformationen

Applikationshinweise: 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

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 mg/mL

Konservierungsmittel: Sodium azide

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

Handhabung: Avoid repeated freezing and thawing.

Lagerung: 4 °C/-20 °C

Informationen zur Lagerung: Store at 4°C short term or -20°C long term.

Details zu Ubiquilin 2

Target: Ubiquilin 2 (UBQLN2)

Andere Bezeichnung: Ubiquilin 2

Hintergrund: 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.