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Biosensis/Mouse monoclonal antibody to Ubiquilin 2/M-1656-100/1 Ea
Biosensis/Mouse monoclonal antibody to Ubiquilin 2/M-1656-100/1 Ea
商品编号：
M-1656-100
品牌：
Biosensis inc
市场价：
¥5940.00
美元价：
3564.00
产品分类：
功能性抗体
公司分类：
Functional_antibody
联系Q Q：
3392242852
电话号码：
4000-520-616
电子邮箱：
info@ebiomall.com
商品介绍
| 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. |
| Antigen | Recombinant human ubiquilin 2 expressed and purified from E. coli. |
| Isotype | IgG1 |
| Produced in | Mouse |
| Applications | 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. |
| Specificity | In primary mouse neuron and glia cell culture, endogenous ubiquilin 2 appears as a weak band at 68kDa in all tranduced and non-transduced cells, indicating low endogenous expression of mouse ubiquilin 2. Strong bands are seen in cells transduced with human wild type or mutant ubiquilin 2. Small proteins which run at 50 kDa in these cells are the fragments of ubiquilin 2. Note, ubiquilin 2 runs at ~66 kDa in human Hela cells and 68 kDa in rodent 3T3 cells. The antibody has also been used successfully for immunocytochemistry. |
| Species Against | Human and mouse |
| Antibody Against | Ubiquilin 2 (PLIC2; Chap1) |
| Form | Lyophilized from PBS containing 5% trehalose and 0.5% sodium azide. |
| Reconstitution | Reconstitute with 100 uL sterile water. |
| Storage | Aliquot and store at -20°C for up to six months after date of receipt. Avoid freeze-thaw cycles. |
| Expiry Date | 12 months after purchase unopened. |
| General References | 1. Kleijnen MF, et al. The hPLIC proteins may provide a link between the ubiquitination machinery and the proteasome. Molec. Cell 6: 409-419 (2000). 2. N'Diaye EN, et al. PLIC proteins or ubiquilins regulate autophagy-dependent cell survival during nutrient starvation. EMBO Rep. 10:173-9 (2009). 3. Rothenberg C, Srinivasan D, Mah L, Kaushik S, Peterhoff CM, Ugolino J, Fang S, Cuervo AM, Nixon RA, Monteiro MJ. Ubiquilin functions in autophagy and is degraded by chaperone-mediated autophagy. Hum Mol Genet. Aug 15;19 (16): 3219-32. Epub Jun 7 (2010). 4. Deng HX, et al. Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia. Nature. 2011 Aug 21;477(7363):211-5 5. Brettschneider J, et al. Pattern of ubiquilin pathology in ALS and FTLD indicates presence of c9orf72 hexanucleotide expansion. Acta Neuropathol. 2012 Jun;123(6):825-39 6. Renton AE, Majounie E, Waite AA, et al. Hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD. Neuron. 2011 Oct 20;72(2):257-68 7. DeJesus-Hernandez M, et al. Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Neuron. 2011 Oct 20;72(2):245-56 |
品牌介绍
Biosensis是一家由生命科学家和商业团队组成的公司，致力于为全球学术界和工业界的研究人员提供支持。该团队在基础和应用生命科学研究方面拥有超过100年的经验，并在生命科学研究和诊断市场的生命科学抗体，试剂和试剂盒的商业化方面拥有50多年的经验。
品牌分类
TR-200-BG型
PE-1243-50型
抗体试剂盒
PE-1246-5型
TR-100-BG型
S-1754-500型
神经营养因子
糖尿病与肥胖
细胞内贩运
电话：PE-1239-5
神经肽和受体
B-100-PS型
受体和通道分子
2个平板ELISA试剂盒
M-1763-100英尺
TR-200-BGT公司
电话：PE-1239-2
PE-1242-5型
M-1249-100号
神经营养因子受体
细胞周期调节器
S-1759-100号
PE-1606-100型
PE-1762-100型
B-200-PS型
PE-019-100型
PE-1241-5型
PE-003-100
1个平板ELISA试剂盒
BL-003-1000型
PE-1246-2型
感觉神经系统
PE-1253-100型
TR-100-FJ
BL-005-500型
神经内分泌
M-1728-100型
DNA损伤与修复
PE-001-100型
PE-1608-1型
TR-400-AG公司
PE-1243-25
细胞标记
PE-1608-5型
TR-100-FJT
自噬
PE-1242-2型
其他神经元
神经递质和酶
抗氧化酶
神经元标志物
标记抗体
BL-004-500型
PE-019-25
PE-1608-10型
BL-002-1250型
受体体嵌合体
信令与转录
基质和粘附分子
BL-001-1250型
癌症
PE-019-500
干细胞
连接蛋白
TR-300-AG型
细胞凋亡
PE-1252-100型
生长因子
发展
阿尔茨海默氏症/帕金森氏症
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