UBQLN2 Antibody, FITC conjugated

說明書

中文名稱：

UBQLN2兔多克隆抗體, FITC偶聯
貨號：

CSB-PA883393LC01HU
規格：

￥880
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) UBQLN2 Polyclonal antibody
Uniprot No.：

Q9UHD9
基因名：

UBQLN2
別名：

UBQLN2 antibody; N4BP4 antibody; PLIC2 antibody; HRIHFB2157Ubiquilin-2 antibody; Chap1 antibody; DSK2 homolog antibody; Protein linking IAP with cytoskeleton 2 antibody; PLIC-2 antibody; hPLIC-2 antibody; Ubiquitin-like product Chap1/Dsk2 antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Ubiquilin-2 protein (473-542AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

FITC
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
產品提供形式：

Liquid
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

功能：

Plays an important role in the regulation of different protein degradation mechanisms and pathways including ubiquitin-proteasome system (UPS), autophagy and the endoplasmic reticulum-associated protein degradation (ERAD) pathway. Mediates the proteasomal targeting of misfolded or accumulated proteins for degradation by binding (via UBA domain) to their polyubiquitin chains and by interacting (via ubiquitin-like domain) with the subunits of the proteasome. Plays a role in the ERAD pathway via its interaction with ER-localized proteins FAF2/UBXD8 and HERPUD1 and may form a link between the polyubiquitinated ERAD substrates and the proteasome. Involved in the regulation of macroautophagy and autophagosome formation; required for maturation of autophagy-related protein LC3 from the cytosolic form LC3-I to the membrane-bound form LC3-II and may assist in the maturation of autophagosomes to autolysosomes by mediating autophagosome-lysosome fusion. Negatively regulates the endocytosis of GPCR receptors: AVPR2 and ADRB2, by specifically reducing the rate at which receptor-arrestin complexes concentrate in clathrin-coated pits (CCPs).
基因功能參考文獻：
1. The genome of Drosophila contains a single UBQLN homolog (dUbqn) that shows high similarity to UBQLN1 and UBQLN2; therefore, the fly is a useful model for characterizing the role of UBQLN in vivo in neurological disorders affecting locomotion and learning abilities. PMID: 29247619
2. Its PXX domain missense mutations linked to amyotrophic lateral sclerosis and atypical hereditary spastic paraplegia phenotype through defective HSP70-mediated proteolysis. PMID: 28716533
3. Its mutation is a cause of amyotrophic lateral sclerosis in New Zealand. PMID: 27480424
4. Ubiquilins are a family of chaperones for cytosolically exposed transmembrane domains and use ubiquitin to triage clients for degradation via coordinated intra- and intermolecular interactions. PMID: 27345149
5. We analyzed mutations in the UBQLN2 gene in a Chinese cohort with sporadic ALS (sALS). A novel missense mutation was detected in one sALS patient. The p.M392V mutation substitutes a highly conserved residue, has not been reported in the population databases, and previously, at the same residue, a missense mutation p.M392I was detected in two Turkey ALS patients and was considered to be pathogenic. PMID: 28125704
6. Frontotemporal dementia -linked mutations in gene ubiquilin 2 encoding autophagy adaptor proteins , indicate that impaired autophagy might cause Frontotemporal dementia. PMID: 27166223
7. excess UBQLN2 is toxic rather than protective to neurons and that uncontrolled enhancement of UBQLN2 function is involved in UBQLN2 pathogenesis PMID: 27456931
8. Ubiquilin-2 immunostaining - a new marker as a diagnostic supplement in urine cytology? PMID: 27168037
9. UBQLN2 is specifically expressed in the urine of urothelial carcinoma patients. PMID: 26303000
10. Results showed that UBQLN2 is selectively recruited to nuclear inclusions in Huntington's disease but not spinocerebellar ataxia type 3 PMID: 26141599
11. These findings provide a molecular basis for the development of ALS/FTD-associated proteinopathy and establish novel therapeutic targets for ALS. PMID: 26944018
12. Mutations in UBQLN2 gene cause dominant inheritance of amyotrophic lateral sclerosis due to Defective Proteasome Delivery. PMID: 26075709
13. UBQLN2 may be a new molecular target for chemotherapeutics and a useful clinicopathological marker in human osteosarcoma. PMID: 25672654
14. ALS-linked mutations in ubiquilin-2 or hnRNPA1 reduce interaction between ubiquilin-2 and hnRNPA1 PMID: 25616961
15. A single putative mutation of UBQLN2 in a cohort of patients with front temporal lobar degeneration was found. PMID: 25179229
16. Data indicate cognitive deficits in mutant ubiquilin 2 protein UBQLN2P497H transgenic mice. PMID: 25246588
17. Causative mutation in the UBQLN2 gene is rare in Korean patients with either familial or sporadic ALS. PMID: 24684794
18. As ubiquilin-2-positive inclusions are identified in brain, this mutant peptide predisposes to protein misfolding and accumulation. PMID: 24771548
19. The P506S mutation in UBQLN2 can affect both males and females with frontotemporal dementia/amyotrophic lateral sclerosis (FTD/ALS). PMID: 23944734
20. no evidence for involvement of ubiquilin 2 in tau pathology PMID: 24086754
21. The dtat of study suggest that UBQLN2 is generally involved in the pathogenesis of ALS. PMID: 24085347
22. Its mutations are not frequent cause of amyotrophic lateral sclerosis in Ireland. PMID: 23973441
23. results were confirmed by similar findings for ubiquilin-1 and -2 in human brain tissue sections, where accumulation was observed in huntingtin inclusions PMID: 23774650
24. Genetic variations in UBQLN2 in a predominantly Flanders-Belgian cohort of frontotemporal lobar degeneration patients are extremely rare. PMID: 23312802
25. Its mutations related to ALS/FTLD are extremely rare in French FTLD and FTLD-ALS patients. PMID: 23582661
26. No causative mutations within the PXX domain of the UBQLN2 gene are found in familial frontotemoral dementia patients. PMID: 22729385
27. data support the role of the UBQLN2 gene in the pathogenesis of FALS, being conversely a rare genetic cause in SALS even when complicated by FTD. PMID: 23138764
28. This study reported 3 novel UBQLN2 mutations, accounting for 1.2% (2/161) ALS and 2.2% (1/45) FTD patients, including a patient with pure FTD. PMID: 22892309
29. A novel missense UBQLN2 mutation (c.1460C>T, p.T487I) was identified in 2 apparently unrelated multigenerational amyotrophic lateral sclerosis families with no evidence of frontotemporal dementia. This mutation segregated with the disease. PMID: 22717235
30. The results of this study support support a causative role of the UBQLN2 gene in the pathogenesis of ALS and suggest that UBQLN2 mutations are rare in the French and French-Canadian population. PMID: 22560112
31. The results of this study suggested that UBQLN2 was not found to be a cause of familial ALS in the Netherlands. PMID: 22676852
32. Found a pathophysiological link between C9ORF72 expansions and ubiquilin-2 (UBQLN) proteins in amyotrophic lateral sclerosis and frontotemporal lobar degeneration that is associated with a highly characteristic pattern of UBQLN pathology. PMID: 22426854
33. The results of this study suggested that UBQLN2 gene mutations are rare in French amyotrophic lateral sclerosis. PMID: 22169395
34. findings link abnormalities in ubiquilin 2 to defects in the protein degradation pathway, abnormal protein aggregation and neurodegeneration, indicating a common pathogenic mechanism that can be exploited for therapeutic intervention PMID: 21857683
35. solution structure of the ubl domain of hPLIC-2 PMID: 11827521
36. hPLIC-2 interferes with the ubiquitin-mediated proteolysis of p53 and interacts with proteasomes PMID: 12972570
37. Ubiquilin is capable of forming dimers. Dimerization requires the central region of ubiquilin, but not its UBL or the UBA domains. Monomeric ubiquilin is likely to be the active form that is involved in binding presenilin proteins. PMID: 16813565
38. hHR23a and hPLIC2 interact via UBL/UBA domain interactions PMID: 17098253
39. Ubiquitin-like protein PLIC-2 is identified as a negative regulator of G protein-coupled receptor endocytosis. PMID: 18199683
40. siRNA-mediated UBQLN2 depletion made cells more susceptible to starvation-induced cell death. UBQLN2 regulates cell survival during starvation. PMID: 19148225
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相關疾病：

Amyotrophic lateral sclerosis 15, with or without frontotemporal dementia (ALS15)
亞細胞定位：

Cytoplasm. Nucleus. Membrane. Cytoplasmic vesicle, autophagosome.
數據庫鏈接：

HGNC: 12509

OMIM: 300264

KEGG: hsa:29978

STRING: 9606.ENSP00000345195

UniGene: Hs.179309

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