VAPB Antibody

說明書

中文名稱：

VAPB兔多克隆抗體
貨號：

CSB-PA025790ESR2HU
規格：

￥440
圖片：
Western blot
All lanes: Vesicle-associated membrane protein-associated protein B/C antibody at 3μg/ml + Mouse brain tissue
Secondary
Goat polyclonal to rabbit IgG at 1/10000 dilution
Predicted band size: 28, 12 kDa
Observed band size: 28 kDa
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) VAPB Polyclonal antibody
Uniprot No.：

O95292
基因名：

VAPB
別名：

VAPB; UNQ484/PRO983; Vesicle-associated membrane protein-associated protein B/C; VAMP-B/VAMP-C; VAMP-associated protein B/C; VAP-B/VAP-C
宿主：

Rabbit
反應種屬：

Human, Mouse
免疫原：

Recombinant Human Vesicle-associated membrane protein-associated protein B/C protein (10-222AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

Antigen Affinity Purified
濃度：

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

PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
產品提供形式：

Liquid
應用范圍：

ELISA, WB

推薦稀釋比：

Application	Recommended Dilution
WB	1:500-1:5000

Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
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：

Participates in the endoplasmic reticulum unfolded protein response (UPR) by inducing ERN1/IRE1 activity. Involved in cellular calcium homeostasis regulation.
基因功能參考文獻：
1. report on a novel mutation of p.Pro56His in VABP found in a Chinese familial amyotrophic lateral sclerosis pedigree and description of the clinical characteristics of the family. PMID: 28993872
2. the component proteins of the machinery, OSBP, VAP, SAC1, and PITPNB, are all essential host factors for AiV replication. Importantly, the machinery is directly recruited to the RNA replication sites through previously unknown interactions of VAP/OSBP/SAC1 with the AiV proteins and with ACBD3. PMID: 29367253
3. Lifelong elevation of neuronal VAPB slowed the decline of neurological impairment, delayed denervation of hindlimb muscles, and prolonged survival of spinal motor neurons. PMID: 28173107
4. Study showed that alpha-synuclein perturbs endoplasmic reticulum-mitochondria associations and that this involves disruption to the VAPB-PTPIP51 tethering proteins. Using a range of assays including immunoprecipitation, cellular glutathione S-transferase pull-down, proximity ligation and in vitro binding of recombinant proteins, study showed that alpha-synuclein is a direct binding partner for VAPB. PMID: 28337542
5. ACBD5-VAPB interaction regulates peroxisome-endoplasmic reticulum associations. Loss of PO-ER association perturbs PO membrane expansion and increases PO movement. PMID: 28108524
6. VAP-ACBD5-mediated contact between the endoplasmic reticulum and peroxisomes mediate organelle maintenance and lipid homeostasis. PMID: 28108526
7. Effects of the combined absence of VAPA and VAPB in human cells were studied; cells lacking VAP accumulate high levels of PI4P, actin comets, and trans-Golgi proteins on endosomes. Such defects are mimicked by downregulation of OSBP, a VAP interactor and PI4P transporter that participates in VAP-dependent endoplasmic reticulum-endosomes tethers. PMID: 27419871
8. this is the first study to report Amyotrophic lateral sclerosis caused by a VAPB mutation in a Chinese population. PMID: 26566915
9. Our work revealed that VAP-A/B knockdown impaired the processing and secretion of PAUF, which is one of the cargo proteins of carriers of the trans-Golgi network to the cell surface. PMID: 26490117
10. Heterozygous P56S Vapb knock-in mice show mild age-dependent defects in motor behaviors as characteristic features of the disease. The homozygous P56S Vapb knock-in mice show more severe defects compared with heterozygous mice reflecting the dominant and dose-dependent effects of P56S mutation. PMID: 26362257
11. VAPB inhibited the degradation of DeltaF508-CFTR in the ER through interactions with the RMA1-Derlin-BAP31-VCP pathway. PMID: 26740627
12. Study characterizes the human VAPB-HCV NS5B interaction and reveals that NS5B C-linker is intrinsically disordered in solution but capable of binding the human VAPB-MSP domain which overlaps with those for binding HCV NS5A and human Eph receptors. PMID: 26784321
13. The VAPB and its interacting partners cooperatively regulate protein trafficking through the ERGIC by modulating PtdIns4P levels. PMID: 26812496
14. Collectively, these results not only lead to a better understanding of hVAPB function but also point to potentially relevant targets for therapeutic intervention. PMID: 25826266
15. results suggest that the binding of vesicle-associated membrane protein-associated protein B(VAP-B) to Rab3 GTPase activating protein 1(RAB3GAP1) is implicated in the regulation of nuclear envelope formation through ER-Golgi intermediate compartment PMID: 25612670
16. P56S-VAPB was found to suppress adipocyte differentiation by promoting the activation of the ATF4-CHOP pathway PMID: 25824044
17. In patients with familial or sporadic amyotrophic lateral sclerosis (ALS) from Portugal, Iceland and Sweden no association is found with disease and VAMP-associated protein type B (VAPB) mutations. PMID: 23971766
18. Findings provide new pathophysiological mechanisms of P56S VAPB that differentially affect the function and survival of corticospinal and spinal motor neurons in familial amyotrophic lateral sclerosis 8. PMID: 23771029
19. genetic screening for this mutation should be performed in all adult patients with lower motor neuron disease, regardless of family history because of the rarity of this disease, physicians often do not suspect it, and many cases may be missed in Brazil. PMID: 24212516
20. Partial or complete loss of VAPB function leads to motor deficit but is unable to trigger a full-blown amyotrophic lateral sclerosis phenotype. PMID: 23446633
21. this discovery provides a mechanism for ALS-causing VAPB mutants/variants to gain novel functions such as to mediate ER structure before significant accumulation of aggregates occurs. PMID: 23333387
22. These results suggest that changes in wild type VAPB do not play a significant role in amyotrophic lateral sclerosis cases that are not caused by VAPB mutations PMID: 23281774
23. Transfection of a dominant-negative form of the AAA ATPase p97/VCP stabilizes mutant VAPB, suggesting a role for this ATPase in extracting the aggregated protein from the inclusions. PMID: 22611258
24. structual basis of VAPC binding to HCV NS5B PMID: 22815741
25. This study screened a cohort of 755 sporadic ALS patients, 111 familial ALS patients (97 families), and 765 control subjects of Dutch descent for mutations in vesicle-associated membrane protein B (VAPB). PMID: 22878164
26. VAPB promotes breast tumor growth by modulation of Akt activity. PMID: 23049696
27. The binding residues have been successfully mapped out on both NS5A and VAPB, thus allowing the construct of the complex structure. PMID: 22720086
28. The mutation in VAPB that causes amyotrophic lateral sclerosis also causes the block of transport of nucleoporins and emerin to the nuclear envelope. PMID: 22454507
29. ALS mutant VAPBP56S perturbs anterograde mitochondrial axonal transport by disrupting Ca(2+) homeostasis and effecting the Miro1/kinesin-1 interaction with tubulin. PMID: 22258555
30. Loss of either VAPB or PTPIP51 perturbs uptake of Calcium by mitochondria and results in amyotrophic lateral sclerosis. PMID: 22131369
31. T46I mutant of the hVAPB MSP domain is associated with amyotrophic lateral sclerosis. PMID: 22069488
32. endoplasmic reticulum stress and corruption of the proteasome function might contribute to the aberrant protein homeostasis associated with hVAPB PMID: 21998752
33. VAPB protein levels are reduced in motor neurons derived form induced pluripotent stem cells of amyotrophic lateral sclerosis patients. PMID: 21685205
34. three conserved prolines in VAPA and Scs2p confers less vulnerability to mutations equivalent to the amyotrophic lateral sclerosis causing mutation as compared with VAPB, which has only two conserved prolines. PMID: 21144830
35. newly identified mutation in human FALS has a pathogenic effect, supporting and reinforcing the role of VAPB as a causative gene of ALS. PMID: 20940299
36. Mutations in SOD1, ANG, VAPB, TARDBP and FUS genes have been identified in amyotrophic lateral sclerosis. PMID: 20577002
37. Adeno-associated viral-mediated over-expression of both wild-type and mutated form of human VAPB selectively induces death of primary mouse motor neurons, albeit with different kinetics. PMID: 20477942
38. study reports the first identification of the p.P56S mutation in the VAPB gene in a non-Brazilian patient PMID: 20447143
39. VAPB mRNA levels were decreased in the spinal cord of ALS patients compared to controls and Expression of VAPB mRNA and protein was predominantly localised to large motor neurones. PMID: 18701194
40. Pro56Ser mutation of VAPB lead to amyotrophic lateral sclerosis by eliminating the native protein structure. PMID: 20377183
41. these results demonstrate that the amyotrophic lateral sclerosis -linked VAPB mutant causes dramatic ER restructuring that may underlie its pathogenicity in motoneurons. PMID: 20008544
42. the mechanism by which VAP-B(P56S) aggregates are formed and induce familial motor neuron diseases PMID: 20207736
43. under conditions of proteasomal inhibition, as encountered in many neurodegenerative diseases including ALS, variant VAPB proteins might accumulate in affected cells and contribute to ALS pathogenesis. PMID: 20227395
44. evidence that Nir (Nir1, Nir2, and Nir3)-VAP-B interactions are mediated through the conserved FFAT (two phenylalanines (FF) in acidic tract) motif present in Nir proteins PMID: 15545272
45. Regulation of ceramide transport protein by oxyste rbinding proteins, sterols, and VAMP reveals a novel mechanism for integrating sterol regulatory signals with ceramide transport and sphingomyelin synthesis in the Golgi apparatus. PMID: 16571669
46. Frequency of the detected exon variation in the VAPB gene was not significantly different between patients and controls. VAPB mutations are not a common cause of adult-onset sporadic amyotrophic lateral sclerosis. PMID: 16729899
47. Overexpression of wild type VAPB promotes unfolded protein response, which is a reaction of the endoplasmic reticulum to suppress accumulation of misfolded proteins. PMID: 16891305
48. it is suggested that VAPB mutations do not significantly contribute to the genetic causes of sporadic amyotrophic lateral sclerosis in the UK and Northern Europe PMID: 17536055
49. P56S mutation in VAP-B may lead to a less stable interaction of this endoplasmic reticulum protein with at least two other proteins: tubulin and GAPDH. PMID: 17540579
50. VAPB is abundant in motor neurons and the P56S substitution causes aggregation of mutant VAPB in immobile tubular ER clusters, perturbs FFAT-motif binding, and traps endogenous VAP in mutant aggregates, which may cause motor neuron degeneration. PMID: 17804640
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相關疾病：

Amyotrophic lateral sclerosis 8 (ALS8); Spinal muscular atrophy, proximal, adult, autosomal dominant (SMAPAD)
亞細胞定位：

Endoplasmic reticulum membrane; Single-pass type IV membrane protein.
蛋白家族：

VAMP-associated protein (VAP) (TC 9.B.17) family
組織特異性：

Ubiquitous. Isoform 1 predominates.
數據庫鏈接：

HGNC: 12649

OMIM: 182980

KEGG: hsa:9217

STRING: 9606.ENSP00000417175

UniGene: Hs.182625

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