MATR3 Recombinant Monoclonal Antibody

說明書

中文名稱：

MATR3 Recombinant Monoclonal Antibody
貨號：

CSB-RA275545A0HU
規格：

￥1320
圖片：
Flow cytometric analysis of matrin 3 expression in HAP-1 cells using matrin 3 antibody. Green, isotype control; red, matrin 3.

Immunocytochemical staining of HAP-1 cells with Matrin 3 antibody. Nuclei were stained blue with DAPI; Matrin 3 was stained magenta with Alexa Fluor? 647. Images were taken using Leica stellaris 5. Protein abundance based on laser Intensity and smart gain: Medium. Scale bar, 20 μm.

Western blotting analysis using matrin 3 antibody. Total cell lysates (30 μg) from various cell lines were loaded and separated by SDS-PAGE. The blot was incubated with matrin 3 antibody and HRP-conjugated goat anti-rabbit secondary antibody respectively.
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

Uniprot No.：

P43243
基因名：

MATR3
別名：

Matrin 3; KIAA0723; VCPDM; Matrin-3; MGC9105; MPD2; Vocal Cord And Pharyngeal Weakness With Distal Myopathy; Myopathy, Distal 2; ALS21
反應種屬：

Human, Mouse, Rat
免疫原：

Recombinant Human MATR3 protein
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
克隆類型：

Monoclonal
抗體亞型：

Rabbit IgG
純化方式：

Affinity-chromatography
克隆號：

17G1
濃度：

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

Rabbit IgG in PBS (pH 7.4) containing 50% glycerol, and 0.02% sodium azide.
產品提供形式：

Liquid
應用范圍：

ELISA, WB, FC, ICC

推薦稀釋比：

Application	Recommended Dilution
WB	1:1000-1:5000
FC	1:200-1:2000
ICC	1:100-1:1000

Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Flow cytometry (FC) 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：

May play a role in transcription or may interact with other nuclear matrix proteins to form the internal fibrogranular network. In association with the SFPQ-NONO heteromer may play a role in nuclear retention of defective RNAs. Plays a role in the regulation of DNA virus-mediated innate immune response by assembling into the HDP-RNP complex, a complex that serves as a platform for IRF3 phosphorylation and subsequent innate immune response activation through the cGAS-STING pathway. May bind to specific miRNA hairpins.
基因功能參考文獻：
1. Depletion of SAFB1 reduced FUS's localization to chromatin-bound fraction and splicing activity, suggesting SAFB1 could tether FUS to chromatin compartment thorough N-terminal DNA-binding motif. Moreover, FUS interacts with another nuclear matrix-associated protein, Matrin3. PMID: 27731383
2. Using a novel RNA pulldown approach that utilized endogenous S1-tagged PINCR (a p53-regulated long noncoding RNA), the authors show that PINCR associates with the enhancer region of these genes by binding to RNA-binding protein Matrin 3 that, in turn, associates with p53. PMID: 28580901
3. Matrin3 physically interacts with intronic pyrimidine-rich sequences and controls alternative splicing. PMID: 28695676
4. It may not be a common genetic factor in Chinese amyotrophic lateral sclerosis patients. PMID: 26708275
5. Its mutations strengthens the role of RNA metabolism in amyotrophic lateral sclerosis etiology. PMID: 26493020
6. A missense mutation in MATR3 was identified in myopathy patients undergoing a needle electromyography. PMID: 26899464
7. Our findings indicate that mutations in Matrin 3 that are associated with ALS and myopathy do not dramatically alter the normal localization of the protein or readily induce inclusion formation. PMID: 26528920
8. Three-dimensional mapping of the lamin A-matrin-3 interface showed that the LMNA truncating mutation Delta303, which lacks the matrin-3 binding domain, was associated with an increased distance between lamin A and matrin-3. PMID: 25948554
9. Suppressing Matrin 3 powers a heightened and broader ZAP restriction of HIV-1 gene expression. PMID: 26129669
10. MATR3 mutation is identified to be a possible cause of amyotrophic lateral sclerosis. PMID: 25771394
11. No mutations were identified, indicating that MATR3 mutations are not a common cause of Amyotrophic lateral sclerosis in Australian familial cases with predominately European ancestry PMID: 25523636
12. MATR3 gene disruption is associated with bicuspid aortic valve, aortic coarctation and patent ductus arteriosus. PMID: 25574029
13. Mutations in MATR3 are rare in French familial ALS and ALS with FTLD patients. PMID: 25158920
14. This study shows a high risk of abnormal respiratory function with progressive worsening in MATR3 myopathy. PMID: 25677933
15. Nuclear matrix protein Matrin3 regulates alternative splicing and forms overlapping regulatory networks with PTB. PMID: 25599992
16. the pY RNA1-s2/Matr3 interaction could play a role in vision PMID: 24558381
17. Sixteen patients from 6 families with late onset distal myopathy associated with the p.S85C MATR3 mutation were characterized. PMID: 25154462
18. This study identified mutations in MATR3 in ALS kindreds. also observed MATR3 pathology in ALS-affected spinal cords with and without MATR3 mutations. PMID: 24686783
19. Data suggest that the cellular level of MATR3, known to be highly regulated, modulates the stability of a group of gene transcripts. PMID: 21858232
20. Rev needs MATR3 to promote the cytoplasmic accumulation and translation of unspliced RRE-containing mRNA. PMID: 21771348
21. MATR3 binds viral RNA and is required for the Rev/RRE mediated nuclear export of unspliced HIV-1 RNAs. PMID: 21771346
22. Matrin 3 binds Rev RNA to stabilize HIV-1 transcripts leading to increased cytoplasmic expression. PMID: 21771347
23. MATR3 to be a novel ATM target in response to DNA damage. PMID: 20421735
24. matrin 3 plays a significant role in controlling cell growth and proliferation PMID: 21182838
25. study of the association of matr3 with chromosome territories and identification of potential interacting proteins PMID: 19562669
26. Data show that the nuclear matrix protein matrin 3, cytoskeletal motor protein HMP, and the circadian clock protein lark were significantly decreased in fetal Down syndrome brain. PMID: 12469345
27. These results suggest that the functions of matrin 3 could be regulated by both Ca(2+)-dependent interaction with calmodulin and caspase-mediated cleavage. PMID: 17658460
28. the spatial proximities among a constellation of functionally related sites that are found within euchromatic regions of the cell nucleus including: HP1gamma, RNA polymerase II, matrin 3, and SAF-A sites PMID: 18618731
29. nonconservative S85C missense mutation in vocal cord and pharyngeal weakness with distal myopathy PMID: 19344878
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相關疾病：

Amyotrophic lateral sclerosis 21 (ALS21)
亞細胞定位：

Nucleus matrix.
數據庫鏈接：

HGNC: 6912

OMIM: 164015

KEGG: hsa:9782

STRING: 9606.ENSP00000354346

UniGene: Hs.268939

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