Recombinant Human Nucleoporin GLE1 (GLE1)

中文名稱(chēng)：

人GLE1重組蛋白
貨號(hào)：

CSB-YP700651HU
說(shuō)明書(shū)：

MSDS Datasheet
規(guī)格：
來(lái)源：

Yeast
其他：

中文名稱(chēng)：

人GLE1重組蛋白
貨號(hào)：

CSB-EP700651HU
說(shuō)明書(shū)：

MSDS Datasheet
規(guī)格：
來(lái)源：

E.coli
其他：

中文名稱(chēng)：

人GLE1重組蛋白
貨號(hào)：

CSB-EP700651HU-B
說(shuō)明書(shū)：

MSDS Datasheet
規(guī)格：
來(lái)源：

E.coli
共軛：

Avi-tag Biotinylated
E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.
其他：

中文名稱(chēng)：

人GLE1重組蛋白
貨號(hào)：

CSB-BP700651HU
說(shuō)明書(shū)：

MSDS Datasheet
規(guī)格：
來(lái)源：

Baculovirus
其他：

中文名稱(chēng)：

人GLE1重組蛋白
貨號(hào)：

CSB-MP700651HU
說(shuō)明書(shū)：

MSDS Datasheet
規(guī)格：
來(lái)源：

Mammalian cell
其他：

產(chǎn)品詳情
產(chǎn)品評(píng)價(jià)
相關(guān)產(chǎn)品
靶點(diǎn)詳情

產(chǎn)品詳情

純度：

>85% (SDS-PAGE)
基因名：

GLE1
Uniprot No.：

Q53GS7
別名：

GLE 1; GLE1; GLE1 like protein; GLE1 like RNA export mediator; GLE1 RNA export mediator homolog; GLE1 RNA export mediator like (yeast); GLE1-like protein; GLE1_HUMAN; GLE1L; hGLE1; LCCS 1; LCCS; LCCS1; Nucleoporin GLE1
種屬：

Homo sapiens (Human)
蛋白長(zhǎng)度：

full length protein
表達(dá)區(qū)域：

1-698
氨基酸序列

MPSEGRCWET LKALRSSDKG RLCYYRDWLL RREDVLEECM SLPKLSSYSG WVVEHVLPHM QENQPLSETS PSSTSASALD QPSFVPKSPD ASSAFSPASP ATPNGTKGKD ESQHTESMVL QSSRGIKVEG CVRMYELVHR MKGTEGLRLW QEEQERKVQA LSEMASEQLK RFDEWKELKQ HKEFQDLREV MEKSSREALG HQEKLKAEHR HRAKILNLKL REAEQQRVKQ AEQERLRKEE GQIRLRALYA LQEEMLQLSQ QLDASEQHKA LLKVDLAAFQ TRGNQLCSLI SGIIRASSES SYPTAESQAE AERALREMRD LLMNLGQEIT RACEDKRRQD EEEAQVKLQE AQMQQGPEAH KEPPAPSQGP GGKQNEDLQV KVQDITMQWY QQLQDASMQC VLTFEGLTNS KDSQAKKIKM DLQKAATIPV SQISTIAGSK LKEIFDKIHS LLSGKPVQSG GRSVSVTLNP QGLDFVQYKL AEKFVKQGEE EVASHHEAAF PIAVVASGIW ELHPRVGDLI LAHLHKKCPY SVPFYPTFKE GMALEDYQRM LGYQVKDSKV EQQDNFLKRM SGMIRLYAAI IQLRWPYGNR QEIHPHGLNH GWRWLAQILN MEPLSDVTAT LLFDFLEVCG NALMKQYQVQ FWKMLILIKE DYFPRIEAIT SSGQMGSFIR LKQFLEKCLQ HKDIPVPKGF LTSSFWRS
蛋白標(biāo)簽：

Tag?type?will?be?determined?during?the?manufacturing?process.

The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
產(chǎn)品提供形式：

Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
復(fù)溶：

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
儲(chǔ)存條件：

Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
保質(zhì)期：

The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
貨期：

Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
注意事項(xiàng)：

Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet ：

Please contact us to get it.

產(chǎn)品評(píng)價(jià)

靶點(diǎn)詳情

功能：

Required for the export of mRNAs containing poly(A) tails from the nucleus into the cytoplasm. May be involved in the terminal step of the mRNA transport through the nuclear pore complex (NPC).
基因功能參考文獻(xiàn)：
1. Pathogenic variants in the GLE1 gene are rare in Chinese ALS patients. PMID: 29398120
2. Data indicate 2 siblings with a homozygous p.I684T mutation in RNA export mediator (GLE1). PMID: 28657126
3. These results imply that DBP5, GLE1 and IP6 have a conserved and individual function in the cytoplasmic mRNA expression. Variations in phenotype are due to the difference in each function of DBP5, GLE1 and IPPK in intracellular mRNA metabolism. PMID: 29746542
4. We identified bi-allelic mutations in GLE1 in two unrelated individuals with motor delays, feeding difficulties, and respiratory insufficiency who survived beyond the perinatal period. Each affected child had missense variants predicted to result in amino acid substitutions near the C-terminus of GLE1 that are predicted to disrupt protein-protein interaction or GLE1 protein targeting. PMID: 28884921
5. It was concluded that the amyotrophic lateral sclerosis-linked Gle1-c.1965-2A>C mutation generates a protein isoform capable of both Gle1A- and Gle1B-ascribed functions, and thereby uncoupled from normal mechanisms of Gle1 regulation. PMID: 26776475
6. We also suggest that lethal congenital contracture syndrome 1 (LCCS1) and lethal arthrogryposis with anterior horn disease (LAAHD), the two AMC subtypes related to GLE1, do not have sufficient clinical or molecular differentiation to be considered allelic disorders. Rather, GLE1 mutations cause a variable spectrum of AMC severity including a non-lethal variant described herein PMID: 27684565
7. Restoration of miR-127-3p and miR-376a-3p counteracts the neoplastic phenotype of giant cell tumor of bone derived stromal cells by targeting COA1, GLE1 and PDIA6. PMID: 26655997
8. Role for Gle1A during stress granule formation and translation regulation during environmental stress responses is examined. PMID: 25694449
9. We report the identification of the first heterozygous mutations in GLE1 ever found to be associated with amyotrophic lateral sclerosis. PMID: 25343993
10. Lethal congenital contracture syndrome 1 and lethal arthrogryposis with anterior horn cell disease are associated with defective Gle1 function during the export of mRNA. [review] PMID: 24275432
11. Report documents a requirement for Gle1 self-association during mRNA export and uncover molecular defects underlying a lethal human disease lethal congenital contracture syndrome-1. PMID: 24243016
12. Dbp5, Gle1-IP6 and Nup159: a working model for mRNP export. PMID: 22064466
13. defective zebrafish GLE1 function in human LCCS1 results in both neurogenic and non-neurogenic defects linked to the apoptosis of proliferative organ precursors PMID: 22357925
14. The unique carboxyl-terminal 43 amino acid region of the hGle1B isoform mediates binding to the C-terminal non-phenylalanine- glycine region of the nucleoporin hCG1/NPL1. PMID: 16000379
15. Mutations in mRNA export mediator GLE1 result in fetal motoneuron disease. PMID: 18204449
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相關(guān)疾病：

Lethal congenital contracture syndrome 1 (LCCS1); Lethal arthrogryposis with anterior horn cell disease (LAAHD)
亞細(xì)胞定位：

Nucleus. Cytoplasm.; [Isoform 1]: Cytoplasm. Nucleus, nuclear pore complex.
蛋白家族：

GLE1 family
數(shù)據(jù)庫(kù)鏈接：

HGNC: 4315

OMIM: 253310

KEGG: hsa:2733

STRING: 9606.ENSP00000308622

UniGene: Hs.522418