GLE1 Antibody, Biotin conjugated

說明書

中文名稱：

GLE1兔多克隆抗體, Biotin偶聯
貨號：

CSB-PA700651ED01HU
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) GLE1 Polyclonal antibody
Uniprot No.：

Q53GS7
基因名：

GLE1
別名：

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

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Nucleoporin GLE1 protein (141-380AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

Biotin
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
應用范圍：

ELISA
Protocols：

ELISA 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：

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：
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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相關疾病：

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

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

GLE1 family
數據庫鏈接：

HGNC: 4315

OMIM: 253310

KEGG: hsa:2733

STRING: 9606.ENSP00000308622

UniGene: Hs.522418

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