Recombinant Human Delta(14)-sterol reductase LBR (LBR), partial

中文名稱(chēng)：

人LBR重組蛋白
貨號(hào)：

CSB-YP613520HU
規(guī)格：
來(lái)源：

Yeast
其他：

中文名稱(chēng)：

人LBR重組蛋白
貨號(hào)：

CSB-EP613520HU
規(guī)格：
來(lái)源：

E.coli
其他：

中文名稱(chēng)：

人LBR重組蛋白
貨號(hào)：

CSB-EP613520HU-B
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LBR重組蛋白
貨號(hào)：

CSB-BP613520HU
規(guī)格：
來(lái)源：

Baculovirus
其他：

中文名稱(chēng)：

人LBR重組蛋白
貨號(hào)：

CSB-MP613520HU
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：

LBR
Uniprot No.：

Q14739
別名：

DHCR 14B; DHCR14B; Integral nuclear envelope inner membrane protein; Lamin-B receptor; LBR; LBR_HUMAN; LMN 2R; LMN2R; MGC9041; PHA; PRO0650
種屬：

Homo sapiens (Human)
蛋白長(zhǎng)度：

Partial
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：

Catalyzes the reduction of the C14-unsaturated bond of lanosterol, as part of the metabolic pathway leading to cholesterol biosynthesis. Plays a critical role in myeloid cell cholesterol biosynthesis which is essential to both myeloid cell growth and functional maturation. Mediates the activation of NADPH oxidases, perhaps by maintaining critical levels of cholesterol required for membrane lipid raft formation during neutrophil differentiation. Anchors the lamina and the heterochromatin to the inner nuclear membrane.
基因功能參考文獻(xiàn)：
1. The authors demonstrate that human LBR is essential for cholesterol synthesis. PMID: 27336722
2. These observations point to a new mechanism regulating the localization of LBR, which is governed by an ELYS-mediated phosphorylation network. PMID: 27802161
3. The primary response of cells to various stresses leading to senescence consists of the down-regulation of LBR and LB1 to attain reversal of the chromatin architecture. PMID: 27760841
4. We have shown here that lamin B receptor (LBR) showed a change in localization in both BrdU-induced and replicative senescent cells. PMID: 27059139
5. Data indicate that proto-oncogene protein c-akt (Akt) phosphorylates distinct sites than SRPK1 protein within the arginine-serine (RS) domain of Lamin B Receptor (LBR). PMID: 27105349
6. Lamin B receptor mRNA expression was directly associated with tumor grade in breast cancer patients(grade 1 vs. grade 3 - 0.00 vs. 0.00; p = 0.0479) and Nottingham Prognostic Index (NPI1 vs. NPI3 - 0.00 vs. 0.00; p = 0.0551). PMID: 24293108
7. Mutation in LBR gene is associated with pelger-huet anomaly and a mild skeletal phenotype. PMID: 23824842
8. Lymphohematopoietic licence: sterol C-14 reductase activity of lamin B receptor (Lbr) is essential for neutrophil differentiation. PMID: 22581323
9. LBR missense mutations can abolish sterol reductase activity, causing lethal Greenberg dysplasia but not Pelger anomaly PMID: 21327084
10. The LBR knockdown cells retain an ovoid shaped nucleus with reduced levels of lamin A/C during in vitro granulopoiesis induced with retinoic acid. PMID: 21327094
11. Our studies reveal for the first time a mechanism that controls the timing of nuclear envelope reassembly through modification of the integral nuclear membrane protein lamin B receptor PMID: 21795390
12. a highly significant gene-dosage effect between the gene copy number and the nuclear segmentation index of neutrophils PMID: 21326950
13. LBR is heavily expressed in papillary thyroid carcinoma cells, but an abnormal folding of the protein might explain its lack of immunohistochemical reactivity and be associated with the anomalous folding of the nuclear membrane. PMID: 21103616
14. LBR targets the membrane precursor vesicles to chromatin by interacting with importin beta in a LBR phosphorylation-dependent manner during the NE assembly at the end of mitosis PMID: 20576617
15. LBR mutant variants and sterol reductases can severely interfere with the regular organization of the nuclear envelope and the endoplasmic reticulum. PMID: 19940018
16. results suggest that LMNA, ZMPSTE24, and LBR sequence variations are not major genetic determinants involved in scleroderma pathogenesis PMID: 19645629
17. The binding of lamin B receptor to chromatin is regulated by phosphorylation in the arginine-serine repeat-containing REGION PMID: 11846796
18. Mutations in the gene encoding the lamin B receptor produce an altered nuclear morphology in granulocytes (Pelger-Huet anomaly). PMID: 12118250
19. Autosomal recessive HEM/Greenberg skeletal dysplasia is caused by 3 beta-hydroxysterol delta 14-reductase deficiency due to mutations in this gene. PMID: 12618959
20. examination of temporal association with protamine 1 during spermiogenesis PMID: 14701833
21. SR protein kinase and cdc2 kinase regulate lamin B receptor binding to chromatin PMID: 14718546
22. MeCP2 interacts in vitro and in vivo with the inner nuclear membrane protein LBR, and the unstructured aminoacidic sequence linking the MBD and TRD domains of MeCP2 is responsible for this association. PMID: 19331822
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相關(guān)疾病：

Pelger-Huet anomaly (PHA); Greenberg dysplasia (GRBGD); Reynolds syndrome (REYNS)
亞細(xì)胞定位：

Nucleus inner membrane; Multi-pass membrane protein. Endoplasmic reticulum membrane. Cytoplasm. Nucleus.
蛋白家族：

ERG4/ERG24 family
組織特異性：

Expressed in the bone marrow, liver, heart, adrenal gland, lung, placenta and uterus. Expressed in osteoclasts and osteoblast-like cells.
數(shù)據(jù)庫(kù)鏈接：

HGNC: 6518

OMIM: 169400

KEGG: hsa:3930

STRING: 9606.ENSP00000272163

UniGene: Hs.435166