Recombinant Mouse Fukutin-related protein (Fkrp), partial

中文名稱：

小鼠Fkrp重組蛋白
貨號：

CSB-YP807019MO
說明書：

MSDS Datasheet
規(guī)格：
來源：

Yeast
其他：

中文名稱：

小鼠Fkrp重組蛋白
貨號：

CSB-EP807019MO
說明書：

MSDS Datasheet
規(guī)格：
來源：

E.coli
其他：

中文名稱：

小鼠Fkrp重組蛋白
貨號：

CSB-EP807019MO-B
說明書：

MSDS Datasheet
規(guī)格：
來源：

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：

小鼠Fkrp重組蛋白
貨號：

CSB-BP807019MO
說明書：

MSDS Datasheet
規(guī)格：
來源：

Baculovirus
其他：

中文名稱：

小鼠Fkrp重組蛋白
貨號：

CSB-MP807019MO
說明書：

MSDS Datasheet
規(guī)格：
來源：

Mammalian cell
其他：

產(chǎn)品詳情
產(chǎn)品評價
靶點詳情

產(chǎn)品詳情

純度：

>85% (SDS-PAGE)
基因名：

Fkrp
Uniprot No.：

Q8CG64
別名：

FkrpFukutin-related protein; EC 2.4.2.-; Ribitol-5-phosphate transferase
種屬：

Mus musculus (Mouse)
蛋白長度：

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：

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)品評價

靶點詳情

功能：

Catalyzes the transfer of CDP-ribitol to ribitol 5-phosphate previously attached by FKTN/fukutin of to the phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine-beta-3-N-acetylglucosamine-beta-4-(phosphate-6-)mannose), a carbohydrate structure present in alpha-dystroglycan (DAG1). This constitutes the second step in the formation of the ribose 5-phosphate tandem repeat which links the phosphorylated O-mannosyl trisaccharide to the ligand binding moiety composed of repeats of 3-xylosyl-alpha-1,3-glucuronic acid-beta-1.
基因功能參考文獻：
1. This study demonstrated that the Expression of glycosylated alpha-dystroglycan in newborn skeletal and cardiac muscles with FKRP mutation mice. PMID: 27515093
2. Reduced Fkrp levels in skeletal muscle are associated with a progressive muscular dystrophy PMID: 24234655
3. FKRP mutations are associated with muscular dystrophy. PMID: 23591631
4. a reduction in Fkrp influences the ability of tissue-specific forms of alpha-dystroglycan to direct the deposition of several laminin isoforms PMID: 21900571
5. investigation of the functional roles of FKRP in muscular dystrophies; FKRP is essential for the functional glycosylation of alpha-dystroglycan;both the mutation itself and the levels of FKRP expression are equally critical for the survival of the animals PMID: 20675713
6. Basement membrane (BM) fragility may underlie abnormal phenotypes in fukutin-null embryos, and maintenance of BM function may require fukutin-mediated glycosylation of alpha-dystroglycan early in embryonic development. PMID: 15837576
7. data offer the first evidence of a fukutin-related protein (FKRP) complex in muscle and suggest that FKRP may influence the glycosylation status of dystroglycan from within the sarcolemmal dystrophin-glycoprotein complex PMID: 17452335
8. results suggest the generation of a mouse model for FKRP related muscular dystrophy requires a knock-down rather than a knock-in strategy in order to give rise to a disease phenotype. PMID: 19155270
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亞細胞定位：

Golgi apparatus membrane; Single-pass type II membrane protein. Secreted. Cell membrane, sarcolemma. Rough endoplasmic reticulum. Cytoplasm.
蛋白家族：

LicD transferase family
組織特異性：

Expressed in the retina, specifically in the inner segments of the photoreceptors, the outer plexiform layers, inner nuclear layers, and ganglion cell layers (at protein level). Expressed at highest levels in brain, lung, heart, kidney and liver.
數(shù)據(jù)庫鏈接：

KEGG: mmu:243853

STRING: 10090.ENSMUSP00000059091

UniGene: Mm.39703