Recombinant Mouse Ribitol-5-phosphate transferase FKTN (Fktn), partial

中文名稱：

小鼠Fktn重組蛋白
貨號：

CSB-YP008709MO
說明書：

MSDS Datasheet
規格：
來源：

Yeast
其他：

中文名稱：

小鼠Fktn重組蛋白
貨號：

CSB-EP008709MO-B
說明書：

MSDS Datasheet
規格：
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Fktn重組蛋白
貨號：

CSB-BP008709MO
說明書：

MSDS Datasheet
規格：
來源：

Baculovirus
其他：

中文名稱：

小鼠Fktn重組蛋白
貨號：

CSB-MP008709MO
說明書：

MSDS Datasheet
規格：
來源：

Mammalian cell
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

純度：

>85% (SDS-PAGE)
基因名：

Fktn
Uniprot No.：

Q8R507
別名：

Fktn; FcmdFukutin; EC 2.4.2.-; Fukuyama-type congenital muscular dystrophy protein; Ribitol-5-phosphate transferase
種屬：

Mus musculus (Mouse)
蛋白長度：

Partial
蛋白標簽：

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.
產品提供形式：

Lyophilized powder Warning: in_array() expects parameter 2 to be array, null given in /www/web/cusabio_cn/public_html/caches/caches_template/default/content/show_product_protein.php on line 662
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.

產品評價

靶點詳情

功能：

Catalyzes the transfer of CDP-ribitol to the distal N-acetylgalactosamine of 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 first step in the formation of the ribitol 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. Required for normal location of POMGNT1 in Golgi membranes, and for normal POMGNT1 activity. May interact with and reinforce a large complex encompassing the outside and inside of muscle membranes. Could be involved in brain development (Probable).
基因功能參考文獻：
1. Rapamycin treatment in fukutin-deficient mouse model of dystroglycanopathy delays/reduces disease burden. PMID: 27257474
2. Fktn deficient mice express moderate to severe muscular dystrophy; glycosylated alpha-dystroglycan has a unique role in muscle regeneration in these mice PMID: 26751696
3. Mouse fukutin deletion impairs dystroglycan processing, recapitulates muscular dystrophy and is relevant to modifications near the dystroglycan O-mannose sugar. PMID: 22922256
4. disease-causing missense mutations cause abnormal folding and localization of fukutin protein PMID: 22275357
5. the highly hydrophobic transmembrane domain of Fukutin-1 was purified; the identity of the peptide and revealed that in hydrophobic solvents mimicking the bilayer, the peptide adopts a well-structured alpha-helix as predicted from the sequence. PMID: 20117215
6. Fukuyama-type congenital muscular dystrophy (FCMD) murine ortholog, Fcmd is 90% identical to that of its human counterpart PMID: 12408965
7. Merosin-deficient congenital muscular dystrophy with mental retardation and cerebellar cysts, unlinked to the FCMD locus, in three Tunisian patients PMID: 12467726
8. These results support the hypothesis that fukutin is involved in the glycosylation process of alpha-DG and that a defect in this process plays an essential role in the pathogenesis of FCMD. PMID: 15351499
9. The basal lamina of the cortical surface in chimeras showed defects at E14, coinciding with the earliest time point at which ectopia were detected PMID: 15907289
10. fukutin plays crucial roles in the myelination of peripheral nerve and formation of neuromuscular junction. suggest that defective glycosylation of alpha-dystroglycan may play a role in the impairment of these processes in the deficiency of fukutin. PMID: 17326765
顯示更多

收起更多
亞細胞定位：

Golgi apparatus membrane; Single-pass type II membrane protein. Cytoplasm. Nucleus. Endoplasmic reticulum.
蛋白家族：

LicD transferase family
組織特異性：

Expressed in the retina, with highest levels found in the inner segments of photoreceptors and the outer plexiform layer (at protein level). Expressed at lower levels in the inner and outer nuclear layers, the inner plexiform layers, and the ganglion cell
數據庫鏈接：

KEGG: mmu:246179

STRING: 10090.ENSMUSP00000061489

UniGene: Mm.247210