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- 文献和实验
- 技术资料
- 保存条件:
We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃.
- 保质期:
Generally, the shelf life of liquid form is 6 months at -20℃/-80℃. The shelf life of lyophilized form is 12 months at -20℃/-80℃.
- 英文名:
Recombinant Mouse Ribitol-5-phosphate transferase FKTN (Fktn), partial
- 库存:
200
- 供应商:
武汉华美生物工程有限公司
- 规格:
20μg
英文名称:
Recombinant Mouse Ribitol-5-phosphate transferase FKTN (Fktn), partial品名简称:
Recombinant Mouse Fktn protein, partial货号:
CSB-EP008709MO规格:
20μg价格:
1344LC-MS/Western Blot:
/SDS-PAGE:
(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.纯度:
Greater than 85% as determined by SDS-PAGE.内毒素:
Not test生物活性:
/基因名:
FktnUniprot No.:
Q8R507别名:
Fukuyama-type congenital muscular dystrophy protein Ribitol-5-phosphate transferase种属:
Mus musculus (Mouse)蛋白长度:
Partial来源:
E.coli分子量:
57.8 kDa表达区域:
29-461aa氨基酸序列:
KHYLSARNGPGSSKSKGNRVGFDSTQWRAVKKFIMLTSSQNVPVFLIDPWILESINKNFEQVKNASQGPASECRFFCVPRDFTAFALQYHLWKNEDGWFRIAENMGFQCLKTESKDPRLDGIDSLSGTEIPLHYVCKLTTHAIHLVVFHERSGNYLWHGHLRLKGHMDRKFVPFRKLQFGRYPGAFDRPELQQVTVDGLDMLIPKDPGRFLEEVPHSRFIECRYKEARAFLQQYIDDNTVDAMVFRKRAKELLQLAAKTLKDLGVPFWLSSGTCLGWYRQCGIIPYSKDVDLGIFIQDYKPDIILAFQEAGLPLKHKFGKVEDSLELSFQGKNDVKLDIFFFYEEADHLWNGGTQARTGKKFKYLFPKFTLCWTEFVDIKVHVPCETVDYIEANYGKTWKIPIKTWDWKSSPPNVQPNGIWPISEWDEVIQLY蛋白标签:
N-terminal 10xHis-tagged and C-terminal Myc-tagged产品提供形式:
Liquid or Lyophilized powder缓冲液:
If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol. If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, pH 8.0.储存条件:
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.保质期:
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℃/-80℃. The shelf life of lyophilized form is 12 months at -20℃/-80℃.货期:
3-7 business days注意事项:
Repeated freezing and thawing is not recommended. Store working aliquots at 4℃ for up to one week.功能1:
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) (PubMed:12471058). 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 (By similarity). Required for normal location of POMGNT1 in Golgi membranes, and for normal POMGNT1 activity (PubMed:19017726). May interact with and reinforce a large complex encompassing the outside and inside of muscle membranes (PubMed:19017726, PubMed:22922256). Could be involved in brain development (Probable).功能2:
/文献:
"Expression of dystroglycan, fukutin and POMGnT1 during mouse cerebellar development." Henion T.R., Qu Q., Smith F.I. Brain Res. Mol. Brain Res. 112:177-181(2003)研究领域:
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文献和实验Duchenne and Becker Muscular Dystrophy
The two forms of dystrophin-associated muscular dystrophies, known as Duchenne and Becker muscular dystrophy (DMD/BMD; OMIM 310200) are caused by genetic defects in the huge DMD gene (79 exons), located at Xp21 and coding for the 427-kDa
Use of Animal Models to Understand Human Muscular Dystrophy
(myotonic dystrophy protein kinase [DMPK]) in myotonic dystrophy (DM) (5 ), and mutations in a novel nuclear protein in Emery-Dreifuss MD (6 ).
Analysis of LAMA2 Gene in Merosin- Deficient Congenital Dystrophy
Congenital muscular dystrophies (CMDs) are a clinically and genetically heterogenous group of muscle disorders, with onset in early infancy, and autosomal recessive inheritance (1 – 3 ). Several forms have been identified: classical
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