Recombinant Mouse Chloride channel protein 1 (Clcn1), partial

中文名稱：

Recombinant Mouse Chloride channel protein 1(Clcn1) ,partial
貨號：

CSB-YP717540MO
說明書：

MSDS Datasheet
規格：
來源：

Yeast
其他：

中文名稱：

Recombinant Mouse Chloride channel protein 1(Clcn1) ,partial
貨號：

CSB-EP717540MO
說明書：

MSDS Datasheet
規格：
來源：

E.coli
其他：

中文名稱：

Recombinant Mouse Chloride channel protein 1(Clcn1) ,partial
貨號：

CSB-EP717540MO-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：

Recombinant Mouse Chloride channel protein 1(Clcn1) ,partial
貨號：

CSB-BP717540MO
說明書：

MSDS Datasheet
規格：
來源：

Baculovirus
其他：

中文名稱：

Recombinant Mouse Chloride channel protein 1(Clcn1) ,partial
貨號：

CSB-MP717540MO
說明書：

MSDS Datasheet
規格：
來源：

Mammalian cell
其他：

產品詳情
產品評價
靶點詳情

產品詳情

純度：

>85% (SDS-PAGE)
基因名：

Clcn1
Uniprot No.：

Q64347
別名：

Clcn1; Clc1Chloride channel protein 1; ClC-1; Chloride channel protein; skeletal muscle
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
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：

Voltage-gated chloride channel. Plays an important role in membrane repolarization in skeletal muscle cells after muscle contraction.
基因功能參考文獻：
1. Data show that Muscleblind-like 1 (Mbnl1) and Muscleblind-Like 3 (Mbnl3) bind skeletal muscle chloride channel CIC-1 (Clc-1) mRNA. PMID: 26501102
2. Sex hormones at high concentration can rapidly modulate ClC-1 in mouse skeletal muscle fibers in vitro. PMID: 23625574
3. Myotonia (delayed muscle relaxation) is the most commonly observed symptom in DM1 patients and is caused by aberrant splicing of the skeletal muscle chloride channel (CLCN1) gene PMID: 23828222
4. Myotonia in adult human skeletal actin transgenic mice may be explained on the basis of a mosaic expression of ClC-1 channels in different fibres and/or on alterations of other conductances. PMID: 23247112
5. The expression of the muscle chloride channel, ClC-1, in Huntington disease muscle was compromised by improper splicing and a corresponding reduction in total Clcn1 (gene for ClC-1) mRNA. PMID: 23671115
6. the majority of functional ClC-1 channels localize to the sarcolemma and provide essential insight into the basis of myofiber excitability in normal and diseased skeletal muscle. PMID: 21078869
7. Expanded CUG repeats trigger aberrant splicing of ClC-1 chloride channel pre-mRNA and hyperexcitability of skeletal muscle in myotonic dystrophy PMID: 12150905
8. The A331T mutation causes an unprecedented alteration of ClC-1 gating and reveals novel processes defining transitions between open and closed states in ClC chloride channels PMID: 12196568
9. A distinct autosomal recessive myotonic mouse in the C57BL/6 background (line B6MT)is reported in which the Clc-1 gene shows polymorphism with no functional consequences. PMID: 12370472
10. CLC-1 deficiency not only affects muscle relaxation (myotonia) but also modulates diaphragm performance during the contractile phase of the contraction-relaxation cycle PMID: 16959550
11. alternative splicing is a posttranscriptional mechanism regulating chloride conductance during muscle development, and the chloride channelopathy in a transgenic mouse model of DM1 results from a failure to execute a splicing transition for CLCN1. PMID: 17135300
12. These results support a molecular mechanism for myotonia in myotonic dystrophy type 1 in which a reduction in both the number of functional sarcolemmal ClC-1 and maximal channel open probability. PMID: 17158949
13. Clc1 expression was dramatically decreased in Znf9+/- mice. PMID: 17335846
14. genetic CLC-1 chloride channel deficiency in mice not only produces myotonia but also substantially worsens the isotonic contractile performance of diaphragm muscle. PMID: 17483199
15. These observations indicate that the myotonia and chloride channelopathy observed in DM both result from abnormal alternative splicing of ClC-1 PMID: 18008009
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亞細胞定位：

Cell membrane; Multi-pass membrane protein.
蛋白家族：

Chloride channel (TC 2.A.49) family, ClC-1/CLCN1 subfamily
組織特異性：

Predominantly expressed in skeletal muscles.
數據庫鏈接：

KEGG: mmu:12723

STRING: 10090.ENSMUSP00000031894

UniGene: Mm.386757