Recombinant Mouse Transmembrane channel-like protein 1 (Tmc1), partial

中文名稱：

小鼠Tmc1重組蛋白
貨號：

CSB-YP840294MO1
說明書：

MSDS Datasheet
規格：
來源：

Yeast
其他：

中文名稱：

小鼠Tmc1重組蛋白
貨號：

CSB-EP840294MO1
說明書：

MSDS Datasheet
規格：
來源：

E.coli
其他：

中文名稱：

小鼠Tmc1重組蛋白
貨號：

CSB-EP840294MO1-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Tmc1重組蛋白
貨號：

CSB-BP840294MO1
說明書：

MSDS Datasheet
規格：
來源：

Baculovirus
其他：

中文名稱：

小鼠Tmc1重組蛋白
貨號：

CSB-MP840294MO1
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：

Tmc1
Uniprot No.：

Q8R4P5
別名：

Tmc1; Bth; dn; Transmembrane channel-like protein 1; Beethoven protein; Deafness protein; Transmembrane cochlear-expressed protein 1
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：

Probable ion channel required for the normal function of cochlear hair cells.
基因功能參考文獻：
1. We generated a model of TMC1 based on X-ray and cryo-EM structures of TMEM16 proteins, revealing the presence of a large cavity near the protein-lipid interface that also harbors the Beethoven mutation, suggesting that it could function as a permeation pathway. PMID: 30063209
2. Transmembrane cochlear expressed gene 1 protein (TMC1)-dependent channels have larger single-channel conductance and in outer hair cells (OHCs) support a tonotopic apex-to-base conductance gradient. PMID: 29872055
3. This study showed that calcium and integrin-binding protein 2 binds to the components of the hair cell mechanotransduction complex, TMC1 and TMC2, and these interactions are disrupted by deafness-causing Cib2 mutations. PMID: 28663585
4. TMC1 and TMC2 are components of the stereocilia mechanoelectrical transduction channel complex. PMID: 26321635
5. The results suggest that a major component of channel adaptation is regulated by changes in intracellular Ca(2+). PMID: 26324676
6. This study demonstrated that the M412K point mutation in TMC1 of Beethoven mice leads to a reduced Ca2+ permeability, more so in Tmc1Bth/Bth than in Tmc1Bh/+, and conductance of the MET channel of Mouse Outer Hair Cells. PMID: 26758827
7. gene augmentation with Tmc1 or Tmc2 is well suited for further development as a strategy for restoration of auditory function in deaf patients who carry TMC1 mutations PMID: 26157030
8. During the first postnatal week, we observed a normal mechanotransducer current in hair cells lacking Tmc1 or Tmc2; however, in the absence of both isoforms, we recorded a large MT current that was phase-shifted 180 degrees . PMID: 24127526
9. This study demonstrate TMC1 is components of hair cell transduction channels and contribute to permeation properties. PMID: 23871232
10. Tmc1 is expressed in mouse vestibular & cochlear hair cells near the stereocilia tips. Deletion of Tmc1 & Tmc2 causes deafness. Restoration of Tmc1 rescues mechanotransduction. PMID: 22105175
11. Tmc1 was present within the endoplasmic reticulum as an integral membrane protein containing six transmembrane domains and cytosolic N- and C-termini. PMID: 20672865
12. role in mouse models of deafness [review] PMID: 14552423
13. Excess of social and/or physical stimulation in Ts65Dn mice may affect cognition by disturbing the emotional and behavioral components of the learning process. PMID: 15941601
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相關疾病：

Defects in Tmc1 are the cause of the dominant deaf mutant Beethoven (BTH). Heterozygotes show progressive hair-cell degeneration from day 20 onwards, leading to severe depletion of inner hair cells and scattered loss of outer hair cells, and progressive loss of the Preyer reflex from around day 30. Homozygotes show almost complete degeneration of inner hair cells, and little or no Preyer reflex at any age.; DISEASE: Note=Defects in Tmc1 are the cause of recessive deaf mutant dn. The dn mutant shows profound deafness with degeneration of the organ of Corti, stria vascularis, and occasionally the saccular macula, starting at about 10 days after birth (PubMed:11850618).
亞細胞定位：

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

TMC family
組織特異性：

Detected in cochlear inner and outer hair cells and in neurosensory epithelia of the vestibular end organs. Also expressed in cortex, cerebellum, eye, colon, ovary and testis.
數據庫鏈接：

KEGG: mmu:13409

STRING: 10090.ENSMUSP00000040859

UniGene: Mm.219585