Recombinant Mouse Transmembrane channel-like protein 1 (Tmc1)-VLPs

中文名稱：

Recombinant Mouse Transmembrane channel-like protein 1 (Tmc1)-VLPs
貨號：

CSB-MP840294MO
說明書：

MSDS Datasheet
規(guī)格：
其他：

產(chǎn)品詳情
產(chǎn)品評價
相關(guān)產(chǎn)品
靶點詳情

產(chǎn)品詳情

基因名：

Tmc1
Uniprot No.：

Q8R4P5
別名：

(Beethoven protein)(Deafness protein)(Transmembrane cochlear-expressed protein 1)
種屬：

Mus musculus (Mouse)
蛋白長度：

Full Length
來源：

Mammalian cell
表達區(qū)域：

1-757aa
氨基酸序列

MLQIQVEEKEEDTEESSSEEEEDKLPRRESLRPKRKRTRDVINEDDPEPEPEDEETRKAREKERRRRLRRGAEEEEEIDEEELERLKALLDENRQMIATVKCKPWKMEKKIEVLKEAKKFVSENEGALGKGKGKKWFAFKMMMAKKWAKFLRDFENFKAACVPWENKIKAIESQFGSSVASYFLFLRWMYGVNMVLFVLTFSLIMLPEYLWGLPYGSLPRKTVPRAEEASAANFGVLYDFNGLAQYSVLFYGYYDNKRTIGWLNFRLPLSYFLVGIMCIGYSFLVVLKAMTKNIGDDGGGDDNTFNFSWKVFCSWDYLIGNPETADNKFNSITMNFKEAIIEERAAQVEENIHLIRFLRFLANFFVFLTLGASGYLIFWAVKRSQEFAQQDPDTLGWWEKNEMNMVMSLLGMFCPTLFDLFAELEDYHPLIALKWLLGRIFALLLGNLYVFILALMDEINNKIEEEKLVKANITLWEANMIKAYNESLSGLSGNTTGAPFFVHPADVPRGPCWETMVGQEFVRLTVSDVLTTYVTILIGDFLRACFVRFCNYCWCWDLEYGYPSYTEFDISGNVLALIFNQGMIWMGSFFAPSLPGINILRLHTSMYFQCWAVMCCNVPEARVFKASRSNNFYLGMLLLILFLSTMPVLYMIVSLPPSFDCGPFSGKNRMFEVIGETLEHDFPSWMAKILRQLSNPGLVIAVILVMVLTIYYLNATAKGQKAANLDLKKKMKQQALENKMRNKKMAAARAAAAAGGQ
Note: The complete sequence may include tag sequence, target protein sequence, linker sequence and extra sequence that is translated with the protein sequence for the purpose(s) of secretion, stability, solubility, etc.
If the exact amino acid sequence of this recombinant protein is critical to your application, please explicitly request the full and complete sequence of this protein before ordering.
蛋白標(biāo)簽：

C-terminal 10xHis-tagged
If you have specified tag type, please tell us and we will check if it's possible to develop.
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.
緩沖液：

Lyophilized from PBS, 6% Trehalose, pH 7.4.
復(fù)溶：

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein indeionized sterile water to a concentration of 0.1-1.0 mg/mL.Aliquot for long-term storage at -80℃. Solubilize for 60 minutes at room temperature with occasional gentle mixing. Avoid vigorous shaking or vortexing.
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.
注意事項：

The VLPs are expressed from human 293 cells (HEK293).Mix the sample gently by repeatedly pipetting it up and down. Do not vortex. Repeated freezing and thawing is not recommended.Store the protein at -20℃/-80℃ upon receiving it, and ensure to avoid repeated freezing and thawing, otherwise, it will affect the protein activity. The immunization strategy should be optimized (antigen dose, regimen and adjuvant).
Datasheet & COA：

Please contact us to get it.

產(chǎn)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(guān)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(xì)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(shù)據(jù)庫鏈接：

KEGG: mmu:13409

STRING: 10090.ENSMUSP00000040859

UniGene: Mm.219585