Recombinant Mouse Mucolipin-3 (Mcoln3)

in vitro E.coli expression system

中文名稱：

小鼠Mcoln3重組蛋白
貨號：

CSB-CF823183MO
說明書：

MSDS Datasheet
規格：
來源：

in vitro E.coli expression system
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

基因名：

Mcoln3
Uniprot No.：

Q8R4F0
別名：

Mcoln3; Mucolipin-3; Transient receptor potential channel mucolipin 3; TRPML3
種屬：

Mus musculus (Mouse)
蛋白長度：

full length protein
表達區域：

1-553
氨基酸序列

MANPEVLVSSCRARQDESPCTFHPSSSPSEQLLLEDQMRRKLKFFFMNPCEKFWARGRKP WKLAIQILKIAMVTIQLVLFGLSNQMVVAFKEENTIAFKHLFLKGYMDRMDDTYAVYTQS EVYDQIIFAVTQYLQLQNISVGNHAYENKGTKQSAMAICQHFYRQGTICPGNDTFDIDPE VETECFLVEPDEASHLGTPGENKLNLSLDFHRLLTVELQFKLKAINLQTVRHQELPDCYD FTLTITFDNKAHSGRIKISLDNDISIKECKDWHVSGSIQKNTHYMMIFDAFVILTCLASL VLCARSVIRGLQLQQEFVNFFLLHYKKEVSASDQMEFINGWYIMIIISDILTIVGSVLKM EIQAKSLTSYDVCSILLGTSTMLVWLGVIRYLGFFAKYNLLILTLQAALPNVMRFCCCAA MIYLGYCFCGWIVLGPYHEKFRSLNRVSECLFSLINGDDMFSTFAKMQQKSYLVWLFSRV YLYSFISLFIYMILSLFIALITDTYETIKHYQQDGFPETELRKFIAECKDLPNSGKYRLE DDPPGSLLCCCKK
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.
蛋白標簽：

N-terminal 10xHis-tagged
產品提供形式：

Liquid or 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 Tris/PBS-based buffer, 6% Trehalose, pH 8.0
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：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. 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 & COA：

Please contact us to get it.

產品評價

靶點詳情

功能：

Nonselective ligand-gated cation channel probably playing a role in the regulation of membrane trafficking events. Acts as Ca(2+)-permeable cation channel with inwardly rectifying activity. Mediates release of Ca(2+) from endosomes to the cytoplasm, contributes to endosomal acidification and is involved in the regulation of membrane trafficking and fusion in the endosomal pathway. Does not seem to act as mechanosensory transduction channel in inner ear sensory hair cells. Proposed to play a critical role at the cochlear stereocilia ankle-link region during hair-bundle growth. Involved in the regulation of autophagy. Through association with GABARAPL2 may be involved in autophagosome formation possibly providing Ca(2+) for the fusion process. Through a possible and probably tissue-specific heteromerization with MCOLN1 may be at least in part involved in many lysosome-dependent cellular events. Possible heteromeric ion channel assemblies with TRPV5 show pharmacological similarity with TRPML3.
基因功能參考文獻：
1. Study shows that, when uropathogenic E. coli infect bladder epithelial cells, they are targeted by autophagy but avoid degradation because of their capacity to neutralize lysosomal pH. This change is detected by TRPML3, TRPML3 activation then spontaneously initiates lysosome exocytosis, resulting in expulsion of exosome-encased bacteria. PMID: 26027738
2. Trpml3 inactivation does not lead to hearing and vestibular impairment in mice PMID: 21179200
3. Results describe the expression and subcellular distribution of TRPML3 in the inner ear as well as in other sensory organs. PMID: 21344404
4. prominent role for MCOLN3 in regulating Ca(2+) homeostasis at the endosomal pathway PMID: 21245134
5. Mutations are associated with deafness and pigmentation defects in varitint-waddler (Va) mice PMID: 12403827
6. TRPML3 is critical for stereocilia bundle formation during development and may function during endocytosis or exocytosis. PMID: 17329082
7. The A419P mutation affects TRPLMe channel glycosylation and causes massive cell death PMID: 17962195
8. This study provides the first direct mechanistic link of a mutation in a TRPML3 with mammalian hearing loss. PMID: 18048323
9. TRPML3(A419P) and (I362T+A419P) at physiological potentials may have a role in hair cell degeneration and deafness PMID: 18162548
10. The varitint-waddler mouse phenotypes and the TRPML3 ion channel mutation: cause and consequence. (Review) PMID: 18504603
11. an adverse effect of mutant TRPML3 on bundle development and mechano-electrical transduction is the main cause of hearing loss in Va(J)/+ mutant mice PMID: 18801844
12. The deaf-waddler isoform of PMCA2, operating at 30% efficacy, showed a significantly decreased ability to rescue the Ca(2+) loading of cells expressing TRPML3(A419P). PMID: 19299509
13. In animal disease model of neuropathic pain, the TRPML3 showed the most dramatic change. PMID: 19446956
顯示更多

收起更多
相關疾病：

Defects in Mcoln3 are the cause of the varitin-waddler (Va) phenotype. Classical Va mice exhibit early-onset hearing loss, vestibular defects, pigmentation abnormalities and perinatal lethality. The phenotype varitin-waddler Jackcon (Va-J), which arose in a cross segregating for Va, is similar but less severe.
亞細胞定位：

Early endosome membrane; Multi-pass membrane protein. Late endosome membrane; Multi-pass membrane protein. Cytoplasmic vesicle, autophagosome membrane; Multi-pass membrane protein. Cell projection, stereocilium membrane; Multi-pass membrane protein.
蛋白家族：

Transient receptor (TC 1.A.4) family, Polycystin subfamily, MCOLN3 sub-subfamily
組織特異性：

Expressed in the cochlea; particularly in the inner and outer hair cells (at protein level).
數據庫鏈接：

KEGG: mmu:171166

STRING: 10090.ENSMUSP00000038801

UniGene: Mm.114683