Recombinant Human Mitochondrial translation release factor in rescue (MTRFR)

中文名稱：

Recombinant Human Mitochondrial translation release factor in rescue(MTRFR)
貨號：

CSB-YP880965HU
說明書：

MSDS Datasheet
規格：
來源：

Yeast
其他：

中文名稱：

Recombinant Human Mitochondrial translation release factor in rescue(MTRFR)
貨號：

CSB-EP880965HU
說明書：

MSDS Datasheet
規格：
來源：

E.coli
其他：

中文名稱：

Recombinant Human Mitochondrial translation release factor in rescue(MTRFR)
貨號：

CSB-EP880965HU-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 Human Mitochondrial translation release factor in rescue(MTRFR)
貨號：

CSB-BP880965HU
說明書：

MSDS Datasheet
規格：
來源：

Baculovirus
其他：

中文名稱：

Recombinant Human Mitochondrial translation release factor in rescue(MTRFR)
貨號：

CSB-MP880965HU
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：

C12orf65
Uniprot No.：

Q9H3J6
別名：

C12orf65; My030Probable peptide chain release factor C12orf65; mitochondrial
種屬：

Homo sapiens (Human)
蛋白長度：

Full Length of Mature Protein
表達區域：

36-166
氨基酸序列

IAVTP VQMAGKKDYP ALLSLDENEL EEQFVKGHGP GGQATNKTSN CVVLKHIPSG IVVKCHQTRS VDQNRKLARK ILQEKVDVFY NGENSPVHKE KREAAKKKQE RKKRAKETLE KKKLLKELWE SSKKVH
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：

Part of a mitoribosome-associated quality control pathway that prevents aberrant translation by responding to interruptions during elongation. As heterodimer with MTRES1, ejects the unfinished nascent chain and peptidyl transfer RNA (tRNA), respectively, from stalled ribosomes. Recruitment of mitoribosome biogenesis factors to these quality control intermediates suggests additional roles for MTRES1 and MTRF during mitoribosome rescue.
基因功能參考文獻：
1. We demonstrate that the identical nonsense mutation in C12orf65 can result in different clinical features PMID: 25995486
2. our study delineates the broad spectrum of C12orf65 defects and establishes a distinct genotype-phenotype correlation. The obligatory clinical triad is optic atrophy, peripheral neuropathy, and spastic paraparesis. PMID: 24424123
3. The C12orf65 gene encodes a mitochondrial matrix protein that is critical for the release of newly synthesized proteins from mitochondrial ribosomes. PMID: 24284555
4. Truncating mutations in C12ORF65 lead to a variable phenotype with intellectual disability, spastic paraplegia, and ophthalmoplegia in 2 patients. PMID: 24080142
5. This work describes a mutation in the C12orf65 gene that causes recessive form of CMT6 and confirms the role of mitochondrial dysfunction in this complex axonal neuropathy. PMID: 24198383
6. A homozygous mutation of C12orf65 causes spastic paraplegia with optic atrophy and neuropathy. PMID: 23188110
7. Knockdown of C12orf65 resulted in increased reactive oxidative species production and apoptosis, leading to inhibition of cell proliferation. PMID: 22821833
8. C12orf65 might play a role in recycling abortive peptidyl-tRNA species, released from the ribosome during the elongation phase of translation. PMID: 20598281
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相關疾病：

Combined oxidative phosphorylation deficiency 7 (COXPD7); Spastic paraplegia 55, autosomal recessive (SPG55)
亞細胞定位：

Mitochondrion.
蛋白家族：

Prokaryotic/mitochondrial release factor family
組織特異性：

Expressed in all areas of the brain tested.
數據庫鏈接：

HGNC: 26784

OMIM: 613541

KEGG: hsa:91574

STRING: 9606.ENSP00000253233

UniGene: Hs.319128