Recombinant Human tRNA modification GTPase GTPBP3, mitochondrial (GTPBP3)

中文名稱：

人GTPBP3重組蛋白
貨號：

CSB-YP856890HU
說明書：

MSDS Datasheet
規格：
來源：

Yeast
其他：

中文名稱：

人GTPBP3重組蛋白
貨號：

CSB-EP856890HU
說明書：

MSDS Datasheet
規格：
來源：

E.coli
其他：

中文名稱：

人GTPBP3重組蛋白
貨號：

CSB-EP856890HU-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：

人GTPBP3重組蛋白
貨號：

CSB-BP856890HU
說明書：

MSDS Datasheet
規格：
來源：

Baculovirus
其他：

中文名稱：

人GTPBP3重組蛋白
貨號：

CSB-MP856890HU
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：

GTPBP3
Uniprot No.：

Q969Y2
別名：

GTPBP3; MTGP1; tRNA modification GTPase GTPBP3; mitochondrial; GTP-binding protein 3; Mitochondrial GTP-binding protein 1
種屬：

Homo sapiens (Human)
蛋白長度：

Full Length of Mature Protein
表達區域：

82-492
氨基酸序列

LSDPRSGEP LDRALVLWFP GPQSFTGEDC VEFHVHGGPA VVSGVLQALG SVPGLRPAEA GEFTRRAFAN GKLNLTEVEG LADLIHAETE AQRRQALRQL DGELGHLCRG WAETLTKALA HVEAYIDFGE DDNLEEGVLE QADIEVRALQ VALGAHLRDA RRGQRLRSGV HVVVTGPPNA GKSSLVNLLS RKPVSIVSPE PGTTRDVLET PVDLAGFPVL LSDTAGLREG VGPVEQEGVR RARERLEQAD LILAMLDASD LASPSSCNFL ATVVASVGAQ SPSDSSQRLL LVLNKSDLLS PEGPGPGPDL PPHLLLSCLT GEGLDGLLEA LRKELAAVCG DPSTDPPLLT RARHQHHLQG CLDALGHYKQ SKDLALAAEA LRVARGHLTR LTGGGGTEEI LDIIFQDFCV GK
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：

GTPase involved in the 5-carboxymethylaminomethyl modification (mnm(5)s(2)U34) of the wobble uridine base in mitochondrial tRNAs.
基因功能參考文獻：
1. Defects in the mitochondrial tRNA modification enzymes MTO1 and GTPBP3 promote different metabolic reprogramming through a HIF-PPARgamma-UCP2-AMPK axis. PMID: 29348686
2. GTPBP3 defective expression is associated with an mitochondrial-tRNA hypomodification status.GTPBP3 plays a role in the regulation of UCP2 and MCP1 proteins through AMPK signaling. PMID: 26642043
3. Most individuals with GTPBP3 mutations developed neurological symptoms and MRI involvement of thalamus, putamen, and brainstem resembling Leigh syndrome. PMID: 25434004
4. GTPBP3 localizes in the mitochondria and is a deafness-associated homolog of yeast MSS1. PMID: 12370316
5. Phenotype of non-syndromic deafness associated with the mitochondrial A1555G mutation is modulated by mitochondrial RNA modifying enzymes MTO1 and GTPBP3. PMID: 15542390
6. Data show that the two most abundant GTPBP3 isoforms exhibit moderate affinity for guanine nucleotides like their bacterial homologue, MnmE, although they hydrolyze GTP at a 100-fold lower rate. PMID: 18852288
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相關疾病：

Combined oxidative phosphorylation deficiency 23 (COXPD23)
亞細胞定位：

Mitochondrion.
蛋白家族：

TRAFAC class TrmE-Era-EngA-EngB-Septin-like GTPase superfamily, TrmE GTPase family
組織特異性：

Ubiquitously expressed.
數據庫鏈接：

HGNC: 14880

OMIM: 580000

KEGG: hsa:84705

UniGene: Hs.334885