Recombinant Human tRNA modification GTPase GTPBP3, mitochondrial(GTPBP3)

Code CSB-YP856890HU
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Source Yeast
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Code CSB-EP856890HU
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Source E.coli
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Code CSB-EP856890HU-B
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Source E.coli
Conjugate 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.
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Code CSB-BP856890HU
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Source Baculovirus
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Code CSB-MP856890HU
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Source Mammalian cell
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Product Details

Purity >85% (SDS-PAGE)
Target Names GTPBP3
Uniprot No. Q969Y2
Alternative Names GTPBP3; MTGP1; tRNA modification GTPase GTPBP3; mitochondrial; GTP-binding protein 3; Mitochondrial GTP-binding protein 1
Species Homo sapiens (Human)
Expression Region 82-492
Target Protein Sequence 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
Protein Length Full Length of Mature Protein
Tag Info The following tags are available.
N-terminal His-tagged
Tag-Free
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.
Form 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.
Buffer before Lyophilization Tris/PBS-based buffer, 6% Trehalose, pH 8.0
Reconstitution 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.
Troubleshooting
and FAQs
Protein FAQs
Storage Condition Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
Shelf Life 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.
Lead Time 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.
Notes 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.

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Target Background

Function
GTPase involved in the 5-carboxymethylaminomethyl modification (mnm(5)s(2)U34) of the wobble uridine base in mitochondrial tRNAs.
Gene References into Functions
  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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Involvement in disease Combined oxidative phosphorylation deficiency 23 (COXPD23)
Subcellular Location Mitochondrion.
Protein Families TRAFAC class TrmE-Era-EngA-EngB-Septin-like GTPase superfamily, TrmE GTPase family
Tissue Specificity Ubiquitously expressed.
Database Links

HGNC: 14880

OMIM: 580000

KEGG: hsa:84705

UniGene: Hs.334885

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