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Identification
HMDB Protein ID HMDBP08440
Secondary Accession Numbers
  • 14152
Name tRNA modification GTPase GTPBP3, mitochondrial
Synonyms
  1. GTP-binding protein 3
  2. Mitochondrial GTP-binding protein 1
Gene Name GTPBP3
Protein Type Unknown
Biological Properties
General Function Involved in GTP binding
Specific Function GTPase involved in the 5-carboxymethylaminomethyl modification (mnm(5)s(2)U34) of the wobble uridine base in mitochondrial tRNAs (Probable)
Pathways Not Available
Reactions Not Available
GO Classification
Component
cell part
intracellular
Function
purine nucleotide binding
binding
nucleotide binding
catalytic activity
hydrolase activity
guanyl nucleotide binding
guanyl ribonucleotide binding
gtp binding
gtpase activity
nucleoside-triphosphatase activity
hydrolase activity, acting on acid anhydrides
hydrolase activity, acting on acid anhydrides, in phosphorus-containing anhydrides
pyrophosphatase activity
Process
rna metabolic process
ncrna metabolic process
trna metabolic process
trna processing
metabolic process
macromolecule metabolic process
cellular macromolecule metabolic process
trna modification
Cellular Location
  1. Mitochondrion
Gene Properties
Chromosome Location Chromosome:1
Locus 19p13.11
SNPs GTPBP3
Gene Sequence
>1479 bp
ATGTGGCGGGGGCTTTGGACCCTGGCGGCCCAAGCGGCACGTGGGCCTCGCAGATTGTGC
ACGCGCCGGAGCAGCGGCGCACCAGCCCCCGGCTCCGGCGCCACCATCTTCGCGCTAAGC
TCTGGCCAAGGCCGCTGCGGCATCGCAGTGATCCGGACCAGCGGCCCCGCCAGCGGCCAC
GCCCTCCGAATTCTCACAGCACCCCGAGACCTGCCCCTTGCTCGCCACGCCAGCCTGCGC
CTGCTCAGCGATCCCCGCTCCGGGGAGCCTCTGGACCGCGCACTGGTGCTCTGGTTCCCA
GGTCCCCAGAGTTTCACCGGTGAGGACTGCGTGGAGTTCCACGTGCATGGAGGCCCGGCA
GTGGTGAGCGGCGTCCTGCAGGCCTTGGGCAGCGTGCCAGGGCTTCGACCGGCGGAGGCA
GGCGAGTTCACCAGACGGGCGTTCGCCAATGGGAAGCTGAACCTGACCGAAGTGGAGGGG
CTGGCGGACCTTATCCACGCGGAAACAGAGGCGCAGCGGCGGCAGGCCCTCAGGCAGCTG
GACGGAGAGCTGGGCCACCTCTGCCGTGGCTGGGCCGAGACCCTCACCAAAGCTCTGGCC
CACGTGGAGGCCTATATCGATTTCGGCGAGGATGACAACCTGGAGGAGGGGGTCCTGGAG
CAAGCCGACATCGAAGTACGGGCACTGCAGGTGGCCCTGGGTGCACATCTACGAGATGCC
AGGCGCGGGCAGAGGCTCCGCTCAGGGGTGCACGTAGTGGTCACTGGACCCCCCAATGCG
GGCAAGAGCAGCCTAGTGAACCTGCTCAGTCGGAAGCCTGTGTCCATCGTGTCCCCGGAG
CCAGGGACCACCCGTGACGTGCTGGAGACCCCAGTCGACCTGGCCGGATTTCCTGTGCTG
CTGAGCGACACGGCTGGGTTGCGGGAGGGCGTGGGGCCCGTGGAGCAGGAGGGCGTGCGG
CGCGCCCGGGAGAGGCTAGAGCAGGCTGACCTCATTCTGGCCATGCTGGATGCTTCTGAC
CTGGCCTCTCCCTCCAGTTGCAACTTCCTGGCCACCGTCGTAGCCTCTGTGGGAGCCCAG
AGCCCCAGTGACAGCAGCCAGCGCCTCCTCCTGGTGCTGAACAAGTCGGACCTGCTGTCC
CCGGAGGGCCCAGGTCCCGGTCCTGACCTGCCCCCGCACCTGCTGCTGTCCTGTCTGACG
GGAGAGGGGCTGGACGGCCTCCTGGAGGCGCTGAGGAAGGAGCTAGCTGCAGTGTGTGGG
GACCCGTCCACAGATCCCCCGCTGCTGACCCGAGCAAGGCACCAGCACCACCTCCAGGGT
TGCCTGGATGCCCTCGGCCACTACAAGCAGTCAAAAGACCTGGCCCTGGCGGCAGAGGCG
CTGCGGGTGGCCCGGGGTCACCTGACCCGGCTCACAGGTGGAGGGGGTACCGAGGAGATC
CTGGACATCATCTTCCAGGACTTCTGTGTGGGCAAGTGA
Protein Properties
Number of Residues 492
Molecular Weight 52029.5
Theoretical pI 6.46
Pfam Domain Function
Signals
  • None
Transmembrane Regions
  • None
Protein Sequence
>tRNA modification GTPase GTPBP3, mitochondrial
MWRGLWTLAAQAARGPRRLCTRRSSGAPAPGSGATIFALSSGQGRCGIAVIRTSGPASGH
ALRILTAPRDLPLARHASLRLLSDPRSGEPLDRALVLWFPGPQSFTGEDCVEFHVHGGPA
VVSGVLQALGSVPGLRPAEAGEFTRRAFANGKLNLTEVEGLADLIHAETEAQRRQALRQL
DGELGHLCRGWAETLTKALAHVEAYIDFGEDDNLEEGVLEQADIEVRALQVALGAHLRDA
RRGQRLRSGAHVVVTGPPNAGKSSLVNLLSRKPVSIVSPEPGTTRDVLETPVDLAGFPVL
LSDTAGLREGVGPVEQEGVRRARERLEQADLILAMLDASDLASPSSCNFLATVVASVGAQ
SPSDSSQRLLLVLNKSDLLSPEGPGPGPDLPPHLLLSCLTGEGLDGLLEALRKELAAVCG
DPSTDPPLLTRARHQHHLQGCLDALGHYKQSKDLALAAEALRVARGHLTRLTGGGGTEEI
LDIIFQDFCVGK
GenBank ID Protein 193082991
UniProtKB/Swiss-Prot ID Q969Y2
UniProtKB/Swiss-Prot Entry Name GTPB3_HUMAN
PDB IDs Not Available
GenBank Gene ID NM_032620.3
GeneCard ID GTPBP3
GenAtlas ID GTPBP3
HGNC ID HGNC:14880
References
General References
  1. Ota T, Suzuki Y, Nishikawa T, Otsuki T, Sugiyama T, Irie R, Wakamatsu A, Hayashi K, Sato H, Nagai K, Kimura K, Makita H, Sekine M, Obayashi M, Nishi T, Shibahara T, Tanaka T, Ishii S, Yamamoto J, Saito K, Kawai Y, Isono Y, Nakamura Y, Nagahari K, Murakami K, Yasuda T, Iwayanagi T, Wagatsuma M, Shiratori A, Sudo H, Hosoiri T, Kaku Y, Kodaira H, Kondo H, Sugawara M, Takahashi M, Kanda K, Yokoi T, Furuya T, Kikkawa E, Omura Y, Abe K, Kamihara K, Katsuta N, Sato K, Tanikawa M, Yamazaki M, Ninomiya K, Ishibashi T, Yamashita H, Murakawa K, Fujimori K, Tanai H, Kimata M, Watanabe M, Hiraoka S, Chiba Y, Ishida S, Ono Y, Takiguchi S, Watanabe S, Yosida M, Hotuta T, Kusano J, Kanehori K, Takahashi-Fujii A, Hara H, Tanase TO, Nomura Y, Togiya S, Komai F, Hara R, Takeuchi K, Arita M, Imose N, Musashino K, Yuuki H, Oshima A, Sasaki N, Aotsuka S, Yoshikawa Y, Matsunawa H, Ichihara T, Shiohata N, Sano S, Moriya S, Momiyama H, Satoh N, Takami S, Terashima Y, Suzuki O, Nakagawa S, Senoh A, Mizoguchi H, Goto Y, Shimizu F, Wakebe H, Hishigaki H, Watanabe T, Sugiyama A, Takemoto M, Kawakami B, Yamazaki M, Watanabe K, Kumagai A, Itakura S, Fukuzumi Y, Fujimori Y, Komiyama M, Tashiro H, Tanigami A, Fujiwara T, Ono T, Yamada K, Fujii Y, Ozaki K, Hirao M, Ohmori Y, Kawabata A, Hikiji T, Kobatake N, Inagaki H, Ikema Y, Okamoto S, Okitani R, Kawakami T, Noguchi S, Itoh T, Shigeta K, Senba T, Matsumura K, Nakajima Y, Mizuno T, Morinaga M, Sasaki M, Togashi T, Oyama M, Hata H, Watanabe M, Komatsu T, Mizushima-Sugano J, Satoh T, Shirai Y, Takahashi Y, Nakagawa K, Okumura K, Nagase T, Nomura N, Kikuchi H, Masuho Y, Yamashita R, Nakai K, Yada T, Nakamura Y, Ohara O, Isogai T, Sugano S: Complete sequencing and characterization of 21,243 full-length human cDNAs. Nat Genet. 2004 Jan;36(1):40-5. Epub 2003 Dec 21. [PubMed:14702039 ]
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  4. Li X, Guan MX: A human mitochondrial GTP binding protein related to tRNA modification may modulate phenotypic expression of the deafness-associated mitochondrial 12S rRNA mutation. Mol Cell Biol. 2002 Nov;22(21):7701-11. [PubMed:12370316 ]
  5. Bykhovskaya Y, Mengesha E, Wang D, Yang H, Estivill X, Shohat M, Fischel-Ghodsian N: Phenotype of non-syndromic deafness associated with the mitochondrial A1555G mutation is modulated by mitochondrial RNA modifying enzymes MTO1 and GTPBP3. Mol Genet Metab. 2004 Nov;83(3):199-206. [PubMed:15542390 ]