gtp phosphohydrolases


Summary: Enzymes that hydrolyze GTP to GDP. EC 3.6.1.-.

Top Publications

  1. An H, Cho G, Lee J, Paik S, Kim Y, Lee H. Higd-1a interacts with Opa1 and is required for the morphological and functional integrity of mitochondria. Proc Natl Acad Sci U S A. 2013;110:13014-9 pubmed publisher
    ..Collectively, our observations demonstrate that Higd-1a inhibits Opa1 cleavage and is required for mitochondrial fusion by virtue of its interaction with Opa1. ..
  2. Haldar A, Saka H, Piro A, Dunn J, Henry S, Taylor G, et al. IRG and GBP host resistance factors target aberrant, "non-self" vacuoles characterized by the missing of "self" IRGM proteins. PLoS Pathog. 2013;9:e1003414 pubmed publisher
    ..These data reveal that intracellular immune recognition of organelle-like structures by IRG and GBP proteins is partly dictated by the missing of "self" IRGM proteins from these structures...
  3. Dorn G. Mitochondrial dynamism and cardiac fate--a personal perspective. Circ J. 2013;77:1370-9 pubmed
  4. Neidig A, Yeung A, Rosay T, Tettmann B, Strempel N, Rueger M, et al. TypA is involved in virulence, antimicrobial resistance and biofilm formation in Pseudomonas aeruginosa. BMC Microbiol. 2013;13:77 pubmed publisher
    ..TypA is a GTPase that has recently been identified to modulate virulence in enteric Gram-negative pathogens...
  5. Ruan Y, Li H, Zhang K, Jian F, Tang J, Song Z. Loss of Yme1L perturbates mitochondrial dynamics. Cell Death Dis. 2013;4:e896 pubmed publisher
    ..In addition, there is interaction between SLP-2 with Yme1L and shYme1L cells retain stress-induced mitochondrial hyperfusion. Taken together, our results clarify how Yme1L regulates mitochondrial morphology. ..
  6. Springer H, Schramm M, Taylor G, Howard J. Irgm1 (LRG-47), a regulator of cell-autonomous immunity, does not localize to mycobacterial or listerial phagosomes in IFN-?-induced mouse cells. J Immunol. 2013;191:1765-74 pubmed publisher
    ..We discuss these findings in the context of evidence that Irgm1 functions as a negative regulator of other members of the immunity-related GTPase protein family. ..
  7. Li N, Chen Y, Guo Q, Zhang Y, Yuan Y, Ma C, et al. Cryo-EM structures of the late-stage assembly intermediates of the bacterial 50S ribosomal subunit. Nucleic Acids Res. 2013;41:7073-83 pubmed publisher
    ..Especially, H38 plays an essential role in stabilizing the central protuberance, through the interaction with the 5S rRNA, and the correctly orientated H38 is likely a prerequisite for further maturation of the 50S subunit. ..
  8. Leong V, Kent M, Jomaa A, Ortega J. Escherichia coli rimM and yjeQ null strains accumulate immature 30S subunits of similar structure and protein complement. RNA. 2013;19:789-802 pubmed publisher
    ..Functionally related factors will bind to this intermediate to catalyze the last steps of maturation leading to the mature 30S subunit. ..
  9. Fröhlich C, Grabiger S, Schwefel D, Faelber K, Rosenbaum E, Mears J, et al. Structural insights into oligomerization and mitochondrial remodelling of dynamin 1-like protein. EMBO J. 2013;32:1280-92 pubmed publisher
    ..Based on these results and electron microscopy reconstructions, we propose an oligomerization mode for DNM1L which differs from that of dynamin and might be adapted to the remodelling of mitochondria. ..

More Information


  1. Agarwal N, Pareek M, Thakur P, Pathak V. Functional characterization of EngA(MS), a P-loop GTPase of Mycobacterium smegmatis. PLoS ONE. 2012;7:e34571 pubmed publisher
    ..Moreover, EngA(MS) devoid of N-terminal region interacts well with 50S even in the absence of GTP, indicating a regulatory role of the N-terminal domain in EngA(MS)-50S interaction. ..
  2. Wang S, Song J, Tan M, Albers K, Jia J. Mitochondrial fission proteins in peripheral blood lymphocytes are potential biomarkers for Alzheimer's disease. Eur J Neurol. 2012;19:1015-22 pubmed publisher
    ..Altered mitochondrial fission proteins Drp1, SNO-Drp1, and Fis1 in PBL were relatively sensitive and specific in identifying AD patients and could be serving as a biomarker in the procedure of diagnosis. ..
  3. Kushnareva Y, Gerencser A, Bossy B, Ju W, White A, Waggoner J, et al. Loss of OPA1 disturbs cellular calcium homeostasis and sensitizes for excitotoxicity. Cell Death Differ. 2013;20:353-65 pubmed publisher
    ..Thus, our data show that whereas OPA1 is required for mitochondrial fusion, maintenance of crista morphology and oxidative phosphorylation, loss of OPA1 also results in defective Ca(2+) homeostasis. ..
  4. Hamahata T, Fujimaki T, Fujiki K, Miyazaki A, Mizota A, Murakami A. OPA1 mutations in Japanese patients suspected to have autosomal dominant optic atrophy. Jpn J Ophthalmol. 2012;56:91-7 pubmed publisher
    ..After a detailed clinical assessment of the proband, the screening of the OPA1 gene may be helpful for precise diagnosis of ADOA, provided the relevant information of the family members is limited. ..
  5. Kim J, Oh M, Bernard L, Macara I, Zhang H. The RhoG/ELMO1/Dock180 signaling module is required for spine morphogenesis in hippocampal neurons. J Biol Chem. 2011;286:37615-24 pubmed publisher
    ..Together, our findings uncover a role for the RhoG/ELMO1/Dock180 signaling module in spine morphogenesis in hippocampal neurons. ..
  6. Kashatus D, Lim K, Brady D, Pershing N, Cox A, Counter C. RALA and RALBP1 regulate mitochondrial fission at mitosis. Nat Cell Biol. 2011;13:1108-15 pubmed publisher
    ..Thus, the two mitotic kinases Aurora A and cyclin B-CDK1 converge on RALA and RALBP1 to promote mitochondrial fission, the appropriate distribution of mitochondria to daughter cells and ultimately proper mitochondrial function. ..
  7. Zhang Z, Wakabayashi N, Wakabayashi J, Tamura Y, Song W, Sereda S, et al. The dynamin-related GTPase Opa1 is required for glucose-stimulated ATP production in pancreatic beta cells. Mol Biol Cell. 2011;22:2235-45 pubmed publisher
    ..Consequently, mice lacking Opa1 in beta cells develop hyperglycemia. The data suggest that the function of Opa1 in the maintenance of the electron transport chain is physiologically relevant in beta cells...
  8. Qi X, Qvit N, Su Y, Mochly Rosen D. A novel Drp1 inhibitor diminishes aberrant mitochondrial fission and neurotoxicity. J Cell Sci. 2013;126:789-802 pubmed publisher
    ..Taken together, our findings suggest that P110, as a selective peptide inhibitor of Drp1, might be useful for the treatment of diseases in which excessive mitochondrial fission and mitochondrial dysfunction occur. ..
  9. Zhao J, Liu T, Jin S, Wang X, Qu M, Uhlen P, et al. Human MIEF1 recruits Drp1 to mitochondrial outer membranes and promotes mitochondrial fusion rather than fission. EMBO J. 2011;30:2762-78 pubmed publisher
    ..As MIEF1 is vertebrate-specific, these data also reveal important differences between yeast and vertebrates in the regulation of mitochondrial dynamics. ..
  10. Kornmann B, Osman C, Walter P. The conserved GTPase Gem1 regulates endoplasmic reticulum-mitochondria connections. Proc Natl Acad Sci U S A. 2011;108:14151-6 pubmed publisher
    ..Furthermore, we provide evidence that the metazoan Gem1 ortholog Miro-1 localizes to sites of ER-mitochondrial contact, suggesting that some of the features ascribed to Gem1 may be evolutionarily conserved. ..
  11. Quirós P, Ramsay A, Sala D, Fernandez Vizarra E, Rodriguez F, Peinado J, et al. Loss of mitochondrial protease OMA1 alters processing of the GTPase OPA1 and causes obesity and defective thermogenesis in mice. EMBO J. 2012;31:2117-33 pubmed publisher
    ..This study provides the first description of an unexpected role in energy metabolism for the metalloprotease OMA1 and reinforces the importance of mitochondrial quality control for normal metabolic function. ..
  12. Moss T, Andreazza C, Verma A, Daga A, McNew J. Membrane fusion by the GTPase atlastin requires a conserved C-terminal cytoplasmic tail and dimerization through the middle domain. Proc Natl Acad Sci U S A. 2011;108:11133-8 pubmed publisher
    ..GTP-dependent dimerization of atlastin generates an enzymatically active protein that drives membrane fusion after nucleotide hydrolysis and conformational reorganization. ..
  13. Guo Q, Goto S, Chen Y, Feng B, Xu Y, Muto A, et al. Dissecting the in vivo assembly of the 30S ribosomal subunit reveals the role of RimM and general features of the assembly process. Nucleic Acids Res. 2013;41:2609-20 pubmed publisher
    ..More importantly, this study reveals intriguing similarities and dissimilarities between the in vitro and the in vivo assembly pathways, suggesting that they are in general similar but with subtle differences. ..
  14. Shirendeb U, Calkins M, Manczak M, Anekonda V, Dufour B, McBride J, et al. Mutant huntingtin's interaction with mitochondrial protein Drp1 impairs mitochondrial biogenesis and causes defective axonal transport and synaptic degeneration in Huntington's disease. Hum Mol Genet. 2012;21:406-20 pubmed publisher
  15. Lerner D, McCoy D, Isabella A, Mahowald A, Gerlach G, Chaudhry T, et al. A Rab10-dependent mechanism for polarized basement membrane secretion during organ morphogenesis. Dev Cell. 2013;24:159-68 pubmed publisher
    ..Significantly, Tango1 and Rab10 are also planar polarized at the basal epithelial surface. We propose that the spatial control of BM production along two tissue axes promotes exocytic efficiency, BM remodeling, and organ morphogenesis...
  16. Polke J, Laura M, Pareyson D, Taroni F, Milani M, Bergamin G, et al. Recessive axonal Charcot-Marie-Tooth disease due to compound heterozygous mitofusin 2 mutations. Neurology. 2011;77:168-73 pubmed publisher
    ..Novel genetic findings include an intragenic MFN2 deletion and nonsense-mediated decay. Carrier parents were asymptomatic, suggesting that MFN2 null alleles can be nonpathogenic unless coinherited with another mutation. ..
  17. Guo Y, Chen X, Zhang H, Li N, Yang X, Cheng W, et al. Association of OPA1 polymorphisms with NTG and HTG: a meta-analysis. PLoS ONE. 2012;7:e42387 pubmed publisher
    ..Moreover, stratified analyses for NTG detecting the effects of both OPA1 polymorphisms seemed to vary with ethnicity. Further investigations are needed to validate the association. ..
  18. Schnoor M, Lai F, Zarbock A, Kläver R, Polaschegg C, Schulte D, et al. Cortactin deficiency is associated with reduced neutrophil recruitment but increased vascular permeability in vivo. J Exp Med. 2011;208:1721-35 pubmed publisher
    ..Our results represent the first physiological evidence that cortactin is crucial for orchestrating the molecular events leading to proper endothelial barrier function and leukocyte recruitment in vivo. ..
  19. Fülöp L, Szanda G, Enyedi B, Varnai P, Spät A. The effect of OPA1 on mitochondrial Ca²? signaling. PLoS ONE. 2011;6:e25199 pubmed publisher
    ..The participation of Na?/Ca²? and Ca²?/H? antiporters in this transport process is indicated by pharmacological data. Altogether, our observations reveal the significance of OPA1 in the control of mitochondrial Ca²? metabolism. ..
  20. Wang X, Yan M, Fujioka H, Liu J, Wilson Delfosse A, Chen S, et al. LRRK2 regulates mitochondrial dynamics and function through direct interaction with DLP1. Hum Mol Genet. 2012;21:1931-44 pubmed publisher
    ..We concluded that LRRK2 regulates mitochondrial dynamics by increasing mitochondrial DLP1 through its direct interaction with DLP1, and LRRK2 kinase activity plays a critical role in this process. ..
  21. Achila D, Gulati M, Jain N, Britton R. Biochemical characterization of ribosome assembly GTPase RbgA in Bacillus subtilis. J Biol Chem. 2012;287:8417-23 pubmed publisher
    ..These results support a model in which RbgA promotes a late step in ribosome biogenesis and that one role of GTP hydrolysis is to stimulate dissociation of RbgA from the ribosome. ..
  22. Hwang J, Tseitin V, Ramnarayan K, Shenderovich M, Inouye M. Structure-based design and screening of inhibitors for an essential bacterial GTPase, Der. J Antibiot (Tokyo). 2012;65:237-43 pubmed publisher
    ..Therefore, our lead compounds inhibiting Der GTPase provide scaffolds for the development of novel antibiotics against antibiotic-resistant pathogenic bacteria. ..
  23. Leboucher G, Tsai Y, Yang M, Shaw K, Zhou M, Veenstra T, et al. Stress-induced phosphorylation and proteasomal degradation of mitofusin 2 facilitates mitochondrial fragmentation and apoptosis. Mol Cell. 2012;47:547-57 pubmed publisher
    ..These findings demonstrate how proximal signaling events can influence both mitochondrial dynamics and apoptosis through phosphorylation-stimulated degradation of the mitochondrial fusion machinery. ..
  24. Wang Y, Luo Y, Hong Y, Peng J, Lo L. Ribosome biogenesis factor Bms1-like is essential for liver development in zebrafish. J Genet Genomics. 2012;39:451-62 pubmed publisher
    ..Therefore, our findings demonstrate that Bms1l is necessary for zebrafish liver development...
  25. Chan D. Fusion and fission: interlinked processes critical for mitochondrial health. Annu Rev Genet. 2012;46:265-87 pubmed publisher
    ..A better understanding of these processes likely will ultimately lead to improvements in human health. ..
  26. Armengod M, Moukadiri I, Prado S, Ruiz Partida R, Benítez Páez A, Villarroya M, et al. Enzymology of tRNA modification in the bacterial MnmEG pathway. Biochimie. 2012;94:1510-20 pubmed publisher
    ..This review summarizes the present state of knowledge on these pathways and what we still need to know, with special emphasis on the MnmEG pathway. ..
  27. Barboni P, Savini G, Parisi V, Carbonelli M, La Morgia C, Maresca A, et al. Retinal nerve fiber layer thickness in dominant optic atrophy measurements by optical coherence tomography and correlation with age. Ophthalmology. 2011;118:2076-80 pubmed publisher
    ..The author(s) have no proprietary or commercial interest in any materials discussed in this article. ..
  28. Chen Y, Liu Y, Dorn G. Mitochondrial fusion is essential for organelle function and cardiac homeostasis. Circ Res. 2011;109:1327-31 pubmed publisher
    ..Interruption of mitochondrial fusion causes lethal cardiac failure at a time corresponding to 3 or 4 cycles of unopposed mitochondrial fission. ..
  29. Feely S, Laura M, Siskind C, Sottile S, Davis M, Gibbons V, et al. MFN2 mutations cause severe phenotypes in most patients with CMT2A. Neurology. 2011;76:1690-6 pubmed publisher
    ..Disruption of functional domains of the protein was particularly likely to cause neuropathy. ..
  30. Namekata K, Watanabe H, Guo X, Kittaka D, Kawamura K, Kimura A, et al. Dock3 regulates BDNF-TrkB signaling for neurite outgrowth by forming a ternary complex with Elmo and RhoG. Genes Cells. 2012;17:688-97 pubmed publisher
    ..These results suggest that Dock3 plays important roles downstream of BDNF signaling in the central nervous system where it stimulates actin polymerization by multiple pathways. ..
  31. Ryu S, Choi K, Park J, Park Y, Kim S, Choi C. Mitofusin 1 inhibits an apoptosis-associated amino-terminal conformational change in Bax, but not its mitochondrial translocation, in a GTPase-dependent manner. Cancer Lett. 2012;323:62-8 pubmed publisher
    ..These results collectively suggest a role for Mfn1 in regulating the activation of Bax on the outer mitochondrial membrane in a GTPase-dependent manner. ..
  32. Liu T, Bian X, Sun S, Hu X, Klemm R, Prinz W, et al. Lipid interaction of the C terminus and association of the transmembrane segments facilitate atlastin-mediated homotypic endoplasmic reticulum fusion. Proc Natl Acad Sci U S A. 2012;109:E2146-54 pubmed publisher
    ..Our results suggest that protein-lipid and protein-protein interactions within the membrane cooperate with the conformational change of the cytosolic domain to achieve homotypic ER membrane fusion. ..
  33. Van Bergen N, Crowston J, Kearns L, Staffieri S, Hewitt A, Cohn A, et al. Mitochondrial oxidative phosphorylation compensation may preserve vision in patients with OPA1-linked autosomal dominant optic atrophy. PLoS ONE. 2011;6:e21347 pubmed publisher
    ..Identification of genetic variants that enable this response may provide novel therapeutic insights into OXPHOS compensation for preventing vision loss in optic neuropathies. ..
  34. Kageyama Y, Zhang Z, Sesaki H. Mitochondrial division: molecular machinery and physiological functions. Curr Opin Cell Biol. 2011;23:427-34 pubmed publisher
    ..We summarize recent progress in understanding how the division machinery assembles onto mitochondria and how mitochondrial division contributes to cellular physiology and human diseases. ..
  35. Stiburek L, Cesnekova J, Kostková O, Fornuskova D, Vinsova K, Wenchich L, et al. YME1L controls the accumulation of respiratory chain subunits and is required for apoptotic resistance, cristae morphogenesis, and cell proliferation. Mol Biol Cell. 2012;23:1010-23 pubmed publisher
  36. Ferguson S, De Camilli P. Dynamin, a membrane-remodelling GTPase. Nat Rev Mol Cell Biol. 2012;13:75-88 pubmed publisher
    ..In addition, several connections between dynamin and human disease have also emerged, highlighting specific contributions of this GTPase to the physiology of different tissues. ..
  37. Shen K, Arslan S, Akopian D, Ha T, Shan S. Activated GTPase movement on an RNA scaffold drives co-translational protein targeting. Nature. 2012;492:271-5 pubmed publisher
  38. Otera H, Ishihara N, Mihara K. New insights into the function and regulation of mitochondrial fission. Biochim Biophys Acta. 2013;1833:1256-68 pubmed publisher
  39. Bussiere C, Hashem Y, Arora S, Frank J, Johnson A. Integrity of the P-site is probed during maturation of the 60S ribosomal subunit. J Cell Biol. 2012;197:747-59 pubmed publisher
    ..We propose that Efl1 promotes a functional check of the integrity of the 60S subunit before its first round of translation. ..
  40. Schmid S, Frolov V. Dynamin: functional design of a membrane fission catalyst. Annu Rev Cell Dev Biol. 2011;27:79-105 pubmed publisher
  41. Wong C, Traynor D, Basse N, Kay R, Warren A. Defective ribosome assembly in Shwachman-Diamond syndrome. Blood. 2011;118:4305-12 pubmed publisher
    ..These findings in Dictyostelium and SDS patient cells provide compelling support for the hypothesis that SDS is a ribosomopathy caused by corruption of an essential cytoplasmic step in 60S subunit maturation. ..
  42. Chen Y, Jia X, Wang P, Xiao X, Li S, Guo X, et al. Mutation survey of the optic atrophy 1 gene in 193 Chinese families with suspected hereditary optic neuropathy. Mol Vis. 2013;19:292-302 pubmed
    ..Lack of awareness of the mild phenotype of DOA may contribute to the low frequency of OPA1-related DOA in Chinese. The phenotype associated with compound heterozygous OPA1 mutations may suggest a possible addictive effect. ..
  43. Cohen M, Amiott E, Day A, Leboucher G, Pryce E, Glickman M, et al. Sequential requirements for the GTPase domain of the mitofusin Fzo1 and the ubiquitin ligase SCFMdm30 in mitochondrial outer membrane fusion. J Cell Sci. 2011;124:1403-10 pubmed publisher
  44. Ash M, Maher M, Guss J, Jormakka M. The initiation of GTP hydrolysis by the G-domain of FeoB: insights from a transition-state complex structure. PLoS ONE. 2011;6:e23355 pubmed publisher
    ..Together with the crystal structure, the findings suggest a new mechanism for hydrolysis initiation in small G-proteins, in which the attacking water molecule is aligned by contacts with the protein backbone only. ..
  45. Efeyan A, Zoncu R, Chang S, Gumper I, Snitkin H, Wolfson R, et al. Regulation of mTORC1 by the Rag GTPases is necessary for neonatal autophagy and survival. Nature. 2013;493:679-83 pubmed publisher
    ..Thus, the Rag GTPases signal glucose and amino-acid concentrations to mTORC1, and have an unexpectedly key role in neonates in autophagy induction and thus nutrient homeostasis and viability. ..
  46. Nguyen D, Alavi M, Kim K, Kang T, Scott R, Noh Y, et al. A new vicious cycle involving glutamate excitotoxicity, oxidative stress and mitochondrial dynamics. Cell Death Dis. 2011;2:e240 pubmed publisher
    ..Therefore, we propose a new vicious cycle involved in neurodegeneration that includes glutamate excitotoxicity, oxidative stress, and mitochondrial dynamics. ..
  47. Bar Peled L, Schweitzer L, Zoncu R, Sabatini D. Ragulator is a GEF for the rag GTPases that signal amino acid levels to mTORC1. Cell. 2012;150:1196-208 pubmed publisher
    ..Thus, we provide mechanistic insight into how mTORC1 senses amino acids by identifying Ragulator as a guanine nucleotide exchange factor (GEF) for the Rag GTPases. ..
  48. Strunk B, Novak M, Young C, Karbstein K. A translation-like cycle is a quality control checkpoint for maturing 40S ribosome subunits. Cell. 2012;150:111-21 pubmed publisher
    ..This cycle thus provides a functional test of 60S subunit binding and the GTPase site before ribosomes enter the translating pool. ..
  49. Verstraeten N, Fauvart M, Versées W, Michiels J. The universally conserved prokaryotic GTPases. Microbiol Mol Biol Rev. 2011;75:507-42, second and third pages of table of contents pubmed publisher
    ..Conserved roles in eukaryotic homologs will be discussed as well. A comprehensive overview summarizing current knowledge on prokaryotic GTPases will aid in further elucidating the function of these important proteins. ..
  50. Heidler J, Al Furoukh N, Kukat C, Salwig I, Ingelmann M, Seibel P, et al. Nitric oxide-associated protein 1 (NOA1) is necessary for oxygen-dependent regulation of mitochondrial respiratory complexes. J Biol Chem. 2011;286:32086-93 pubmed publisher
    ..mNOA1 is transcriptionally regulated in an oxygen-sensitive manner. We propose that oxygen-dependent regulation of mNOA1 is instrumental to adjusting OXPHOS activity to oxygen availability, thereby controlling mitochondrial metabolism. ..
  51. Luigetti M, Fabrizi G, Taioli F, Conte A, Del Grande A, Sabatelli M. Clinical, electrophysiological and pathological findings of a patient with CMT2 due to the p.Ala738Val mitofusin 2 mutation. J Neurol Sci. 2011;307:168-70 pubmed publisher
    ..2213C>T, p.Ala738Val MFN2 mutation. This mutation has been already described to be only associated with an early onset and moderately severe CMT2A phenotype. ..
  52. Paul M, Alushin G, Barros M, Rak M, Tzagoloff A. The putative GTPase encoded by MTG3 functions in a novel pathway for regulating assembly of the small subunit of yeast mitochondrial ribosomes. J Biol Chem. 2012;287:24346-55 pubmed publisher
    ..The rescue is accompanied by an increase in processed 15 S rRNA. This suggests that Mtg3p and Mrpl4p jointly regulate assembly of the small subunit by modulating processing of the 15 S rRNA precursor. ..
  53. Papanicolaou K, Ngoh G, Dabkowski E, O Connell K, Ribeiro R, Stanley W, et al. Cardiomyocyte deletion of mitofusin-1 leads to mitochondrial fragmentation and improves tolerance to ROS-induced mitochondrial dysfunction and cell death. Am J Physiol Heart Circ Physiol. 2012;302:H167-79 pubmed publisher