UniProtKB - Q8IXI2 (MIRO1_HUMAN)
Mitochondrial Rho GTPase 1
RHOT1
Functioni
Mitochondrial GTPase involved in mitochondrial trafficking (PubMed:12482879, PubMed:16630562, PubMed:22396657).
Probably involved in control of anterograde transport of mitochondria and their subcellular distribution (PubMed:12482879, PubMed:16630562, PubMed:22396657).
Promotes mitochondrial fission during high calcium conditions (PubMed:27716788).
4 PublicationsSites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Metal bindingi | 197 | Calcium 1PROSITE-ProRule annotation | 1 | |
Metal bindingi | 199 | Calcium 1PROSITE-ProRule annotation | 1 | |
Metal bindingi | 201 | Calcium 1PROSITE-ProRule annotation | 1 | |
Metal bindingi | 203 | Calcium 1PROSITE-ProRule annotation | 1 | |
Metal bindingi | 208 | Calcium 1PROSITE-ProRule annotation | 1 | |
Metal bindingi | 317 | Calcium 2Curated | 1 | |
Metal bindingi | 319 | Calcium 2Curated | 1 | |
Metal bindingi | 321 | Calcium 2Curated | 1 | |
Metal bindingi | 328 | Calcium 2Curated | 1 |
Regions
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Nucleotide bindingi | 11 – 18 | GTP 1 | 8 | |
Nucleotide bindingi | 57 – 61 | GTP 1Sequence analysis | 5 | |
Nucleotide bindingi | 118 – 121 | GTP 1Sequence analysis | 4 | |
Nucleotide bindingi | 425 – 432 | GTP 2 | 8 | |
Nucleotide bindingi | 463 – 467 | GTP 2Sequence analysis | 5 | |
Nucleotide bindingi | 527 – 530 | GTP 2Sequence analysis | 4 |
GO - Molecular functioni
- calcium ion binding Source: InterPro
- GTPase activity Source: GO_Central
- GTP binding Source: GO_Central
GO - Biological processi
- cellular homeostasis Source: UniProtKB
- mitochondrial outer membrane permeabilization Source: UniProtKB
- mitochondrion organization Source: GO_Central
- mitochondrion transport along microtubule Source: UniProtKB
- regulation of mitochondrion organization Source: GO_Central
- small GTPase mediated signal transduction Source: InterPro
Keywordsi
Molecular function | Hydrolase |
Ligand | Calcium, GTP-binding, Metal-binding, Nucleotide-binding |
Enzyme and pathway databases
PathwayCommonsi | Q8IXI2 |
Reactomei | R-HSA-5689880, Ub-specific processing proteases R-HSA-9013425, RHOT1 GTPase cycle |
SignaLinki | Q8IXI2 |
Names & Taxonomyi
Protein namesi | Recommended name: Mitochondrial Rho GTPase 1 (EC:3.6.5.-)Short name: MIRO-1 Short name: hMiro-1 Alternative name(s): Rac-GTP-binding protein-like protein Ras homolog gene family member T1 |
Gene namesi | Name:RHOT1 Synonyms:ARHT1 |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
HGNCi | HGNC:21168, RHOT1 |
MIMi | 613888, gene |
neXtProti | NX_Q8IXI2 |
VEuPathDBi | HostDB:ENSG00000126858 |
Subcellular locationi
Mitochondrion
- Mitochondrion outer membrane 2 Publications; Single-pass type IV membrane protein 2 Publications
Note: Colocalizes with MGARP and RHOT2 at the mitochondria.
Mitochondrion
- integral component of mitochondrial outer membrane Source: UniProtKB
- mitochondrial outer membrane Source: Reactome
- mitochondrion Source: ParkinsonsUK-UCL
Other locations
- membrane Source: UniProtKB
Topology
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Topological domaini | 1 – 592 | CytoplasmicSequence analysisAdd BLAST | 592 | |
Transmembranei | 593 – 615 | Helical; Anchor for type IV membrane proteinSequence analysisAdd BLAST | 23 | |
Topological domaini | 616 – 618 | Mitochondrial intermembraneSequence analysis | 3 |
Keywords - Cellular componenti
Membrane, Mitochondrion, Mitochondrion outer membranePathology & Biotechi
Mutagenesis
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Mutagenesisi | 13 | P → V: Causes constitutive activation inducing an aggregation of the mitochondrial network. 3 Publications | 1 | |
Mutagenesisi | 18 | T → N: Causes constitutive inactivation. 2 Publications | 1 | |
Mutagenesisi | 156 | S → A: No effect on PINK1-PRKN-mediated degradation. 1 Publication | 1 | |
Mutagenesisi | 208 | E → K: Abolishes the formation of thread-like mitochondria. 1 Publication | 1 | |
Mutagenesisi | 328 | E → K: Abolishes the formation of thread-like mitochondria. 1 Publication | 1 | |
Mutagenesisi | 427 | K → V: No effect. 1 Publication | 1 | |
Mutagenesisi | 432 | S → N: No effect. 1 Publication | 1 |
Organism-specific databases
DisGeNETi | 55288 |
OpenTargetsi | ENSG00000126858 |
PharmGKBi | PA134906318 |
Miscellaneous databases
Pharosi | Q8IXI2, Tbio |
Genetic variation databases
BioMutai | RHOT1 |
DMDMi | 108860796 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
ChainiPRO_0000239313 | 1 – 618 | Mitochondrial Rho GTPase 1Add BLAST | 618 |
Post-translational modificationi
Keywords - PTMi
Ubl conjugationProteomic databases
EPDi | Q8IXI2 |
jPOSTi | Q8IXI2 |
MassIVEi | Q8IXI2 |
MaxQBi | Q8IXI2 |
PaxDbi | Q8IXI2 |
PeptideAtlasi | Q8IXI2 |
PRIDEi | Q8IXI2 |
ProteomicsDBi | 71002 [Q8IXI2-1] 71003 [Q8IXI2-2] 71004 [Q8IXI2-3] 71005 [Q8IXI2-4] 71006 [Q8IXI2-5] 71007 [Q8IXI2-6] |
PTM databases
GlyGeni | Q8IXI2, 1 site, 1 O-linked glycan (1 site) |
iPTMneti | Q8IXI2 |
PhosphoSitePlusi | Q8IXI2 |
Expressioni
Tissue specificityi
Gene expression databases
Bgeei | ENSG00000126858, Expressed in substantia nigra and 247 other tissues |
ExpressionAtlasi | Q8IXI2, baseline and differential |
Genevisiblei | Q8IXI2, HS |
Organism-specific databases
HPAi | ENSG00000126858, Low tissue specificity |
Interactioni
Subunit structurei
Interacts with the kinesin-binding proteins TRAK1/OIP106 and TRAK2/GRIF1, forming a link between mitochondria and the trafficking apparatus of the microtubules (PubMed:16630562).
Interacts with RAP1GDS1 (PubMed:27716788).
Interacts with ARMCX1 (By similarity).
Found in a complex with KIF5B, OGT, RHOT2 and TRAK1 (PubMed:24995978).
By similarity3 PublicationsBinary interactionsi
Q8IXI2
Protein-protein interaction databases
BioGRIDi | 120576, 83 interactors |
CORUMi | Q8IXI2 |
DIPi | DIP-39114N |
IntActi | Q8IXI2, 30 interactors |
MINTi | Q8IXI2 |
Miscellaneous databases
RNActi | Q8IXI2, protein |
Structurei
Secondary structure
3D structure databases
AlphaFoldDBi | Q8IXI2 |
SMRi | Q8IXI2 |
ModBasei | Search... |
PDBe-KBi | Search... |
Family & Domainsi
Domains and Repeats
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Domaini | 2 – 168 | Miro 1PROSITE-ProRule annotationAdd BLAST | 167 | |
Domaini | 184 – 219 | EF-hand 1PROSITE-ProRule annotationAdd BLAST | 36 | |
Domaini | 304 – 339 | EF-hand 2PROSITE-ProRule annotationAdd BLAST | 36 | |
Domaini | 416 – 579 | Miro 2PROSITE-ProRule annotationAdd BLAST | 164 |
Sequence similaritiesi
Keywords - Domaini
Repeat, Transmembrane, Transmembrane helixPhylogenomic databases
eggNOGi | KOG1707, Eukaryota |
GeneTreei | ENSGT00940000155641 |
HOGENOMi | CLU_014255_3_1_1 |
InParanoidi | Q8IXI2 |
OMAi | HVSVTWN |
OrthoDBi | 538388at2759 |
PhylomeDBi | Q8IXI2 |
TreeFami | TF300814 |
Family and domain databases
Gene3Di | 3.40.50.300, 2 hits |
InterProi | View protein in InterPro IPR011992, EF-hand-dom_pair IPR018247, EF_Hand_1_Ca_BS IPR013566, EF_hand_assoc_1 IPR013567, EF_hand_assoc_2 IPR002048, EF_hand_dom IPR021181, Miro IPR029506, Miro-1 IPR020860, MIRO_dom IPR027417, P-loop_NTPase IPR005225, Small_GTP-bd_dom IPR001806, Small_GTPase IPR003578, Small_GTPase_Rho |
PANTHERi | PTHR24072, PTHR24072, 1 hit PTHR24072:SF124, PTHR24072:SF124, 1 hit |
Pfami | View protein in Pfam PF08355, EF_assoc_1, 1 hit PF08356, EF_assoc_2, 1 hit PF00071, Ras, 1 hit |
PIRSFi | PIRSF037488, Mt_Rho_GTPase, 1 hit |
SMARTi | View protein in SMART SM00054, EFh, 2 hits SM00174, RHO, 1 hit |
SUPFAMi | SSF47473, SSF47473, 1 hit SSF52540, SSF52540, 2 hits |
TIGRFAMsi | TIGR00231, small_GTP, 1 hit |
PROSITEi | View protein in PROSITE PS00018, EF_HAND_1, 1 hit PS50222, EF_HAND_2, 2 hits PS51423, MIRO, 2 hits |
s (7+)i Sequence
Sequence statusi: Complete.
This entry describes 7 produced by isoformsialternative splicing. AlignAdd to basketThis entry has 7 described isoforms and 9 potential isoforms that are computationally mapped.Show allAlign All
This isoform has been chosen as the sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry. canonicali
10 20 30 40 50
MKKDVRILLV GEPRVGKTSL IMSLVSEEFP EEVPPRAEEI TIPADVTPER
60 70 80 90 100
VPTHIVDYSE AEQSDEQLHQ EISQANVICI VYAVNNKHSI DKVTSRWIPL
110 120 130 140 150
INERTDKDSR LPLILVGNKS DLVEYSSMET ILPIMNQYTE IETCVECSAK
160 170 180 190 200
NLKNISELFY YAQKAVLHPT GPLYCPEEKE MKPACIKALT RIFKISDQDN
210 220 230 240 250
DGTLNDAELN FFQRICFNTP LAPQALEDVK NVVRKHISDG VADSGLTLKG
260 270 280 290 300
FLFLHTLFIQ RGRHETTWTV LRRFGYDDDL DLTPEYLFPL LKIPPDCTTE
310 320 330 340 350
LNHHAYLFLQ STFDKHDLDR DCALSPDELK DLFKVFPYIP WGPDVNNTVC
360 370 380 390 400
TNERGWITYQ GFLSQWTLTT YLDVQRCLEY LGYLGYSILT EQESQASAVT
410 420 430 440 450
VTRDKKIDLQ KKQTQRNVFR CNVIGVKNCG KSGVLQALLG RNLMRQKKIR
460 470 480 490 500
EDHKSYYAIN TVYVYGQEKY LLLHDISESE FLTEAEIICD VVCLVYDVSN
510 520 530 540 550
PKSFEYCARI FKQHFMDSRI PCLIVAAKSD LHEVKQEYSI SPTDFCRKHK
560 570 580 590 600
MPPPQAFTCN TADAPSKDIF VKLTTMAMYP HVTQADLKSS TFWLRASFGA
610
TVFAVLGFAM YKALLKQR
Computationally mapped potential isoform sequencesi
There are 9 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketH7BXZ6 | H7BXZ6_HUMAN | Mitochondrial Rho GTPase | RHOT1 hCG_1991479 | 597 | Annotation score: | ||
J3KSR5 | J3KSR5_HUMAN | Mitochondrial Rho GTPase 1 | RHOT1 | 51 | Annotation score: | ||
A0A494C061 | A0A494C061_HUMAN | Mitochondrial Rho GTPase 1 | RHOT1 | 214 | Annotation score: | ||
J3QSG2 | J3QSG2_HUMAN | Mitochondrial Rho GTPase 1 | RHOT1 | 40 | Annotation score: | ||
J3QLG2 | J3QLG2_HUMAN | Mitochondrial Rho GTPase 1 | RHOT1 | 134 | Annotation score: | ||
K7EIQ7 | K7EIQ7_HUMAN | Mitochondrial Rho GTPase 1 | RHOT1 | 190 | Annotation score: | ||
K7EP86 | K7EP86_HUMAN | Mitochondrial Rho GTPase 1 | RHOT1 | 156 | Annotation score: | ||
J3KRG7 | J3KRG7_HUMAN | Mitochondrial Rho GTPase 1 | RHOT1 | 57 | Annotation score: | ||
A0A2R8Y4I2 | A0A2R8Y4I2_HUMAN | Mitochondrial Rho GTPase 1 | RHOT1 | 48 | Annotation score: |
Sequence cautioni
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 75 | A → V in AAM15734 (Ref. 3) Curated | 1 | |
Sequence conflicti | 246 | L → F in AAH60781 (PubMed:15489334).Curated | 1 | |
Sequence conflicti | 246 | L → F in BAA91969 (PubMed:14702039).Curated | 1 | |
Sequence conflicti | 331 | D → G in AAH60781 (PubMed:15489334).Curated | 1 | |
Sequence conflicti | 587 | L → P in CAD56956 (PubMed:12482879).Curated | 1 |
Alternative sequence
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Alternative sequenceiVSP_019153 | 224 – 247 | QALED…DSGLT → RFGFEQVLVLLFLQFWALLC TKHY in isoform 6. 1 PublicationAdd BLAST | 24 | |
Alternative sequenceiVSP_019154 | 248 – 618 | Missing in isoform 6. 1 PublicationAdd BLAST | 371 | |
Alternative sequenceiVSP_019155 | 580 | P → PEDHYRDRLSRDMGHTDRIE NLRKIWVFLKTAL in isoform 2. 2 Publications | 1 | |
Alternative sequenceiVSP_019156 | 580 | P → PEDHYRDRLSRDMGHTDRIE NLRKIWVFLKTAFHARLRCM CTCNRCTFCICQNFLNSDLL QSVKNKIFTAVLNR in isoform 3. 3 Publications | 1 | |
Alternative sequenceiVSP_047651 | 580 | P → PHARLRCMCTCNRCTFCICQ NFLNSDLLQSVKNKIFTAVL NR in isoform 7. Curated | 1 | |
Alternative sequenceiVSP_019157 | 581 – 618 | Missing in isoform 4. 1 PublicationAdd BLAST | 38 | |
Alternative sequenceiVSP_019158 | 582 – 618 | VTQAD…LLKQR → ARLRCMCTCNRCTFCICQNF LNSDLLQSVKNKIFTAVLNR IISA in isoform 5. 1 PublicationAdd BLAST | 37 |
Sequence databases
Genome annotation databases
Ensembli | ENST00000333942.10; ENSP00000334724.6; ENSG00000126858.19 ENST00000358365.7; ENSP00000351132.3; ENSG00000126858.19 [Q8IXI2-3] ENST00000394692.6; ENSP00000378184.2; ENSG00000126858.19 [Q8IXI2-2] ENST00000545287.7; ENSP00000439737.2; ENSG00000126858.19 [Q8IXI2-7] ENST00000581031.5; ENSP00000464094.1; ENSG00000126858.19 [Q8IXI2-5] ENST00000581094.5; ENSP00000462669.1; ENSG00000126858.19 [Q8IXI2-4] |
GeneIDi | 55288 |
KEGGi | hsa:55288 |
MANE-Selecti | ENST00000545287.7; ENSP00000439737.2; NM_001033566.3; NP_001028738.1 [Q8IXI2-7] |
UCSCi | uc002hgv.4, human [Q8IXI2-1] |
Keywords - Coding sequence diversityi
Alternative splicingSimilar proteinsi
Cross-referencesi
Sequence databases
3D structure databases
Select the link destinations: PDBei RCSB PDBi PDBji Links Updated | PDB entry | Method | Resolution (Å) | Chain | Positions | PDBsum |
5KSO | X-ray | 2.25 | A | 411-592 | [»] | |
5KSP | X-ray | 2.16 | A/B | 411-592 | [»] | |
5KSY | X-ray | 2.48 | A | 411-592 | [»] | |
5KSZ | X-ray | 2.50 | A | 177-592 | [»] | |
5KTY | X-ray | 2.52 | A | 177-592 | [»] | |
5KU1 | X-ray | 2.50 | A | 177-592 | [»] | |
6D71 | X-ray | 1.72 | A/B | 2-180 | [»] | |
AlphaFoldDBi | Q8IXI2 | |||||
SMRi | Q8IXI2 | |||||
ModBasei | Search... | |||||
PDBe-KBi | Search... |
Protein-protein interaction databases
BioGRIDi | 120576, 83 interactors |
CORUMi | Q8IXI2 |
DIPi | DIP-39114N |
IntActi | Q8IXI2, 30 interactors |
MINTi | Q8IXI2 |
PTM databases
GlyGeni | Q8IXI2, 1 site, 1 O-linked glycan (1 site) |
iPTMneti | Q8IXI2 |
PhosphoSitePlusi | Q8IXI2 |
Genetic variation databases
BioMutai | RHOT1 |
DMDMi | 108860796 |
Proteomic databases
EPDi | Q8IXI2 |
jPOSTi | Q8IXI2 |
MassIVEi | Q8IXI2 |
MaxQBi | Q8IXI2 |
PaxDbi | Q8IXI2 |
PeptideAtlasi | Q8IXI2 |
PRIDEi | Q8IXI2 |
ProteomicsDBi | 71002 [Q8IXI2-1] 71003 [Q8IXI2-2] 71004 [Q8IXI2-3] 71005 [Q8IXI2-4] 71006 [Q8IXI2-5] 71007 [Q8IXI2-6] |
Protocols and materials databases
Antibodypediai | 2419, 245 antibodies from 25 providers |
DNASUi | 55288 |
Genome annotation databases
Organism-specific databases
CTDi | 55288 |
DisGeNETi | 55288 |
GeneCardsi | RHOT1 |
HGNCi | HGNC:21168, RHOT1 |
HPAi | ENSG00000126858, Low tissue specificity |
MIMi | 613888, gene |
neXtProti | NX_Q8IXI2 |
OpenTargetsi | ENSG00000126858 |
PharmGKBi | PA134906318 |
VEuPathDBi | HostDB:ENSG00000126858 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG1707, Eukaryota |
GeneTreei | ENSGT00940000155641 |
HOGENOMi | CLU_014255_3_1_1 |
InParanoidi | Q8IXI2 |
OMAi | HVSVTWN |
OrthoDBi | 538388at2759 |
PhylomeDBi | Q8IXI2 |
TreeFami | TF300814 |
Enzyme and pathway databases
PathwayCommonsi | Q8IXI2 |
Reactomei | R-HSA-5689880, Ub-specific processing proteases R-HSA-9013425, RHOT1 GTPase cycle |
SignaLinki | Q8IXI2 |
Miscellaneous databases
BioGRID-ORCSi | 55288, 32 hits in 1078 CRISPR screens |
ChiTaRSi | RHOT1, human |
GeneWikii | RHOT1 |
GenomeRNAii | 55288 |
Pharosi | Q8IXI2, Tbio |
PROi | PR:Q8IXI2 |
RNActi | Q8IXI2, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000126858, Expressed in substantia nigra and 247 other tissues |
ExpressionAtlasi | Q8IXI2, baseline and differential |
Genevisiblei | Q8IXI2, HS |
Family and domain databases
Gene3Di | 3.40.50.300, 2 hits |
InterProi | View protein in InterPro IPR011992, EF-hand-dom_pair IPR018247, EF_Hand_1_Ca_BS IPR013566, EF_hand_assoc_1 IPR013567, EF_hand_assoc_2 IPR002048, EF_hand_dom IPR021181, Miro IPR029506, Miro-1 IPR020860, MIRO_dom IPR027417, P-loop_NTPase IPR005225, Small_GTP-bd_dom IPR001806, Small_GTPase IPR003578, Small_GTPase_Rho |
PANTHERi | PTHR24072, PTHR24072, 1 hit PTHR24072:SF124, PTHR24072:SF124, 1 hit |
Pfami | View protein in Pfam PF08355, EF_assoc_1, 1 hit PF08356, EF_assoc_2, 1 hit PF00071, Ras, 1 hit |
PIRSFi | PIRSF037488, Mt_Rho_GTPase, 1 hit |
SMARTi | View protein in SMART SM00054, EFh, 2 hits SM00174, RHO, 1 hit |
SUPFAMi | SSF47473, SSF47473, 1 hit SSF52540, SSF52540, 2 hits |
TIGRFAMsi | TIGR00231, small_GTP, 1 hit |
PROSITEi | View protein in PROSITE PS00018, EF_HAND_1, 1 hit PS50222, EF_HAND_2, 2 hits PS51423, MIRO, 2 hits |
MobiDBi | Search... |
Entry informationi
Entry namei | MIRO1_HUMAN | |
Accessioni | Q8IXI2Primary (citable) accession number: Q8IXI2 Secondary accession number(s): A4FVB6 Q9NUZ2 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | June 13, 2006 |
Last sequence update: | June 13, 2006 | |
Last modified: | May 25, 2022 | |
This is version 175 of the entry and version 2 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program | |
Disclaimer | Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care. |
Miscellaneousi
Keywords - Technical termi
3D-structure, Reference proteomeDocuments
- Human chromosome 17
Human chromosome 17: entries, gene names and cross-references to MIM - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - PDB cross-references
Index of Protein Data Bank (PDB) cross-references - SIMILARITY comments
Index of protein domains and families