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Protein

Mitochondrial Rho GTPase 2

Gene

RHOT2

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Mitochondrial GTPase involved in mitochondrial trafficking. Probably involved in control of anterograde transport of mitochondria and their subcellular distribution (By similarity).By similarity1 Publication

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi11 – 18GTP 1Sequence analysis8
Nucleotide bindingi57 – 61GTP 1Sequence analysis5
Nucleotide bindingi118 – 121GTP 1Sequence analysis4
Calcium bindingi197 – 2081PROSITE-ProRule annotationAdd BLAST12
Calcium bindingi317 – 3282PROSITE-ProRule annotationAdd BLAST12
Nucleotide bindingi423 – 430GTP 2Sequence analysis8
Nucleotide bindingi459 – 463GTP 2Sequence analysis5
Nucleotide bindingi524 – 527GTP 2Sequence analysis4

GO - Molecular functioni

GO - Biological processi

  • cellular homeostasis Source: UniProtKB
  • mitochondrial outer membrane permeabilization Source: UniProtKB
  • mitochondrion transport along microtubule Source: UniProtKB
  • regulation of small GTPase mediated signal transduction Source: Reactome
  • small GTPase mediated signal transduction Source: InterPro
Complete GO annotation...

Keywords - Molecular functioni

Hydrolase

Keywords - Ligandi

Calcium, GTP-binding, Metal-binding, Nucleotide-binding

Enzyme and pathway databases

ReactomeiR-HSA-194840. Rho GTPase cycle.

Names & Taxonomyi

Protein namesi
Recommended name:
Mitochondrial Rho GTPase 2 (EC:3.6.5.-)
Short name:
MIRO-2
Short name:
hMiro-2
Alternative name(s):
Ras homolog gene family member T2
Gene namesi
Name:RHOT2
Synonyms:ARHT2, C16orf39
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 16

Organism-specific databases

HGNCiHGNC:21169. RHOT2.

Subcellular locationi

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini1 – 592Mitochondrial intermembraneSequence analysisAdd BLAST592
Transmembranei593 – 615Helical; Anchor for type IV membrane proteinSequence analysisAdd BLAST23
Topological domaini616 – 618CytoplasmicSequence analysis3

GO - Cellular componenti

  • cytosol Source: Reactome
  • extracellular exosome Source: UniProtKB
  • integral component of mitochondrial outer membrane Source: UniProtKB
  • membrane Source: UniProtKB
  • plasma membrane Source: Reactome
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion outer membrane

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi13A → V: Causes constitutive activation inducing an aggregation of the mitochondrial network. 2 Publications1
Mutagenesisi18T → N: Induces an aggregation of the mitochondrial network. 1 Publication1

Organism-specific databases

OpenTargetsiENSG00000140983.
PharmGKBiPA134889674.

Polymorphism and mutation databases

BioMutaiRHOT2.
DMDMi108860798.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00002393181 – 618Mitochondrial Rho GTPase 2Add BLAST618

Proteomic databases

EPDiQ8IXI1.
MaxQBiQ8IXI1.
PaxDbiQ8IXI1.
PeptideAtlasiQ8IXI1.
PRIDEiQ8IXI1.

PTM databases

iPTMnetiQ8IXI1.
PhosphoSitePlusiQ8IXI1.
SwissPalmiQ8IXI1.

Expressioni

Tissue specificityi

Ubiquitously expressed. Highly expressed in heart, liver, skeletal muscle, kidney and pancreas.2 Publications

Gene expression databases

BgeeiENSG00000140983.
CleanExiHS_RHOT2.
ExpressionAtlasiQ8IXI1. baseline and differential.
GenevisibleiQ8IXI1. HS.

Organism-specific databases

HPAiHPA012624.
HPA012895.

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.By similarity

Binary interactionsi

WithEntry#Exp.IntActNotes
TRAK1Q9UPV93EBI-1396563,EBI-1105048

Protein-protein interaction databases

BioGridi124646. 38 interactors.
IntActiQ8IXI1. 10 interactors.
MINTiMINT-1191538.
STRINGi9606.ENSP00000321971.

Structurei

Secondary structure

1618
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi415 – 423Combined sources9
Helixi429 – 436Combined sources8
Beta strandi453 – 460Combined sources8
Beta strandi463 – 473Combined sources11
Helixi476 – 479Combined sources4
Beta strandi486 – 494Combined sources9
Helixi500 – 511Combined sources12
Turni512 – 514Combined sources3
Beta strandi515 – 517Combined sources3
Beta strandi519 – 524Combined sources6
Beta strandi534 – 536Combined sources3
Helixi539 – 545Combined sources7
Beta strandi558 – 560Combined sources3
Helixi565 – 575Combined sources11
Helixi577 – 579Combined sources3

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
5KUTX-ray1.69A/B/C409-588[»]
ProteinModelPortaliQ8IXI1.
SMRiQ8IXI1.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini1 – 142Miro 1Add BLAST142
Domaini184 – 219EF-hand 1PROSITE-ProRule annotationAdd BLAST36
Domaini304 – 339EF-hand 2PROSITE-ProRule annotationAdd BLAST36
Domaini410 – 618Miro 2Add BLAST209

Sequence similaritiesi

Belongs to the mitochondrial Rho GTPase family.Curated
Contains 2 EF-hand domains.PROSITE-ProRule annotation
Contains 2 Miro domains.Curated

Keywords - Domaini

Repeat, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG1707. Eukaryota.
ENOG410XRHW. LUCA.
GeneTreeiENSGT00390000014374.
HOVERGENiHBG079778.
InParanoidiQ8IXI1.
KOiK07871.
OMAiVTWSSIQ.
OrthoDBiEOG091G04UF.
PhylomeDBiQ8IXI1.
TreeFamiTF300814.

Family and domain databases

Gene3Di1.10.238.10. 2 hits.
3.40.50.300. 2 hits.
InterProiIPR011992. EF-hand-dom_pair.
IPR018247. EF_Hand_1_Ca_BS.
IPR013566. EF_hand_assoc_1.
IPR013567. EF_hand_assoc_2.
IPR002048. EF_hand_dom.
IPR020860. MIRO_dom.
IPR021181. Mt_Rho_GTPase.
IPR027417. P-loop_NTPase.
IPR029506. Rho_GTPase_2.
IPR001806. Small_GTPase.
[Graphical view]
PANTHERiPTHR24072:SF70. PTHR24072:SF70. 1 hit.
PfamiPF08355. EF_assoc_1. 1 hit.
PF08356. EF_assoc_2. 1 hit.
PF00071. Ras. 1 hit.
[Graphical view]
PIRSFiPIRSF037488. Mt_Rho_GTPase. 1 hit.
SUPFAMiSSF47473. SSF47473. 2 hits.
SSF52540. SSF52540. 2 hits.
PROSITEiPS00018. EF_HAND_1. 1 hit.
PS50222. EF_HAND_2. 2 hits.
PS51423. MIRO. 2 hits.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q8IXI1-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MRRDVRILLL GEAQVGKTSL ILSLVGEEFP EEVPPRAEEI TIPADVTPEK
60 70 80 90 100
VPTHIVDYSE AEQTDEELRE EIHKANVVCV VYDVSEEATI EKIRTKWIPL
110 120 130 140 150
VNGGTTQGPR VPIILVGNKS DLRSGSSMEA VLPIMSQFPE IETCVECSAK
160 170 180 190 200
NLRNISELFY YAQKAVLHPT APLYDPEAKQ LRPACAQALT RIFRLSDQDL
210 220 230 240 250
DQALSDEELN AFQKSCFGHP LAPQALEDVK TVVCRNVAGG VREDRLTLDG
260 270 280 290 300
FLFLNTLFIQ RGRHETTWTI LRRFGYSDAL ELTADYLSPL IHVPPGCSTE
310 320 330 340 350
LNHLGYQFVQ RVFEKHDQDR DGALSPVELQ SLFSVFPAAP WGPELPRTVR
360 370 380 390 400
TEAGRLPLHG YLCQWTLVTY LDVRSCLGHL GYLGYPTLCE QDQAHAITVT
410 420 430 440 450
REKRLDQEKG QTQRSVLLCK VVGARGVGKS AFLQAFLGRG LGHQDTREQP
460 470 480 490 500
PGYAIDTVQV NGQEKYLILC EVGTDGLLAT SLDATCDVAC LMFDGSDPKS
510 520 530 540 550
FAHCASVYKH HYMDGQTPCL FVSSKADLPE GVAVSGPSPA EFCRKHRLPA
560 570 580 590 600
PVPFSCAGPA EPSTTIFTQL ATMAAFPHLV HAELHPSSFW LRGLLGVVGA
610
AVAAVLSFSL YRVLVKSQ
Length:618
Mass (Da):68,118
Last modified:June 13, 2006 - v2
Checksum:iC72ECBCA33D04B6B
GO
Isoform 2 (identifier: Q8IXI1-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-127: Missing.
     251-340: FLFLNTLFIQ...SLFSVFPAAP → EAGCPPVPGE...PWLPVCAESV
     341-618: Missing.

Show »
Length:213
Mass (Da):23,077
Checksum:i76722819B54E29DE
GO

Sequence cautioni

The sequence AAK61240 differs from that shown. Reason: Erroneous gene model prediction.Curated
The sequence BAB15740 differs from that shown. Reason: Erroneous initiation.Curated
The sequence BAC03407 differs from that shown. Reason: Erroneous initiation.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti139P → S in CAD56957 (PubMed:12482879).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_026637245R → Q.1 PublicationCorresponds to variant rs1139897dbSNPEnsembl.1
Natural variantiVAR_026638425R → C.1 PublicationCorresponds to variant rs3177338dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0191621 – 127Missing in isoform 2. 2 PublicationsAdd BLAST127
Alternative sequenceiVSP_019163251 – 340FLFLN…FPAAP → EAGCPPVPGECGEGAVPGAP PALSRCRFPLPEHALHPARP ARDHLDHPAALRLQRCPGAD CGLSLPSDPRAPRLQHGAQP PWLPVCAESV in isoform 2. 2 PublicationsAdd BLAST90
Alternative sequenceiVSP_019164341 – 618Missing in isoform 2. 2 PublicationsAdd BLAST278

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ517413 mRNA. Translation: CAD56957.1.
AY207375 mRNA. Translation: AAP46090.1.
AK024450 mRNA. Translation: BAB15740.1. Different initiation.
AK090426 mRNA. Translation: BAC03407.1. Different initiation.
AE006464 Genomic DNA. Translation: AAK61240.1. Sequence problems.
Z92544 Genomic DNA. Translation: CAM26351.1.
CH471112 Genomic DNA. Translation: EAW85763.1.
BC004327 mRNA. Translation: AAH04327.1.
BC014942 mRNA. Translation: AAH14942.1.
CCDSiCCDS10417.1. [Q8IXI1-1]
RefSeqiNP_620124.1. NM_138769.2. [Q8IXI1-1]
UniGeneiHs.513242.

Genome annotation databases

EnsembliENST00000315082; ENSP00000321971; ENSG00000140983. [Q8IXI1-1]
GeneIDi89941.
KEGGihsa:89941.
UCSCiuc002cip.4. human. [Q8IXI1-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ517413 mRNA. Translation: CAD56957.1.
AY207375 mRNA. Translation: AAP46090.1.
AK024450 mRNA. Translation: BAB15740.1. Different initiation.
AK090426 mRNA. Translation: BAC03407.1. Different initiation.
AE006464 Genomic DNA. Translation: AAK61240.1. Sequence problems.
Z92544 Genomic DNA. Translation: CAM26351.1.
CH471112 Genomic DNA. Translation: EAW85763.1.
BC004327 mRNA. Translation: AAH04327.1.
BC014942 mRNA. Translation: AAH14942.1.
CCDSiCCDS10417.1. [Q8IXI1-1]
RefSeqiNP_620124.1. NM_138769.2. [Q8IXI1-1]
UniGeneiHs.513242.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
5KUTX-ray1.69A/B/C409-588[»]
ProteinModelPortaliQ8IXI1.
SMRiQ8IXI1.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi124646. 38 interactors.
IntActiQ8IXI1. 10 interactors.
MINTiMINT-1191538.
STRINGi9606.ENSP00000321971.

PTM databases

iPTMnetiQ8IXI1.
PhosphoSitePlusiQ8IXI1.
SwissPalmiQ8IXI1.

Polymorphism and mutation databases

BioMutaiRHOT2.
DMDMi108860798.

Proteomic databases

EPDiQ8IXI1.
MaxQBiQ8IXI1.
PaxDbiQ8IXI1.
PeptideAtlasiQ8IXI1.
PRIDEiQ8IXI1.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000315082; ENSP00000321971; ENSG00000140983. [Q8IXI1-1]
GeneIDi89941.
KEGGihsa:89941.
UCSCiuc002cip.4. human. [Q8IXI1-1]

Organism-specific databases

CTDi89941.
GeneCardsiRHOT2.
HGNCiHGNC:21169. RHOT2.
HPAiHPA012624.
HPA012895.
MIMi613889. gene.
neXtProtiNX_Q8IXI1.
OpenTargetsiENSG00000140983.
PharmGKBiPA134889674.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG1707. Eukaryota.
ENOG410XRHW. LUCA.
GeneTreeiENSGT00390000014374.
HOVERGENiHBG079778.
InParanoidiQ8IXI1.
KOiK07871.
OMAiVTWSSIQ.
OrthoDBiEOG091G04UF.
PhylomeDBiQ8IXI1.
TreeFamiTF300814.

Enzyme and pathway databases

ReactomeiR-HSA-194840. Rho GTPase cycle.

Miscellaneous databases

ChiTaRSiRHOT2. human.
GeneWikiiRHOT2.
GenomeRNAii89941.
PROiQ8IXI1.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000140983.
CleanExiHS_RHOT2.
ExpressionAtlasiQ8IXI1. baseline and differential.
GenevisibleiQ8IXI1. HS.

Family and domain databases

Gene3Di1.10.238.10. 2 hits.
3.40.50.300. 2 hits.
InterProiIPR011992. EF-hand-dom_pair.
IPR018247. EF_Hand_1_Ca_BS.
IPR013566. EF_hand_assoc_1.
IPR013567. EF_hand_assoc_2.
IPR002048. EF_hand_dom.
IPR020860. MIRO_dom.
IPR021181. Mt_Rho_GTPase.
IPR027417. P-loop_NTPase.
IPR029506. Rho_GTPase_2.
IPR001806. Small_GTPase.
[Graphical view]
PANTHERiPTHR24072:SF70. PTHR24072:SF70. 1 hit.
PfamiPF08355. EF_assoc_1. 1 hit.
PF08356. EF_assoc_2. 1 hit.
PF00071. Ras. 1 hit.
[Graphical view]
PIRSFiPIRSF037488. Mt_Rho_GTPase. 1 hit.
SUPFAMiSSF47473. SSF47473. 2 hits.
SSF52540. SSF52540. 2 hits.
PROSITEiPS00018. EF_HAND_1. 1 hit.
PS50222. EF_HAND_2. 2 hits.
PS51423. MIRO. 2 hits.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiMIRO2_HUMAN
AccessioniPrimary (citable) accession number: Q8IXI1
Secondary accession number(s): A2IDC2
, Q8NF53, Q96C13, Q96S17, Q9BT60, Q9H7M8
Entry historyi
Integrated into UniProtKB/Swiss-Prot: June 13, 2006
Last sequence update: June 13, 2006
Last modified: November 30, 2016
This is version 136 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny 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, Complete proteome, Reference proteome

Documents

  1. Human chromosome 16
    Human chromosome 16: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.