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Protein

Mitochondrial Rho GTPase 2

Gene

RHOT2

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -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

Keywordsi

Molecular functionHydrolase
LigandCalcium, 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

EuPathDBiHostDB:ENSG00000140983.13
HGNCiHGNC:21169 RHOT2
MIMi613889 gene
neXtProtiNX_Q8IXI1

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

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

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). Interacts with ARMCX3 (By similarity).By similarity

Binary interactionsi

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

Protein-protein interaction databases

BioGridi12464640 interactors.
CORUMiQ8IXI1
IntActiQ8IXI1 13 interactors.
MINTiQ8IXI1
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

Keywords - Domaini

Repeat, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG1707 Eukaryota
ENOG410XRHW LUCA
GeneTreeiENSGT00390000014374
HOVERGENiHBG079778
InParanoidiQ8IXI1
KOiK07871
OMAiTELNHFG
OrthoDBiEOG091G04UF
PhylomeDBiQ8IXI1
TreeFamiTF300814

Family and domain databases

InterProiView 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
IPR020860 MIRO_dom
IPR027417 P-loop_NTPase
IPR001806 Small_GTPase
PfamiView protein in Pfam
PF08355 EF_assoc_1, 1 hit
PF08356 EF_assoc_2, 1 hit
PF00071 Ras, 1 hit
PIRSFiPIRSF037488 Mt_Rho_GTPase, 1 hit
SUPFAMiSSF47473 SSF47473, 2 hits
SSF52540 SSF52540, 2 hits
PROSITEiView protein in PROSITE
PS00018 EF_HAND_1, 1 hit
PS50222 EF_HAND_2, 2 hits
PS51423 MIRO, 2 hits

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 → Q1 PublicationCorresponds to variant dbSNP:rs1139897Ensembl.1
Natural variantiVAR_026638425R → C1 PublicationCorresponds to variant dbSNP:rs3177338Ensembl.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 No translation available.
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

Similar proteinsi

Entry informationi

Entry nameiMIRO2_HUMAN
AccessioniPrimary (citable) accession number: Q8IXI1
Secondary accession number(s): A2IDC2
, Q8NF53, Q96C13, Q96S17, Q9BT60, Q9H7M8
Entry historyiIntegrated into UniProtKB/Swiss-Prot: June 13, 2006
Last sequence update: June 13, 2006
Last modified: March 28, 2018
This is version 148 of the entry and version 2 of the sequence. See 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