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Q8IXI1 (MIRO2_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified July 9, 2014. Version 111. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (5) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Alt products·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Mitochondrial Rho GTPase 2

Short name=MIRO-2
Short name=hMiro-2
EC=3.6.5.-
Alternative name(s):
Ras homolog gene family member T2
Gene names
Name:RHOT2
Synonyms:ARHT2, C16orf39
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length618 AA.
Sequence statusComplete.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

Mitochondrial GTPase involved in mitochondrial trafficking. Probably involved in control of anterograde transport of mitochondria and their subcellular distribution By similarity. Ref.10

Subunit structure

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. Ref.10

Subcellular location

Mitochondrion outer membrane; Single-pass type IV membrane protein. Note: Colocalizes with MGARP and RHOT2 at the mitochondria. Ref.1 Ref.11

Tissue specificity

Ubiquitously expressed. Highly expressed in heart, liver, skeletal muscle, kidney and pancreas. Ref.1 Ref.2

Sequence similarities

Belongs to the mitochondrial Rho GTPase family.

Contains 2 EF-hand domains.

Contains 2 Miro domains.

Sequence caution

The sequence AAK61240.1 differs from that shown. Reason: Erroneous gene model prediction.

The sequence BAB15740.1 differs from that shown. Reason: Erroneous initiation.

The sequence BAC03407.1 differs from that shown. Reason: Erroneous initiation.

Alternative products

This entry describes 2 isoforms produced by alternative splicing. [Align] [Select]
Isoform 1 (identifier: Q8IXI1-1)

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.
Isoform 2 (identifier: Q8IXI1-2)

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

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Chain1 – 618618Mitochondrial Rho GTPase 2
PRO_0000239318

Regions

Topological domain1 – 592592Mitochondrial intermembrane Potential
Transmembrane593 – 61523Helical; Anchor for type IV membrane protein; Potential
Topological domain616 – 6183Cytoplasmic Potential
Domain1 – 142142Miro 1
Domain184 – 21936EF-hand 1
Domain304 – 33936EF-hand 2
Domain410 – 618209Miro 2
Nucleotide binding11 – 188GTP 1 Potential
Nucleotide binding57 – 615GTP 1 Potential
Nucleotide binding118 – 1214GTP 1 Potential
Calcium binding197 – 208121 Potential
Calcium binding317 – 328122 Potential
Nucleotide binding423 – 4308GTP 2 Potential
Nucleotide binding459 – 4635GTP 2 Potential
Nucleotide binding524 – 5274GTP 2 Potential

Natural variations

Alternative sequence1 – 127127Missing in isoform 2.
VSP_019162
Alternative sequence251 – 34090FLFLN…FPAAP → EAGCPPVPGECGEGAVPGAP PALSRCRFPLPEHALHPARP ARDHLDHPAALRLQRCPGAD CGLSLPSDPRAPRLQHGAQP PWLPVCAESV in isoform 2.
VSP_019163
Alternative sequence341 – 618278Missing in isoform 2.
VSP_019164
Natural variant2451R → Q. Ref.1
Corresponds to variant rs1139897 [ dbSNP | Ensembl ].
VAR_026637
Natural variant4251R → C. Ref.1
Corresponds to variant rs3177338 [ dbSNP | Ensembl ].
VAR_026638

Experimental info

Mutagenesis131A → V: Causes constitutive activation inducing an aggregation of the mitochondrial network. Ref.9 Ref.10
Mutagenesis181T → N: Induces an aggregation of the mitochondrial network. Ref.10
Sequence conflict1391P → S in CAD56957. Ref.1

Sequences

Sequence LengthMass (Da)Tools
Isoform 1 [UniParc].

Last modified June 13, 2006. Version 2.
Checksum: C72ECBCA33D04B6B

FASTA61868,118
        10         20         30         40         50         60 
MRRDVRILLL GEAQVGKTSL ILSLVGEEFP EEVPPRAEEI TIPADVTPEK VPTHIVDYSE 

        70         80         90        100        110        120 
AEQTDEELRE EIHKANVVCV VYDVSEEATI EKIRTKWIPL VNGGTTQGPR VPIILVGNKS 

       130        140        150        160        170        180 
DLRSGSSMEA VLPIMSQFPE IETCVECSAK NLRNISELFY YAQKAVLHPT APLYDPEAKQ 

       190        200        210        220        230        240 
LRPACAQALT RIFRLSDQDL DQALSDEELN AFQKSCFGHP LAPQALEDVK TVVCRNVAGG 

       250        260        270        280        290        300 
VREDRLTLDG FLFLNTLFIQ RGRHETTWTI LRRFGYSDAL ELTADYLSPL IHVPPGCSTE 

       310        320        330        340        350        360 
LNHLGYQFVQ RVFEKHDQDR DGALSPVELQ SLFSVFPAAP WGPELPRTVR TEAGRLPLHG 

       370        380        390        400        410        420 
YLCQWTLVTY LDVRSCLGHL GYLGYPTLCE QDQAHAITVT REKRLDQEKG QTQRSVLLCK 

       430        440        450        460        470        480 
VVGARGVGKS AFLQAFLGRG LGHQDTREQP PGYAIDTVQV NGQEKYLILC EVGTDGLLAT 

       490        500        510        520        530        540 
SLDATCDVAC LMFDGSDPKS FAHCASVYKH HYMDGQTPCL FVSSKADLPE GVAVSGPSPA 

       550        560        570        580        590        600 
EFCRKHRLPA PVPFSCAGPA EPSTTIFTQL ATMAAFPHLV HAELHPSSFW LRGLLGVVGA 

       610 
AVAAVLSFSL YRVLVKSQ 

« Hide

Isoform 2 [UniParc].

Checksum: 76722819B54E29DE
Show »

FASTA21323,077

References

« Hide 'large scale' references
[1]"Atypical Rho GTPases have roles in mitochondrial homeostasis and apoptosis."
Fransson A., Ruusala A., Aspenstroem P.
J. Biol. Chem. 278:6495-6502(2003) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1), SUBCELLULAR LOCATION, TISSUE SPECIFICITY, VARIANTS GLN-245 AND CYS-425.
[2]"Cloning and characterization of the mouse Arht2 gene which encodes a putative atypical GTPase."
Shan Y., Hexige S., Guo Z., Wan B., Chen K., Chen X., Ma L., Huang C., Zhao S., Yu L.
Cytogenet. Genome Res. 106:91-97(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1), TISSUE SPECIFICITY.
[3]"Complete sequencing and characterization of 21,243 full-length human cDNAs."
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. expand/collapse author list , 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 T.-O., 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.
Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 2).
Tissue: Spleen.
[4]"The nucleotide sequence of a long cDNA clone isolated from human spleen."
Jikuya H., Takano J., Kikuno R., Nagase T., Ohara O.
Submitted (JUL-2002) to the EMBL/GenBank/DDBJ databases
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 2).
Tissue: Spleen.
[5]"Sequence, structure and pathology of the fully annotated terminal 2 Mb of the short arm of human chromosome 16."
Daniels R.J., Peden J.F., Lloyd C., Horsley S.W., Clark K., Tufarelli C., Kearney L., Buckle V.J., Doggett N.A., Flint J., Higgs D.R.
Hum. Mol. Genet. 10:339-352(2001) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[6]Mural R.J., Istrail S., Sutton G.G., Florea L., Halpern A.L., Mobarry C.M., Lippert R., Walenz B., Shatkay H., Dew I., Miller J.R., Flanigan M.J., Edwards N.J., Bolanos R., Fasulo D., Halldorsson B.V., Hannenhalli S., Turner R. expand/collapse author list , Yooseph S., Lu F., Nusskern D.R., Shue B.C., Zheng X.H., Zhong F., Delcher A.L., Huson D.H., Kravitz S.A., Mouchard L., Reinert K., Remington K.A., Clark A.G., Waterman M.S., Eichler E.E., Adams M.D., Hunkapiller M.W., Myers E.W., Venter J.C.
Submitted (SEP-2005) to the EMBL/GenBank/DDBJ databases
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[7]"The sequence and analysis of duplication-rich human chromosome 16."
Martin J., Han C., Gordon L.A., Terry A., Prabhakar S., She X., Xie G., Hellsten U., Chan Y.M., Altherr M., Couronne O., Aerts A., Bajorek E., Black S., Blumer H., Branscomb E., Brown N.C., Bruno W.J. expand/collapse author list , Buckingham J.M., Callen D.F., Campbell C.S., Campbell M.L., Campbell E.W., Caoile C., Challacombe J.F., Chasteen L.A., Chertkov O., Chi H.C., Christensen M., Clark L.M., Cohn J.D., Denys M., Detter J.C., Dickson M., Dimitrijevic-Bussod M., Escobar J., Fawcett J.J., Flowers D., Fotopulos D., Glavina T., Gomez M., Gonzales E., Goodstein D., Goodwin L.A., Grady D.L., Grigoriev I., Groza M., Hammon N., Hawkins T., Haydu L., Hildebrand C.E., Huang W., Israni S., Jett J., Jewett P.B., Kadner K., Kimball H., Kobayashi A., Krawczyk M.-C., Leyba T., Longmire J.L., Lopez F., Lou Y., Lowry S., Ludeman T., Manohar C.F., Mark G.A., McMurray K.L., Meincke L.J., Morgan J., Moyzis R.K., Mundt M.O., Munk A.C., Nandkeshwar R.D., Pitluck S., Pollard M., Predki P., Parson-Quintana B., Ramirez L., Rash S., Retterer J., Ricke D.O., Robinson D.L., Rodriguez A., Salamov A., Saunders E.H., Scott D., Shough T., Stallings R.L., Stalvey M., Sutherland R.D., Tapia R., Tesmer J.G., Thayer N., Thompson L.S., Tice H., Torney D.C., Tran-Gyamfi M., Tsai M., Ulanovsky L.E., Ustaszewska A., Vo N., White P.S., Williams A.L., Wills P.L., Wu J.-R., Wu K., Yang J., DeJong P., Bruce D., Doggett N.A., Deaven L., Schmutz J., Grimwood J., Richardson P., Rokhsar D.S., Eichler E.E., Gilna P., Lucas S.M., Myers R.M., Rubin E.M., Pennacchio L.A.
Nature 432:988-994(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[8]"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
The MGC Project Team
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1).
Tissue: Placenta and Skin.
[9]"Rho GTPases have diverse effects on the organization of the actin filament system."
Aspenstroem P., Fransson A., Saras J.
Biochem. J. 377:327-337(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: MUTAGENESIS OF ALA-13.
[10]"The atypical Rho GTPases Miro-1 and Miro-2 have essential roles in mitochondrial trafficking."
Fransson S., Ruusala A., Aspenstroem P.
Biochem. Biophys. Res. Commun. 344:500-510(2006) [PubMed] [Europe PMC] [Abstract]
Cited for: FUNCTION, INTERACTION WITH TRAK1 AND TRAK2, MUTAGENESIS OF ALA-13 AND THR-18.
[11]"HUMMR, a hypoxia- and HIF-1alpha-inducible protein, alters mitochondrial distribution and transport."
Li Y., Lim S., Hoffman D., Aspenstrom P., Federoff H.J., Rempe D.A.
J. Cell Biol. 185:1065-1081(2009) [PubMed] [Europe PMC] [Abstract]
Cited for: SUBCELLULAR LOCATION.
[12]"Initial characterization of the human central proteome."
Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P., Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J.
BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
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.
CCDSCCDS10417.1. [Q8IXI1-1]
RefSeqNP_620124.1. NM_138769.2. [Q8IXI1-1]
UniGeneHs.513242.

3D structure databases

ProteinModelPortalQ8IXI1.
SMRQ8IXI1. Positions 5-167, 180-578.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid124646. 28 interactions.
IntActQ8IXI1. 6 interactions.
MINTMINT-1191538.
STRING9606.ENSP00000321971.

PTM databases

PhosphoSiteQ8IXI1.

Polymorphism databases

DMDM108860798.

Proteomic databases

MaxQBQ8IXI1.
PaxDbQ8IXI1.
PRIDEQ8IXI1.

Protocols and materials databases

StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000315082; ENSP00000321971; ENSG00000140983. [Q8IXI1-1]
GeneID89941.
KEGGhsa:89941.
UCSCuc002cip.3. human. [Q8IXI1-1]

Organism-specific databases

CTD89941.
GeneCardsGC16P000826.
HGNCHGNC:21169. RHOT2.
HPAHPA012624.
HPA012895.
MIM613889. gene.
neXtProtNX_Q8IXI1.
PharmGKBPA134889674.
GenAtlasSearch...

Phylogenomic databases

eggNOGCOG1100.
HOVERGENHBG079778.
InParanoidQ8IXI1.
KOK07871.
OMAVLPQITI.
OrthoDBEOG7PK8Z0.
PhylomeDBQ8IXI1.
TreeFamTF300814.

Enzyme and pathway databases

ReactomeREACT_111102. Signal Transduction.

Gene expression databases

ArrayExpressQ8IXI1.
BgeeQ8IXI1.
CleanExHS_RHOT2.
GenevestigatorQ8IXI1.

Family and domain databases

Gene3D1.10.238.10. 2 hits.
3.40.50.300. 2 hits.
InterProIPR011992. 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.
IPR013684. MIRO-like.
IPR027417. P-loop_NTPase.
IPR029506. Rho_GTPase_2.
IPR021181. Rho_GTPase_Mt.
IPR001806. Small_GTPase.
[Graphical view]
PANTHERPTHR24072:SF70. PTHR24072:SF70. 1 hit.
PfamPF08355. EF_assoc_1. 1 hit.
PF08356. EF_assoc_2. 1 hit.
PF08477. Miro. 1 hit.
PF00071. Ras. 1 hit.
[Graphical view]
PIRSFPIRSF037488. Mt_Rho_GTPase. 1 hit.
PRINTSPR00449. RASTRNSFRMNG.
SMARTSM00054. EFh. 2 hits.
[Graphical view]
SUPFAMSSF52540. SSF52540. 2 hits.
PROSITEPS00018. EF_HAND_1. 1 hit.
PS50222. EF_HAND_2. 2 hits.
PS51423. MIRO. 2 hits.
[Graphical view]
ProtoNetSearch...

Other

GeneWikiRHOT2.
GenomeRNAi89941.
NextBio76445.
PROQ8IXI1.
SOURCESearch...

Entry information

Entry nameMIRO2_HUMAN
AccessionPrimary (citable) accession number: Q8IXI1
Secondary accession number(s): A2IDC2 expand/collapse secondary AC list , Q8NF53, Q96C13, Q96S17, Q9BT60, Q9H7M8
Entry history
Integrated into UniProtKB/Swiss-Prot: June 13, 2006
Last sequence update: June 13, 2006
Last modified: July 9, 2014
This is version 111 of the entry and version 2 of the sequence. [Complete history]
Entry statusReviewed (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.

Relevant documents

SIMILARITY comments

Index of protein domains and families

MIM cross-references

Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot

Human polymorphisms and disease mutations

Index of human polymorphisms and disease mutations

Human entries with polymorphisms or disease mutations

List of human entries with polymorphisms or disease mutations

Human chromosome 16

Human chromosome 16: entries, gene names and cross-references to MIM