Aliases for RHOT1 Gene

Aliases for RHOT1 Gene

  • Ras Homolog Family Member T1 2 3 5
  • MIRO-1 2 3 4
  • Ras Homolog Gene Family, Member T1 2 3
  • Mitochondrial Rho (MIRO) GTPase 1 2 3
  • Mitochondrial Rho GTPase 1 3 4
  • ARHT1 3 4
  • MIRO1 2 3
  • Rac-GTP Binding Protein-Like Protein 3
  • Rac-GTP-Binding Protein-Like Protein 4
  • Ras Homolog Gene Family Member T1 4
  • EC 3.6.5.- 4
  • FLJ11040 2
  • EC 3.6.5 50
  • HMiro-1 4
  • RHOT1 5

External Ids for RHOT1 Gene

Previous HGNC Symbols for RHOT1 Gene

  • ARHT1

Previous GeneCards Identifiers for RHOT1 Gene

  • GC17P030616
  • GC17P027493
  • GC17P030470
  • GC17P026657

Summaries for RHOT1 Gene

GeneCards Summary for RHOT1 Gene

RHOT1 (Ras Homolog Family Member T1) is a Protein Coding gene. Diseases associated with RHOT1 include Myopathy With Extrapyramidal Signs and Cataract 3, Multiple Types. Among its related pathways are p75 NTR receptor-mediated signalling and Signaling by GPCR. Gene Ontology (GO) annotations related to this gene include calcium ion binding and GTPase activity. An important paralog of this gene is RHOT2.

UniProtKB/Swiss-Prot Summary for RHOT1 Gene

  • Mitochondrial GTPase involved in mitochondrial trafficking. Probably involved in control of anterograde transport of mitochondria and their subcellular distribution.

Gene Wiki entry for RHOT1 Gene

No data available for Entrez Gene Summary , CIViC Summary , Tocris Summary , PharmGKB "VIP" Summary , Rfam classification and piRNA Summary for RHOT1 Gene

Genomics for RHOT1 Gene

GeneHancer (GH) Regulatory Elements Pubs

Promoters and enhancers for RHOT1 Gene
- Elite GeneHancer and/or Elite GeneHancer-gene association Download GeneHancer data from 2017 publication | Request up-to-date GeneHancer data (full dataset)

GeneHancers around RHOT1 on the GeneHancer Hub at the UCSC Golden Path

Cistromic (ChIP-Seq) regulation report from SPP (The Signaling Pathways Project) for RHOT1

Top Transcription factor binding sites by QIAGEN in the RHOT1 gene promoter:
  • AhR
  • Arnt
  • FOXD1
  • FOXO3a
  • FOXO3b
  • HFH-1
  • HTF
  • SRF
  • SRF (504 AA)
  • SRY

Genomic Locations for RHOT1 Gene

Latest Assembly
110,921 bases
Plus strand

Previous Assembly
(GRCh37/hg19 by Entrez Gene)
83,226 bases
Plus strand

(GRCh37/hg19 by Ensembl)
110,921 bases
Plus strand

Genomic View for RHOT1 Gene

Genes around RHOT1 on UCSC Golden Path with GeneCards custom track

Cytogenetic band:
RHOT1 Gene in genomic location: bands according to Ensembl, locations according to GeneLoc (and/or Entrez Gene and/or Ensembl if different)
Genomic Location for RHOT1 Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for RHOT1 Gene

Proteins for RHOT1 Gene

  • Protein details for RHOT1 Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Recommended name:
    Mitochondrial Rho GTPase 1
    Protein Accession:
    Secondary Accessions:
    • A4FVB6
    • A6NFV0
    • B4DG48
    • J9JIH9
    • Q6NUR3
    • Q6P9F8
    • Q6PJG1
    • Q6YMW8
    • Q86UB0
    • Q8IW28
    • Q8IXJ7
    • Q9H067
    • Q9H9N8
    • Q9NUZ2

    Protein attributes for RHOT1 Gene

    618 amino acids
    Molecular mass:
    70784 Da
    Quaternary structure:
    • 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 ARMCX1 (By similarity). Found in a complex with KIF5B, OGT, RHOT2 and TRAK1 (PubMed:24995978).
    • Sequence=AAH41114.1; Type=Erroneous initiation; Evidence={ECO:0000305}; Sequence=AAH68463.1; Type=Erroneous initiation; Evidence={ECO:0000305}; Sequence=BAA91969.1; Type=Erroneous initiation; Evidence={ECO:0000305}; Sequence=BAB14185.1; Type=Erroneous initiation; Evidence={ECO:0000305};

    Three dimensional structures from OCA and Proteopedia for RHOT1 Gene

    Alternative splice isoforms for RHOT1 Gene

neXtProt entry for RHOT1 Gene

Selected DME Specific Peptides for RHOT1 Gene


Post-translational modifications for RHOT1 Gene

  • Ubiquitinated by PRKN during mitophagy, leading to its degradation and enhancement of mitophagy. Deubiquitinated by USP30.
  • Ubiquitination at Lys153, Lys187, and Lys194
  • Modification sites at PhosphoSitePlus

Antibodies for research

  • Abcam antibodies for RHOT1
  • Boster Bio Antibodies for RHOT1

Domains & Families for RHOT1 Gene

Gene Families for RHOT1 Gene

Human Protein Atlas (HPA):
  • Predicted intracellular proteins
  • Predicted membrane proteins

Protein Domains for RHOT1 Gene

  • Miro-like
  • Ras small GTPase, Ras type
  • Ras small GTPase, Rho type
  • EF hand associated, type-2

Suggested Antigen Peptide Sequences for RHOT1 Gene

GenScript: Design optimal peptide antigens:
  • Ras homolog gene family member T1 (MIRO1_HUMAN)

Graphical View of Domain Structure for InterPro Entry



  • Belongs to the mitochondrial Rho GTPase family.
  • Belongs to the mitochondrial Rho GTPase family.
genes like me logo Genes that share domains with RHOT1: view

Function for RHOT1 Gene

Molecular function for RHOT1 Gene

UniProtKB/Swiss-Prot Function:
Mitochondrial GTPase involved in mitochondrial trafficking. Probably involved in control of anterograde transport of mitochondria and their subcellular distribution.

Enzyme Numbers (IUBMB) for RHOT1 Gene

Phenotypes From GWAS Catalog for RHOT1 Gene

Gene Ontology (GO) - Molecular Function for RHOT1 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0000166 nucleotide binding IEA --
GO:0003674 molecular_function ND --
GO:0003924 GTPase activity IEA,IBA 21873635
GO:0005509 calcium ion binding IEA --
GO:0005515 protein binding IPI 16630562
genes like me logo Genes that share ontologies with RHOT1: view
genes like me logo Genes that share phenotypes with RHOT1: view

Animal Models for RHOT1 Gene

MGI Knock Outs for RHOT1:
  • Rhot1 Rhot1<tm1.2Jmsu>
  • Rhot1 Rhot1<tm1d(EUCOMM)Wtsi>
  • Rhot1 Rhot1<tm1a(EUCOMM)Wtsi>

Inhibitory RNAs for research

  • Search GeneCopoeia for shRNA, lentivirus and/or AAV clone products for RHOT1

Clone products for research

  • Addgene plasmids for RHOT1

No data available for Human Phenotype Ontology , Transcription Factor Targets and HOMER Transcription for RHOT1 Gene

Localization for RHOT1 Gene

Subcellular locations from UniProtKB/Swiss-Prot for RHOT1 Gene

Mitochondrion outer membrane. Single-pass type IV membrane protein. Note=Colocalizes with MGARP and RHOT2 at the mitochondria.

Subcellular locations from

Extracellular space Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi Apparatus Nucleus Mitochondrion 0 1 2 3 4 5 Confidence
COMPARTMENTS Subcellular localization image for RHOT1 gene
Compartment Confidence
mitochondrion 5
cytosol 5
plasma membrane 4
extracellular 2
cytoskeleton 2
peroxisome 2
nucleus 2
endoplasmic reticulum 2
lysosome 2
endosome 1
golgi apparatus 1

Gene Ontology (GO) - Cellular Components for RHOT1 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005739 mitochondrion NAS 22078885
GO:0005741 mitochondrial outer membrane TAS --
GO:0005829 cytosol TAS --
GO:0005886 plasma membrane TAS --
GO:0016020 membrane IEA,HDA 19946888
genes like me logo Genes that share ontologies with RHOT1: view

No data available for Subcellular locations from the Human Protein Atlas (HPA) for RHOT1 Gene

Pathways & Interactions for RHOT1 Gene

genes like me logo Genes that share pathways with RHOT1: view

Pathways by source for RHOT1 Gene

Gene Ontology (GO) - Biological Process for RHOT1 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0007005 mitochondrion organization IBA 21873635
GO:0010821 regulation of mitochondrion organization IBA 21873635
GO:0016579 protein deubiquitination TAS --
GO:0019725 cellular homeostasis IMP 12482879
GO:0034640 establishment of mitochondrion localization by microtubule attachment IEA --
genes like me logo Genes that share ontologies with RHOT1: view

No data available for SIGNOR curated interactions for RHOT1 Gene

Drugs & Compounds for RHOT1 Gene

(1) Drugs for RHOT1 Gene - From: HMDB

Name Status Disease Links Group Role Mechanism of Action Clinical Trials
Guanosine triphosphate Experimental Pharma 0
genes like me logo Genes that share compounds with RHOT1: view

Transcripts for RHOT1 Gene

mRNA/cDNA for RHOT1 Gene

20 NCBI additional mRNA sequence :
19 Ensembl transcripts including schematic representations, and UCSC links to gene/alias where relevant :

Inhibitory RNAs for research

  • Search GeneCopoeia for shRNA, lentivirus and/or AAV clone products for RHOT1

Clone products for research

  • Addgene plasmids for RHOT1

Alternative Splicing Database (ASD) splice patterns (SP) for RHOT1 Gene

ExUns: 1 ^ 2 ^ 3 ^ 4a · 4b ^ 5a · 5b ^ 6 ^ 7a · 7b ^ 8 ^ 9 ^ 10 ^ 11a · 11b ^ 12a · 12b ^ 13 ^ 14a · 14b · 14c ^ 15 ^ 16a · 16b ^ 17 ^ 18a ·
SP1: -
SP2: - - -
SP3: - -
SP5: - -
SP6: - -
SP7: - -
SP8: -

ExUns: 18b ^ 19a · 19b · 19c · 19d · 19e
SP2: -
SP7: -
SP8: -

Relevant External Links for RHOT1 Gene

GeneLoc Exon Structure for

Expression for RHOT1 Gene

mRNA expression in normal human tissues from GTEx, Illumina, BioGPS, and SAGE for RHOT1 Gene

Protein differential expression in normal tissues from HIPED for RHOT1 Gene

This gene is overexpressed in Frontal cortex (7.8) and Retina (6.9).

Integrated Proteomics: protein expression in normal tissues and cell lines from ProteomicsDB, MaxQB, and MOPED for RHOT1 Gene

Protein tissue co-expression partners for RHOT1 Gene

- Elite partner

Transcriptomic regulation report from SPP (The Signaling Pathways Project) for RHOT1

SOURCE GeneReport for Unigene cluster for RHOT1 Gene:


mRNA Expression by UniProt/SwissProt for RHOT1 Gene:

Tissue specificity: Ubiquitously expressed. Expressed at high level in heart and skeletal muscle.

Evidence on tissue expression from TISSUES for RHOT1 Gene

  • Nervous system(4.8)
  • Lung(4.5)
  • Liver(4.4)
  • Heart(2.7)
  • Kidney(2.6)
  • Muscle(2.6)
  • Skin(2.3)
  • Spleen(2.1)
  • Eye(2.1)
  • Adrenal gland(2)
genes like me logo Genes that share expression patterns with RHOT1: view

No data available for mRNA expression in embryonic tissues and stem cells from LifeMap Discovery , mRNA differential expression in normal tissues and Phenotype-based relationships between genes and organs from Gene ORGANizer for RHOT1 Gene

Orthologs for RHOT1 Gene

This gene was present in the common ancestor of eukaryotes.

Orthologs for RHOT1 Gene

Organism Taxonomy Gene Similarity Type Details
(Pan troglodytes)
Mammalia RHOT1 29 30
  • 99.43 (n)
(Canis familiaris)
Mammalia RHOT1 29 30
  • 95.4 (n)
(Bos Taurus)
Mammalia RHOT1 29 30
  • 94.77 (n)
(Ornithorhynchus anatinus)
Mammalia RHOT1 30
  • 94 (a)
(Mus musculus)
Mammalia Rhot1 29 16 30
  • 92.72 (n)
(Rattus norvegicus)
Mammalia Rhot1 29
  • 92.18 (n)
(Monodelphis domestica)
Mammalia RHOT1 30
  • 90 (a)
(Gallus gallus)
Aves RHOT1 29 30
  • 84.74 (n)
(Anolis carolinensis)
Reptilia RHOT1 30
  • 93 (a)
Tropical Clawed Frog
(Silurana tropicalis)
Amphibia LOC100492251 29
  • 74.7 (n)
(Danio rerio)
Actinopterygii RHOT1 (3 of 3) 30
  • 85 (a)
rhot1b 30
  • 79 (a)
rhot1a 29 30
  • 74.49 (n)
Dr.6639 29
Fruit Fly
(Drosophila melanogaster)
Insecta Miro 29 30
  • 56.08 (n)
African malaria mosquito
(Anopheles gambiae)
Insecta AgaP_AGAP007998 29
  • 55.57 (n)
(Caenorhabditis elegans)
Secernentea miro-1 29 30
  • 51.85 (n)
miro-2 30
  • 42 (a)
Baker's yeast
(Saccharomyces cerevisiae)
Saccharomycetes GEM1 30 32
  • 30 (a)
Thale Cress
(Arabidopsis thaliana)
eudicotyledons MIRO2 29
  • 48.94 (n)
Sea Squirt
(Ciona savignyi)
Ascidiacea -- 30
  • 50 (a)
Species where no ortholog for RHOT1 was found in the sources mined by GeneCards:
  • A. gosspyii yeast (Eremothecium gossypii)
  • Actinobacteria (Mycobacterium tuberculosis)
  • African clawed frog (Xenopus laevis)
  • Alicante grape (Vitis vinifera)
  • Alpha proteobacteria (Wolbachia pipientis)
  • Amoeba (Dictyostelium discoideum)
  • Archea (Pyrococcus horikoshii)
  • Barley (Hordeum vulgare)
  • Beta proteobacteria (Neisseria meningitidis)
  • Bread mold (Neurospora crassa)
  • Chromalveolata (Phytophthora infestans)
  • Common water flea (Daphnia pulex)
  • Corn (Zea mays)
  • E. coli (Escherichia coli)
  • Filamentous fungi (Aspergillus nidulans)
  • Firmicute Bacteria (Streptococcus pneumoniae)
  • Fission Yeast (Schizosaccharomyces pombe)
  • Green Algae (Chlamydomonas reinhardtii)
  • Honey Bee (Apis mellifera)
  • K. Lactis Yeast (Kluyveromyces lactis)
  • Loblloly Pine (Pinus taeda)
  • Malaria Parasite (Plasmodium falciparum)
  • Medicago Trunc (Medicago Truncatula)
  • Moss (Physcomitrella patens)
  • Orangutan (Pongo pygmaeus)
  • Pig (Sus scrofa)
  • Rainbow Trout (Oncorhynchus mykiss)
  • Rice (Oryza sativa)
  • Rice Blast Fungus (Magnaporthe grisea)
  • Schistosome Parasite (Schistosoma mansoni)
  • Sea Anemone (Nematostella vectensis)
  • Sea Vase (Ciona intestinalis)
  • Sea Urchin (Strongylocentrotus purpuratus)
  • Sorghum (Sorghum bicolor)
  • Soybean (Glycine max)
  • Stem Rust Fungus (Puccinia graminis)
  • Sugarcane (Saccharum officinarum)
  • Tomato (Lycopersicon esculentum)
  • Toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)
  • Wheat (Triticum aestivum)

Evolution for RHOT1 Gene

Gene Tree for RHOT1 (if available)
Gene Tree for RHOT1 (if available)
Evolutionary constrained regions (ECRs) for RHOT1: view image
Alliance of Genome Resources:
Additional Orthologs for RHOT1

Paralogs for RHOT1 Gene

(1) SIMAP similar genes for RHOT1 Gene using alignment to 9 proteins:

  • K7EP86_HUMAN Pseudogenes for RHOT1 Gene

genes like me logo Genes that share paralogs with RHOT1: view

Variants for RHOT1 Gene

Sequence variations, with clinical significance, from ClinVar and Humsavar, with links to dbSNP for RHOT1 Gene

SNP ID Clinical significance and condition Chr 17 pos Variation AA Info Type
rs995710160 Not Provided: not provided 32,211,267(+) G/A

dbSNP identifiers (rs#s) for variants without ClinVar clinical significance for RHOT1 Gene

All consequence types are included: molecular consequences (e.g. missense, synonymous), and location-based (e.g. intron, upstream).

Structural Variations from Database of Genomic Variants (DGV) for RHOT1 Gene

Variant ID Type Subtype PubMed ID
esv2669243 CNV deletion 23128226
esv2758684 CNV gain 17122850
esv3640377 CNV gain 21293372
esv3640378 CNV loss 21293372
esv3640379 CNV gain 21293372
nsv1057055 CNV gain 25217958
nsv1066680 CNV gain 25217958
nsv574692 CNV gain 21841781
nsv574693 CNV gain 21841781
nsv978412 CNV duplication 23825009

Variation tolerance for RHOT1 Gene

Residual Variation Intolerance Score: 27.3% of all genes are more intolerant (likely to be disease-causing)
Gene Damage Index Score: 1.03; 21.14% of all genes are more intolerant (likely to be disease-causing)

Additional Variant Information for RHOT1 Gene

SNPedia medical, phenotypic, and genealogical associations of SNPs for
Leiden Open Variation Database (LOVD)

SNP Genotyping and Copy Number Assays for research

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot for RHOT1 Gene

Disorders for RHOT1 Gene

MalaCards: The human disease database

(8) MalaCards diseases for RHOT1 Gene - From: COP

- elite association - COSMIC cancer census association via MalaCards
Search RHOT1 in MalaCards View complete list of genes associated with diseases

Additional Disease Information for RHOT1

Human Genome Epidemiology Navigator
ATLAS of Genetics and Cytogenetics in Oncology and Haematology
Open Targets Platform
Open Targets Platform
genes like me logo Genes that share disorders with RHOT1: view

No data available for UniProtKB/Swiss-Prot and Genatlas for RHOT1 Gene

Publications for RHOT1 Gene

  1. Atypical Rho GTPases have roles in mitochondrial homeostasis and apoptosis. (PMID: 12482879) Fransson A … Aspenström P (The Journal of biological chemistry 2003) 2 3 4
  2. Glucose regulates mitochondrial motility via Milton modification by O-GlcNAc transferase. (PMID: 24995978) Pekkurnaz G … Schwarz TL (Cell 2014) 3 4
  3. Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression. (PMID: 20877624) Hendrickson SL … O'Brien SJ (PloS one 2010) 3 40
  4. Personalized smoking cessation: interactions between nicotine dose, dependence and quit-success genotype score. (PMID: 20379614) Rose JE … Uhl GR (Molecular medicine (Cambridge, Mass.) 2010) 3 40
  5. HUMMR, a hypoxia- and HIF-1alpha-inducible protein, alters mitochondrial distribution and transport. (PMID: 19528298) Li Y … Rempe DA (The Journal of cell biology 2009) 3 4

Products for RHOT1 Gene

Sources for RHOT1 Gene