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Details on Person UniProt:Q8IXI2-4 RHOT1

Class:IdReferenceIsoform:194895
_chainChangeLogchain:1-618 added on Fri February 6 2015
_displayNameUniProt:Q8IXI2-4 RHOT1
_timestamp2025-08-15 22:01:54
chainchain:1-618
checksumFE47AC3A4AF6F6C2
commentFUNCTION Atypical mitochondrial nucleoside-triphosphatase (NTPase) involved in mitochondrial trafficking (PubMed:12482879, PubMed:16630562, PubMed:22396657, PubMed:30513825). 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). Can hydrolyze GTP, ATP and UTP (PubMed:30513825).CATALYTIC ACTIVITY GTP + H2O = GDP + phosphate + H(+)CATALYTIC ACTIVITY ATP + H2O = ADP + phosphate + H(+)CATALYTIC ACTIVITY UTP + H2O = UDP + phosphate + H(+)SUBUNIT Homodimer (PubMed:27605430, PubMed:33132189). 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).INTERACTION Colocalizes with MGARP and RHOT2 at the mitochondria.ALTERNATIVE PRODUCTS Ubiquitously expressed. Expressed at high level in heart and skeletal muscle.DOMAIN The Miro 2 domain is necessary for efficient ubiquitination by PRKN.PTM Ubiquitinated by PRKN during mitophagy, leading to its degradation and enhancement of mitophagy (PubMed:22396657, PubMed:24896179, PubMed:27605430). Deubiquitinated by USP30 (PubMed:24896179).PTM Acetylation on Lys-92 decreases sensitivity of mitochondrial transport to elevated Ca(2+) levels, increases mitochondrial transport and promotes axon growth. Deacetylated by HDAC6 which blocks mitochondrial transport and mediates axon growth inhibition.SIMILARITY Belongs to the mitochondrial Rho GTPase family.SEQUENCE CAUTION Extended N-terminus.SEQUENCE CAUTION Extended N-terminus.SEQUENCE CAUTION Truncated N-terminus.SEQUENCE CAUTION Truncated N-terminus.
created[InstanceEdit:194867] Gopinathrao, G, 2007-03-30 18:18:39
descriptionrecommendedName: Mitochondrial Rho GTPase 1 shortName: MIRO-1 shortName: hMiro-1 ecNumber evidence="15"3.6.5.- alternativeName: Rac-GTP-binding protein-like protein alternativeName: Ras homolog gene family member T1
geneNameRHOT1
ARHT1
identifierQ8IXI2
isoformParent
isSequenceChangedFALSE
keyword3D-structure
Acetylation
Alternative splicing
Calcium
GTP-binding
Hydrolase
Isopeptide bond
Membrane
Metal-binding
Mitochondrion
Mitochondrion outer membrane
Nucleotide-binding
Proteomics identification
Reference proteome
Repeat
Transmembrane
Transmembrane helix
Ubl conjugation
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9948485] Weiser, Joel, 2025-05-21
[InstanceEdit:9963647] Weiser, Joel, 2025-08-15
nameRHOT1
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8996107] ENSEMBL:ENSG00000126858 RHOT1 [Homo sapiens]
secondaryIdentifierMIRO1_HUMAN
A4FVB6
A6NFV0
B4DG48
J9JIH9
Q6NUR3
Q6P9F8
Q6PJG1
Q6YMW8
Q86UB0
Q8IW28
Q8IXJ7
Q9H067
Q9H9N8
Q9NUZ2
sequenceLength618
species[Species:48887] Homo sapiens
variantIdentifierQ8IXI2-4
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