Protein: PEO1
Twinkle mtDNA helicase (EC 5.6.2.3) (Progressive external ophthalmoplegia 1 protein) (T7 gp4-like protein with intramitochondrial nucleoid localization) (T7-like mitochondrial DNA helicase) (Twinkle protein, mitochondrial)

Gene: PEO1, TWNK
Organism: Homo sapiens
Interactions: 138
UniProt ID: Q96RR1

Gene Ontology (top 5):
GO:0000262 - mitochondrial chromosome
GO:0002020 - protease binding
GO:0003678 - DNA helicase activity
GO:0003697 - single-stranded DNA binding
GO:0005524 - ATP binding

Download interactions: TXT   MITAB-2.5

Interaction Interactor Experiments Homologs Category Interaction
Score
Confidence

1397698

PRIPO
DNA-directed primase/polymerase protein

2

0

High-throughput

0.602

High

1123386

ICT1
Large ribosomal subunit protein mL62

2

0

High-throughput

0.585

High

171918

ZN263
Zinc finger protein 263

2

1

High-throughput

0.574

High

279871

CISY
Citrate synthase, mitochondrial

2

0

High-throughput

0.559

High

481367

HMGB1
High mobility group protein B1

1

2

High-throughput

0.536

High

1108063

SQSTM
Sequestosome-1

1

1

High-throughput

0.536

High

396761

ESR1
Estrogen receptor

2

1

High-throughput

0.521

High

572150

TRI27
Zinc finger protein RFP

1

0

High-throughput

0.513

High

1006641

SRPK2
SRSF protein kinase 2

1

0

High-throughput

0.513

High

1049694

1433F
14-3-3 protein eta

1

0

High-throughput

0.513

High

1115938

DYR1A
Dual specificity tyrosine-phosphorylation-regulated kinase 1A

1

0

High-throughput

0.513

High

1186502

CHD9
Chromodomain-helicase-DNA-binding protein 9

1

0

High-throughput

0.513

High

1387879

PABP5
Polyadenylate-binding protein 5

1

0

High-throughput

0.513

High

462583

HNRPC
Heterogeneous nuclear ribonucleoproteins C1/C2

1

1

High-throughput

0.489

High

1160048

SRSF7
Serine/arginine-rich splicing factor 7

1

1

High-throughput

0.489

High

282049

MPPB
Mitochondrial-processing peptidase subunit beta

1

1

High-throughput

0.479

High

287355

RF1M
Peptide chain release factor 1, mitochondrial

1

2

High-throughput

0.479

High

315910

RECQ4
ATP-dependent DNA helicase Q4

1

1

Small-scale

0.479

High

856349

IF2M
Translation initiation factor IF-2, mitochondrial

1

4

High-throughput

0.479

High

882832

EFTU
Elongation factor Tu, mitochondrial

1

3

High-throughput

0.479

High

1024150

TFAM
Transcription factor A, mitochondrial

1

1

High-throughput

0.479

High

1048651

SSBP
Single-stranded DNA-binding protein, mitochondrial

1

1

High-throughput

0.479

High

1083712

MPPA
Mitochondrial-processing peptidase subunit alpha

1

1

High-throughput

0.479

High

1117812

AUHM
Methylglutaconyl-CoA hydratase, mitochondrial

1

1

High-throughput

0.479

High

1207108

SYAM
Alanine--tRNA ligase, mitochondrial

1

1

High-throughput

0.479

High

1367076

ESR2
Estrogen receptor beta

1

1

High-throughput

0.479

High

1381108

RRF2M
Ribosome-releasing factor 2, mitochondrial

1

2

High-throughput

0.479

High

1401583

EFGM
Elongation factor G, mitochondrial

1

2

High-throughput

0.479

High

1401805

MTRFR
Mitochondrial translation release factor in rescue

1

2

High-throughput

0.479

High

1401821

RF1ML
Peptide chain release factor 1-like, mitochondrial

1

2

High-throughput

0.479

High

61595

VWA8
von Willebrand factor A domain-containing protein 8

1

0

High-throughput

0.458

High

66270

MET15
12S rRNA N(4)-cytidine methyltransferase METTL15

1

0

High-throughput

0.458

High

183711

RT12
Small ribosomal subunit protein uS12m

1

0

High-throughput

0.458

High

350962

TRUB2
Pseudouridylate synthase TRUB2, mitochondrial

1

0

High-throughput

0.458

High

471195

ODPA
Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial

1

0

High-throughput

0.458

High

519201

COX8A
Cytochrome c oxidase subunit 8A, mitochondrial

1

0

High-throughput

0.458

High

828905

MDHM
Malate dehydrogenase, mitochondrial

1

0

High-throughput

0.458

High

841888

LPPRC
Leucine-rich PPR motif-containing protein, mitochondrial

1

0

High-throughput

0.458

High

851996

EFTS
Elongation factor Ts, mitochondrial

1

0

High-throughput

0.458

High

1058222

C1QBP
Complement component 1 Q subcomponent-binding protein, mitochondrial

1

0

High-throughput

0.458

High

1162505

CLPP
ATP-dependent Clp protease proteolytic subunit, mitochondrial

1

0

High-throughput

0.458

High

1215350

DCAF8
DDB1- and CUL4-associated factor 8

1

0

High-throughput

0.458

High

1310329

HSC20
Iron-sulfur cluster co-chaperone protein HscB

1

0

High-throughput

0.458

High

1381680

FAKD4
FAST kinase domain-containing protein 4

1

0

High-throughput

0.458

High

1384851

CHCH1
Small ribosomal subunit protein mS37

1

0

High-throughput

0.458

High

1385999

RUSD4
Pseudouridylate synthase RPUSD4, mitochondrial

1

0

High-throughput

0.458

High

1387207

TIM14
Mitochondrial import inner membrane translocase subunit TIM14

1

0

High-throughput

0.458

High

1387751

FMT
Methionyl-tRNA formyltransferase, mitochondrial

1

0

High-throughput

0.458

High

1388243

RRFM
Ribosome-recycling factor, mitochondrial

1

0

High-throughput

0.458

High

1388372

MTEF3
Transcription termination factor 3, mitochondrial

1

0

High-throughput

0.458

High

1400799

TEFM
Transcription elongation factor, mitochondrial

1

0

High-throughput

0.458

High

1401796

IMP2L
Mitochondrial inner membrane protease subunit 2

1

0

High-throughput

0.458

High

1401799

TACO1
Translational activator of cytochrome c oxidase 1

1

0

High-throughput

0.458

High

1401800

HINT2
Adenosine 5'-monophosphoramidase HINT2

1

0

High-throughput

0.458

High

1401802

RT26
Small ribosomal subunit protein mS26

1

0

High-throughput

0.458

High

1401803

IF3M
Translation initiation factor IF-3, mitochondrial

1

0

High-throughput

0.458

High

1401806

MTG2
Mitochondrial ribosome-associated GTPase 2

1

0

High-throughput

0.458

High

1401808

MET17
Ribosome assembly protein METTL17, mitochondrial

1

0

High-throughput

0.458

High

1401809

ACAD9
Complex I assembly factor ACAD9, mitochondrial

1

0

High-throughput

0.458

High

1401813

NGRN
Neugrin

1

0

High-throughput

0.458

High

1401815

EXD2
Exonuclease 3'-5' domain-containing protein 2

1

0

High-throughput

0.458

High

1401819

MRES1
Mitochondrial transcription rescue factor 1

1

0

High-throughput

0.458

High

1401827

RM11
Large ribosomal subunit protein uL11m

1

0

High-throughput

0.458

High

465024

SP1
Transcription factor Sp1

1

1

High-throughput

0.457

High

1401801

KLF16
Krueppel-like factor 16

1

1

High-throughput

0.457

High

181927

SMAD9
Mothers against decapentaplegic homolog 9

1

1

High-throughput

0.439

High

482955

TPM1
Tropomyosin alpha-1 chain

1

1

High-throughput

0.437

High

1401797

RAD9A
Cell cycle checkpoint control protein RAD9A

1

1

High-throughput

0.437

High

1401826

ZBTB1
Zinc finger and BTB domain-containing protein 1

1

1

Small-scale

0.437

High

414452

P53
Cellular tumor antigen p53

1

0

Small-scale

0.426

High

873489

PP2BC
Serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform

1

0

High-throughput

0.426

High

104279

F5H4F1
Cell cycle checkpoint control protein RAD9A

1

1

High-throughput

0.407

High

116470

H7BYY1
Tropomyosin 1

1

1

High-throughput

0.407

High

1250508

RBP10
Ran-binding protein 10

1

2

High-throughput

0.407

High

1272727

MAEA
E3 ubiquitin-protein transferase MAEA

1

3

High-throughput

0.407

High

1401795

RANB9
Ran-binding protein 9

1

2

High-throughput

0.407

High

1401816

GID8
Glucose-induced degradation protein 8 homolog

1

3

High-throughput

0.407

High

325061

HEXI1
Protein HEXIM1

4

0

High-throughput

0.362

High

794594

RBMX
RNA-binding motif protein, X chromosome

3

0

High-throughput

0.347

High

1111848

TRA2A
Transformer-2 protein homolog alpha

3

0

High-throughput

0.347

High

868378

CDX1
Homeobox protein CDX-1

2

0

High-throughput

0.328

High

905057

MECP2
Methyl-CpG-binding protein 2

2

0

High-throughput

0.328

High

1133386

ZN169
Zinc finger protein 169

2

0

High-throughput

0.328

High

1290665

ZCH18
Zinc finger CCCH domain-containing protein 18

2

0

High-throughput

0.328

High

1063846

LG3BP
Galectin-3-binding protein

2

0

High-throughput

0.319

High

1235498

IFIX
Pyrin and HIN domain-containing protein 1

2

0

High-throughput

0.318

High

872654

SOX2
Transcription factor SOX-2

2

0

High-throughput

0.310

High

1401810

AKTIP
AKT-interacting protein

1

0

High-throughput

0.282

High

49538

SHSA3
Protein shisa-3 homolog

1

0

High-throughput

0.279

Low

67434

GOG8A
Golgin subfamily A member 8A

1

0

High-throughput

0.279

Low

338937

CBX6
Chromobox protein homolog 6

1

0

High-throughput

0.279

Low

483192

CLCA
Clathrin light chain A

1

0

High-throughput

0.279

Low

654131

XPA
DNA repair protein complementing XP-A cells

1

0

High-throughput

0.279

Low

686360

RL13
Large ribosomal subunit protein eL13

1

0

High-throughput

0.279

Low

698350

MAP4
Microtubule-associated protein 4

1

0

High-throughput

0.279

Low

704480

MZF1
Myeloid zinc finger 1

1

0

High-throughput

0.279

Low

785383

RL4
Large ribosomal subunit protein uL4

1

0

High-throughput

0.279

Low

953835

C1QT8
Complement C1q tumor necrosis factor-related protein 8

1

0

High-throughput

0.279

Low

1099487

SRSF6
Serine/arginine-rich splicing factor 6

1

0

High-throughput

0.279

Low

1124528

MAP7
Ensconsin

1

0

High-throughput

0.279

Low

1229850

CYTSA
Cytospin-A

1

0

High-throughput

0.279

Low

1281450

ZN467
Zinc finger protein 467

1

0

High-throughput

0.279

Low

1286533

PHLB2
Pleckstrin homology-like domain family B member 2

1

0

High-throughput

0.279

Low

1308445

LYSM2
LysM and putative peptidoglycan-binding domain-containing protein 2

1

0

High-throughput

0.279

Low

1359534

CKAP2
Cytoskeleton-associated protein 2

1

0

High-throughput

0.279

Low

1361863

BACD1
BTB/POZ domain-containing adapter for CUL3-mediated RhoA degradation protein 1

1

0

High-throughput

0.279

Low

1385556

ZN524
Zinc finger protein 524

1

0

High-throughput

0.279

Low

1401798

ARP5L
Actin-related protein 2/3 complex subunit 5-like protein

1

0

High-throughput

0.279

Low

1401804

FOXP1
Forkhead box protein P1

1

0

High-throughput

0.279

Low

1401807

ACTL8
Actin-like protein 8

1

0

High-throughput

0.279

Low

1401811

NCOA5
Nuclear receptor coactivator 5

1

0

High-throughput

0.279

Low

1401812

E41L5
Band 4.1-like protein 5

1

0

High-throughput

0.279

Low

1401814

ABC3C
DNA dC->dU-editing enzyme APOBEC-3C

1

0

High-throughput

0.279

Low

1401817

F120A
Constitutive coactivator of PPAR-gamma-like protein 1

1

0

High-throughput

0.279

Low

1401820

B4GT7
Beta-1,4-galactosyltransferase 7

1

0

High-throughput

0.279

Low

1401822

PNMA2
Paraneoplastic antigen Ma2

1

0

High-throughput

0.279

Low

1401825

CLC11
C-type lectin domain family 11 member A

1

0

High-throughput

0.279

Low

508477

UBC
Polyubiquitin-C [Cleaved into: Ubiquitin]

1

0

High-throughput

0.261

Low

725307

TLX1
T-cell leukemia homeobox protein 1

1

0

High-throughput

0.261

Low

1052904

HME1
Homeobox protein engrailed-1

1

0

High-throughput

0.261

Low

129350

MEN1
Menin

1

0

High-throughput

0.249

Low

223268

PRC1
Protein regulator of cytokinesis 1

1

0

High-throughput

0.249

Low

350116

SNAI1
Zinc finger protein SNAI1

1

0

High-throughput

0.249

Low

630002

BTF3
Transcription factor BTF3

1

0

High-throughput

0.249

Low

717580

PDIA3
Protein disulfide-isomerase A3

1

0

High-throughput

0.249

Low

765817

BMI1
Polycomb complex protein BMI-1

1

0

High-throughput

0.249

Low

1015696

RHOG
Rho-related GTP-binding protein RhoG

1

0

High-throughput

0.249

Low

1351950

RBCC1
RB1-inducible coiled-coil protein 1

1

0

High-throughput

0.249

Low

1352700

DOT1L
Histone-lysine N-methyltransferase, H3 lysine-79 specific

1

0

High-throughput

0.249

Low

1401824

HECD1
E3 ubiquitin-protein ligase HECTD1

1

0

High-throughput

0.249

Low

964425

SUMO2
Small ubiquitin-related modifier 2

1

0

High-throughput

0.238

Low

977150

YPEL5
Protein yippee-like 5

1

0

High-throughput

0.232

Low

1307902

ARMC8
Armadillo repeat-containing protein 8

1

0

High-throughput

0.232

Low

1309301

GID4
Glucose-induced degradation protein 4 homolog

1

0

High-throughput

0.232

Low

1326043

RYBP
RING1 and YY1-binding protein

1

0

High-throughput

0.232

Low

1353099

ARH40
Rho guanine nucleotide exchange factor 40

1

0

High-throughput

0.232

Low

1401818

GRHL1
Grainyhead-like protein 1 homolog

1

0

High-throughput

0.232

Low

1401823

MKLN1
Muskelin

1

0

High-throughput

0.232

Low


Service provided by Combinatics, Japan

Last updated: 11Aug2025