Gene Information
Gene NameTWNK
OrganismHomo sapiens (Human)
Gene Length684
Protein NamesTwinkle mtDNA helicase (EC 3.6.4.12) (Progressive external ophthalmoplegia 1 protein) (T7 gp4-like protein with intramitochondrial nucleoid localization) (T7-like mitochondrial DNA helicase) (Twinkle protein, mitochondrial)
Target NameNA
Target TypeNA
Gene Age987 ( ENSG00000107815 )
Evolutionary StageEukaryota
AlphaFoldDBQ96RR1
Gene CardTWNK
Uniprot IDQ96RR1
PfamNA
In ASDno
Allosteric Predictionno (-0.50376645)
Ortholog in AnimalsAlpaca_ TWNK ( ortholog_one2one )
Dog_ TWNK ( ortholog_one2one )
GuineaPig_ TWNK ( ortholog_one2one )
Macaque_ TWNK ( ortholog_one2one )
Mouse_ Twnk ( ortholog_one2one )
Pig_ TWNK ( ortholog_one2one )
Rat_ Twnk ( ortholog_one2one )
Rabbit_ TWNK ( ortholog_one2one )
OhnologsNA
ParalogsNA
TissueNA
Tissue SpecificityHigh relative levels in skeletal muscle, testis and pancreas. Lower levels of expression in the heart, brain, placenta, lung, liver, kidney, spleen, thymus, prostate, ovary, small intestine, colon and leukocytes. Expression is coregulated with MRPL43. {ECO:0000269|PubMed:11431692, ECO:0000269|PubMed:15509589}.
Pharmacological Animal ModelsNA
Gene Ontology
(biological process)
Gene Ontology
(cellular component)
Gene Ontology
(molecular function)
PDB 7T8B  (EM  3.80A  7T8C  (EM  4.50A 
DBSNP rs137852956  rs1159929268  rs111033575  rs80356542  rs80356543  rs28937887  rs62626271  rs111033576  rs758026634  rs111033573  rs1554887075  rs17113613  rs111033579  rs863223920  rs1554887097  rs111033577  rs556445621  rs11542126  rs672601361  rs386834145  rs80356544  rs1554887213  rs111033574  rs11542127  rs111033572  rs1085307937  rs369588002  rs80356540  rs672601360  rs62626293 
Subcellular location [CC]Mitochondrion matrix, mitochondrion nucleoid {ECO:0000269|PubMed:11431692, ECO:0000269|PubMed:18971204}. Note=Colocalizes with mtDNA in mitochondrial nucleoids, a nucleoproteins complex consisting of a number of copies of proteins associated with mtDNA, probably involved in mtDNA maintenance and expression. {ECO:0000269|PubMed:11431692}.
Mouse PG classificationLPG
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