Identification
HMDB Protein ID CDBP05428
Secondary Accession Numbers Not Available
Name Twinkle protein, mitochondrial
Description Not Available
Synonyms
  1. Progressive external ophthalmoplegia 1 protein
  2. T7 gp4-like protein with intramitochondrial nucleoid localization
  3. T7-like mitochondrial DNA helicase
Gene Name PEO1
Protein Type Transcription Factor
Biological Properties
General Function Not Available
Specific Function Involved in mitochondrial DNA (mtDNA) metabolism. Could function as an adenine nucleotide-dependent DNA helicase. Function inferred to be critical for lifetime maintenance of mtDNA integrity. In vitro, forms in combination with POLG, a processive replication machinery, which can use double-stranded DNA (dsDNA) as template to synthesize single-stranded DNA (ssDNA) molecules. May be a key regulator of mtDNA copy number in mammals.
GO Classification
Biological Process
mitochondrial DNA replication
protein homooligomerization
DNA unwinding involved in replication
protein hexamerization
transcription from mitochondrial promoter
cell death
Cellular Component
mitochondrial nucleoid
Molecular Function
single-stranded DNA binding
5'-3' DNA helicase activity
ATP binding
Cellular Location Not Available
Pathways
Gene Properties
Chromosome Location 10
Locus 10q24
SNPs Not Available
Gene Sequence Not Available
Protein Properties
Number of Residues Not Available
Molecular Weight 66014.925
Theoretical pI 8.113
Pfam Domain Function Not Available
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>gi|255304946|ref|NP_001157284.1| twinkle protein, mitochondrial isoform B [Homo sapiens]
MWVLLRSGYPLRILLPLRGEWMGRRGLPRNLAPGPPRRRYRKETLQALDMPVLPVTATEI
RQYLRGHGIP
GenBank ID Protein Not Available
UniProtKB/Swiss-Prot ID Q96RR1
UniProtKB/Swiss-Prot Entry Name Not Available
PDB IDs Not Available
GenBank Gene ID Not Available
GeneCard ID Not Available
GenAtlas ID Not Available
HGNC ID HGNC:1160
References
General References Not Available