Gene:
progressive external ophthalmoplegia 1
Gene ID:
191096
Symbol:
PEO1
Species:
1000
Description:
Twinkle is a mitochondrial protein with structural similarity to the phage T7 primase/helicase (GP4) and other hexameric ring helicases. The twinkle protein colocalizes with mtDNA in mitochondrial nucleoids, and the protein's name derives from the unusual localization pattern reminiscent of twinkling stars (Spelbrink et al., 2001).[supplied by OMIM]