[HTML][HTML] TWINKLE has 5′→ 3′ DNA helicase activity and is specifically stimulated by mitochondrial single-stranded DNA-binding protein

JA Korhonen, M Gaspari, M Falkenberg - Journal of Biological Chemistry, 2003 - ASBMB
Mutations in TWINKLE cause autosomal dominant progressive external ophthalmoplegia, a
human disorder associated with multiple deletions in the mitochondrial DNA. TWINKLE
displays primary sequence similarity to the phage T7 gene 4 primase-helicase, but no
specific enzyme activity has been assigned to the protein. We have purified recombinant
TWINKLE to near homogeneity and demonstrate here that TWINKLE is a DNA helicase with
5′ to 3′ directionality and distinct substrate requirements. The protein needs a stretch of …