| Type: | Family | Name: | Twinkle-like protein |
| Description: | Twinkle protein (T7-like mitochondrial DNA helicase) is a DNA helicase needed for mitochondrial DNA replication []. It catalyses the ATP-dependent unwinding of a DNA duplex with 5' to 3' directionality, and it needs a single-stranded 5'-DNA and a short 3'-tail to initiate unwinding []. In humans, mutations in TWINKLE cause autosomal dominant progressive external ophthalmoplegia, a human disorder associated with multiple deletions in the mitochondrial DNA []. Arabidopsis encodes a homologue to phage T7 gp4/Twinkle proteins that has both DNA primase and DNA helicase activities []. | Short Name: | Twinkle-like |
