The hexameric structure of the human mitochondrial replicative helicase Twinkle

  title={The hexameric structure of the human mitochondrial replicative helicase Twinkle},
  author={Pablo Fern{\'a}ndez-Mill{\'a}n and Melisa L{\'a}zaro and Şirin Cansız-Arda and Joachim M Gerhold and Nina K Rajala and Claus-A Schmitz and Cristina Silva-Espi{\~n}a and David Leon Gil and Pau Bernad{\'o} and Mikel Valle and Johannes N. Spelbrink and Mar{\'i}a del Mar Sola},
  booktitle={Nucleic acids research},
The mitochondrial replicative helicase Twinkle is involved in strand separation at the replication fork of mitochondrial DNA (mtDNA). Twinkle malfunction is associated with rare diseases that include late onset mitochondrial myopathies, neuromuscular disorders and fatal infantile mtDNA depletion syndrome. We examined its 3D structure by electron microscopy (EM) and small angle X-ray scattering (SAXS) and built the corresponding atomic models, which gave insight into the first molecular… CONTINUE READING
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