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Crystal structures of the disease-causing D444V mutant and the relevant wild type human dihydrolipoamide dehydrogenase.

Free radical biology & medicine - 20 Aug 2018

Szabo Eszter, Mizsei Reka, Wilk Piotr, Zambo Zsofia, Torocsik Beata, Weiss Manfred S, Adam-Vizi Vera, Ambrus Attila

Abstract excerpt

We report the crystal structures of the human (dihydro)lipoamide dehydrogenase (hLADH, hE3) and its disease-causing homodimer interface mutant D444V-hE3 at 2.27 and 1.84 Å resolution, respectively. The wild type structure is a unique uncomplexed, unliganded hE3 structure with the true canonical sequence. Based on the structural information a novel molecular pathomechanism is proposed for the impaired catalytic...

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PMID: 29908278
DOI: 10.1016/j.freeradbiomed.2018.06.008

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