Ontology highlight
ABSTRACT:
SUBMITTER: Fox NG
PROVIDER: S-EPMC6525205 | biostudies-literature | 2019 May
REPOSITORIES: biostudies-literature
Fox Nicholas G NG Yu Xiaodi X Feng Xidong X Bailey Henry J HJ Martelli Alain A Nabhan Joseph F JF Strain-Damerell Claire C Bulawa Christine C Yue Wyatt W WW Han Seungil S
Nature communications 20190517 1
The core machinery for de novo biosynthesis of iron-sulfur clusters (ISC), located in the mitochondria matrix, is a five-protein complex containing the cysteine desulfurase NFS1 that is activated by frataxin (FXN), scaffold protein ISCU, accessory protein ISD11, and acyl-carrier protein ACP. Deficiency in FXN leads to the loss-of-function neurodegenerative disorder Friedreich's ataxia (FRDA). Here the 3.2 Å resolution cryo-electron microscopy structure of the FXN-bound active human complex, cont ...[more]