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- Title
Mammalian iron sulfur cluster biogenesis and human diseases.
- Authors
Maio, Nunziata; Rouault, Tracey A.
- Abstract
The fact that an increasing number of newly recognized rare diseases results from a compromise in the Fe-S biogenesis pathway offers the prospect that better understanding of the complexity of Fe-S cluster biogenesis will lead to more effective diagnostics and treatments for a related group of human diseases. Keywords: chaperones; electron transfer in proteins; human molecular disease; iron; mitochondria; oxidants EN chaperones electron transfer in proteins human molecular disease iron mitochondria oxidants 705 714 10 07/01/22 20220701 NES 220701 Abbreviations Fe-S iron-sulfur FRDA Friedreich ataxia FXN frataxin LIAS Lipoic Acid Synthase MMDs Multiple mitochondrial dysfunctions syndromes OMIM Online Mendelian Inheritance in Man INTRODUCTION Fe-S cluster biogenesis is a multistep process that involves a complex sequence of catalyzed protein-protein interactions and coupled conformational changes between the components of several dedicated multimeric complexes. Mouse knock-out of IOP1 protein reveals its essential role in mammalian cytosolic iron-sulfur protein biogenesis. Intensive studies of the assembly process have clarified key points in the biogenesis of Fe-S proteins (see the Chapter "Mammalian iron sulfur cluster biogenesis; from assembly to delivery to recipient proteins with a focus on novel targets of the chaperone and co-chaperone proteins").
- Subjects
TRANSCRIPTION factors; IRON clusters; RNA splicing; MITOCHONDRIA formation; ACYL carrier protein; FRIEDREICH'S ataxia; MOLECULAR clusters; MITOCHONDRIAL proteins
- Publication
IUBMB Life, 2022, Vol 74, Issue 7, p705
- ISSN
1521-6543
- Publication type
Article
- DOI
10.1002/iub.2597