Iron is required for a barrage of essential biochemical functions in virtually every species of life. Perturbation of the availability or utilization of iron in these functions or disruption of other components along iron-requiring pathways can not only lead to cellular/organismal insufficiency of respective biochemical end-products but also result in a broad derangement of iron homeostasis. This is largely because of the elaborate regulatory mechanisms that connect cellular iron utilization with uptake and distribution. Such mechanisms are necessitated by the 'double-edged' nature of the metal, whose very property as a useful biological catalyst also makes it able to generate highly toxic compounds. Since the majority of iron is dispatched onto a functional course by mitochondria-localized pathways, these organelles are in an ideal position within the cellular iron anabolic pathways to be a central site for regulation of iron homeostasis. The goal of this article is to provide an overview of how mitochondria acquire and use iron and examine the ramifications of disturbances in these processes on overall cellular iron homeostasis.
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