Question

The frataxin gene is essential for mitochondrial iron regulation. Mutation of this gene (Friedreich ataxia) results in iron build up with free radical damage. What is the role of the frataxin gene in mitochondrial iron regulation?

1) It helps in the transport of iron into the mitochondria
2) It helps in the export of iron out of the mitochondria
3) It helps in the storage of iron within the mitochondria
4) It helps in the breakdown of iron within the mitochondria

Answer 1

The frataxin gene is responsible for helping in the storage of iron within the mitochondria, playing a vital role in mitochondrial iron regulation. Mutations in this gene can lead to Friedreich ataxia, characterized by iron buildup and free radical damage within the mitochondria.

Step-by-step explanation:

The frataxin gene plays a crucial role in mitochondrial iron regulation. It is particularly important for the assembly of iron-sulfur clusters, which are essential for mitochondrial enzyme function. Mutations in the frataxin gene lead to a condition known as Friedreich ataxia, where iron accumulates within the mitochondria, causing oxidative stress due to the production of free radicals. This buildup of iron and subsequent damage is a result of the disrupted function of the frataxin protein, which, when operating normally, helps in the storage of iron within the mitochondria. Therefore, the correct role of the frataxin gene in mitochondrial iron regulation is to help in the storage of iron within the mitochondria.