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Beatrice Bargagna on Muck Rack

Beatrice Bargagna

As seen in: MDPI
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Understanding the Molecular Basis of the Multiple Mitochondrial Dysfunctions Syndrome 2: The Disease-Causing His96Arg Mutation of BOLA3

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By Lucia Banci, Francesca Camponeschi, Beatrice Bargagna
| MDPI
3. Discussion Human BOLA3 is an essential ISC assembly factor, acting in the late stages of the Fe-S biosynthesis pathway, for the maturation of specific [4Fe-4S]-binding proteins [17,18,34]. BOLA3 interacts with its protein partner GLRX5 forming a [2Fe-2S] cluster-bridged hetero-dimeric complex, that can transfer, in vitro, its [2Fe-2S] cluster to apo NFU1, to form [4Fe-4S]2+-NFU1 in the presence of a reductant [21].
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