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Reconstitution, characterization, and [2Fe-2S] cluster exchange reactivity of a holo human BOLA3 homodimer.

J Biol Inorg Chem. 2019;

Wachnowsky C,, Rao B,, Sen S, Fries B, Howard CJ,, Ottesen JJ,, Cowan JA,.

Products/Services Used	Details	Operation
Gene Synthesis	… trafficking Materials and methods Materials The gene for human BOLA3 in the pET28b(+) vector (UniProt: Q53S33; residues 25–107 [18], with the mitochondrial targeting sequence excluded) was purchased from GenScript …	Get A Quote

摘要

A new class of mitochondrial disease has been identified and characterized as Multiple Mitochondrial Dysfunctions Syndrome (MMDS). Four different forms of the disease have each been attributed to point mutations in proteins involved in iron-sulfur (Fe-S) biosynthesis; in particular, MMDS2 has been associated with the protein BOLA3. To date, this protein has been characterized in vitro concerning its ability to form heterodimeric complexes with two putative Fe-S cluster-binding partners: GLRX5 and NFU. However, BOLA3 has yet to be characterized in its own discrete holo form. Herein we describe procedures to isolate and characterize the human holo BOLA3 protein in terms of Fe-S cluster binding and trafficking and... More