basic FUNCTION
| involved in cell proliferation or cell-cycle regulation |
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play essential roles in the production of [Fe-S] centers for the normal maturation of lipoate-containing 2-oxoacid dehydrogenases, and for the assembly of the respiratory chain complexes |
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NFU1- (and BOLA3-) dependent assembled clusters are directed to complex I and lipoate synthesis |
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with GLRX, help regulate iron metabolism |
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BOLA1 and BOLA3 are specific mitochondrial ISC assembly factors that facilitate [4Fe-4S] cluster insertion into a subset of mitochondrial proteins such as lipoate synthase and succinate dehydrogenase |
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acts as a crucial lynchpin connecting Fe-S-dependent oxidative respiration and glycine homeostasis with endothelial metabolic reprogramming critical to PH pathogenesis |
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BOLA3 can form a functional homodimer capable of engaging in Fe-S cluster transfer |
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BOLA3 is associated with adipose tissue oxidative capacity and it plays an indispensable role in beige adipocyte thermogenesis via maintaining mitochondrial homeostasis and adrenergic signaling-induced lipolysis |