Mechanism of frataxin “bypass” in human iron–sulfur cluster biosynthesis with implications for Friedreich's ataxia
D Das, S Patra, J Bridwell-Rabb… - Journal of Biological …, 2019 - ASBMB
In humans, mitochondrial iron–sulfur cluster biosynthesis is an essential biochemical
process mediated by the assembly complex consisting of cysteine desulfurase (NFS1), LYR
protein (ISD11), acyl-carrier protein (ACP), and the iron–sulfur cluster assembly scaffold
protein (ISCU2). The protein frataxin (FXN) is an allosteric activator that binds the assembly
complex and stimulates the cysteine desulfurase and iron–sulfur cluster assembly activities.
FXN depletion causes loss of activity of iron–sulfur-dependent enzymes and the …
process mediated by the assembly complex consisting of cysteine desulfurase (NFS1), LYR
protein (ISD11), acyl-carrier protein (ACP), and the iron–sulfur cluster assembly scaffold
protein (ISCU2). The protein frataxin (FXN) is an allosteric activator that binds the assembly
complex and stimulates the cysteine desulfurase and iron–sulfur cluster assembly activities.
FXN depletion causes loss of activity of iron–sulfur-dependent enzymes and the …
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