Mitochondrial diseases are a group of genetic disorders that are characterized by defects in
oxidative phosphorylation and caused by mutations in genes in the nuclear DNA (nDNA) …

Lipoic acid (LA) is an essential cofactor required for the activity of five multienzymatic
complexes that play a central role in the mitochondrial energy metabolism: four 2-oxoacid …

Iron-sulfur (Fe-S) clusters are essential for many cellular processes, ranging from aerobic
respiration, metabolite biosynthesis, ribosome assembly and DNA repair. Mutations in NFU1 …

Iron-sulfur metabolism is essential for cellular function and is a key process in mitochondria.
In this review, we focus on the structure and assembly of mitochondrial iron-sulfur clusters …

Abstract Iron–sulfur (Fe–S) clusters are inorganic cofactors that are fundamental to several
biological processes in all three kingdoms of life. In most organisms, Fe–S clusters are …

Background Voltage-gated potassium channels are highly diverse proteins representing the
most complex class of voltage-gated ion channels from structural and functional …

Mutations in NFU1 were recently identified in patients with fatal encephalopathy. NFU1 is an
iron–sulfur cluster protein necessary for the activity of the mitochondrial respiratory chain …

Nonketotic hyperglycinemia (NKH) is caused by defective glycine cleavage enzyme activity.
Classic NKH is caused by mutations in protein coding genes (GLDC and AMT). Disorders of …

铁硫簇蛋白是一类重要的线粒体功能蛋白, 在细胞能量代谢, 电子传递, 底物结合与激活,
铁/硫存储, 酶促反应, 基因表达调控等诸多过程中均发挥了关键作用. 铁硫簇蛋白质组装及转运 …

The iron-sulfur (Fe-S) cluster (ISC) biogenesis pathway is indispensable for many
fundamental biological processes and pathogenic variations in genes encoding several …