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• W3010167460 endingPage "426" @default.
• W3010167460 startingPage "411" @default.
• W3010167460 abstract "Iron-sulfur (Fe-S) proteins have critical roles in essential metabolic pathways ranging from DNA and RNA metabolism to mitochondrial function, and regulation of cell growth and division. Mammalian cells likely contain many more Fe-S proteins than have been identified to date, due to loss of fragile iron–sulfur clusters (ISCs) during purification. Structural determinations of the architecture of the initial ISC biogenesis complex, comprising NFS1, ISD11, ACP, ISCU, and FXN, have shed light on the interactions that govern de novo ISC assembly. In mammalian cells, a full complement of initial ISC biogenesis factors localizes to the cytosol, where it initiates de novo ISC assembly. Further biochemical and clinical investigations of ISC-associated disorders and their phenotypes will elucidate the distinct pathways that assemble and deliver ISCs to the numerous enzymes that require them for function. Iron–sulfur (Fe-S) clusters (ISCs) are ubiquitous cofactors essential to numerous fundamental cellular processes. Assembly of ISCs and their insertion into apoproteins involves the function of complex cellular machineries that operate in parallel in the mitochondrial and cytosolic/nuclear compartments of mammalian cells. The spectrum of diseases caused by inherited defects in genes that encode the Fe-S assembly proteins has recently expanded to include multiple rare human diseases, which manifest distinctive combinations and severities of global and tissue-specific impairments. In this review, we provide an overview of our understanding of ISC biogenesis in mammalian cells, discuss recent work that has shed light on the molecular interactions that govern ISC assembly, and focus on human diseases caused by failures of the biogenesis pathway. Iron–sulfur (Fe-S) clusters (ISCs) are ubiquitous cofactors essential to numerous fundamental cellular processes. Assembly of ISCs and their insertion into apoproteins involves the function of complex cellular machineries that operate in parallel in the mitochondrial and cytosolic/nuclear compartments of mammalian cells. The spectrum of diseases caused by inherited defects in genes that encode the Fe-S assembly proteins has recently expanded to include multiple rare human diseases, which manifest distinctive combinations and severities of global and tissue-specific impairments. In this review, we provide an overview of our understanding of ISC biogenesis in mammalian cells, discuss recent work that has shed light on the molecular interactions that govern ISC assembly, and focus on human diseases caused by failures of the biogenesis pathway. an enzyme that catalyzes one of the early steps in Fe-S cluster biogenesis, namely the abstraction of the inorganic sulfur, required to build the cluster, from cysteine. The gene is named NFS1 in humans, Nfs1 in yeast, and IscS in prokaryotes. In humans, the same NFS1 nuclear gene encodes two isoforms that localize either to the cytosol/nucleus or to mitochondria, as a result of initiation of translation from two alternative initiation codons on the mRNA. the pathway that assembles [2Fe-2S] clusters starting from inorganic sulfur and iron through the coordinated action of several multiprotein complexes. [2Fe-2S] clusters provide the building blocks to assemble [4Fe-4S] clusters and clusters with more complex stoichiometries. a member of the HSP70 family that works in concert with a cochaperone to facilitate Fe-S cluster transfer from the scaffold to the recipient protein utilizing ATP to drive the conformational changes required for cluster transfer and ligation by the Fe-S recipient. The chaperone involved in facilitating cluster transfer downstream of the main scaffold ISCU is encoded by the gene named HSPA9 in humans, Ssq1 in yeast, and HscA in prokaryotes. a member of the Hsp40 family that works in concert with a chaperone and activates the ATPase activity of the cognate chaperone. The cochaperone also specifically recognizes Fe-S clients and the main scaffold ISCU. The cochaperone involved in facilitating cluster transfer downstream of the main scaffold ISCU is encoded by a gene named HSC20 (or HSCB) in humans, Jac1 in yeast, and HscB in prokaryotes. proteins that require Fe-S cofactors to be functional and that are recognized by components of the Fe-S biogenesis machinery to acquire their clusters. a component of the Fe-S biogenesis pathway upon which a [2Fe-2S] cluster is initially assembled starting from inorganic sulfur and iron. The gene encoding the main scaffold is named ISCU in humans, Isu (1 and 2) in yeast, and IscU in prokaryotes. In humans, the same ISCU nuclear gene encodes two alternative splicing isoforms that localize to either the cytosol or the mitochondrial matrix. a component of the Fe-S biogenesis pathway that is able to accept Fe-S clusters from a scaffold protein and is able to donate its cluster to recipient proteins." @default.
• W3010167460 created "2020-03-13" @default.
• W3010167460 creator A5017226392 @default.
• W3010167460 creator A5080924706 @default.
• W3010167460 date "2020-05-01" @default.
• W3010167460 modified "2023-10-14" @default.
• W3010167460 title "Outlining the Complex Pathway of Mammalian Fe-S Cluster Biogenesis" @default.
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