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NFU1 encodes a protein that is localized to mitochondria and plays a critical role in iron-sulfur cluster biogenesis. Zusätzlich bieten wir Ihnen NFU1 Iron-Sulfur Cluster Scaffold Homolog (S. Cerevisiae) Antikörper (5) und viele weitere Produktgruppen zu diesem Protein an.
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a direct role for human Nfu in the process of [2Fe-2S] cluster trafficking and delivery
Analysis of protein stability and oligomeric state demonstrates that the Gly208Cys mutant increases the propensity to dimerize and perturbs the secondary structure composition. These changes appear to underlie the severely decreased ability of mutant NFU1 to accept an Fe/S cluster from physiologically relevant sources.
Study used NMR spectroscopy and small-angle X-ray scattering data to determine the 3D structure of human mitochondrial NFU1 in its apo (zeige C9orf3 Proteine)- and iron-sulfur cluster-containing holo-form. Apo (zeige C9orf3 Proteine)- NFU1 is monomeric, whereas holo-NFU1 consists of a trimer of three [4Fe-4S] cluster-linked dimers.
a leaky splicing regulation due to a splice site mutation (c.545+5G>A) that produces small amounts of wild type NFU1 mRNA that might result in enough protein to partially lipoylate and restore the activity of lipoic acid-dependent enzymes and the assembly and activity of complex I.
A new NFU1 mutation is reported in a patient presenting with a milder phenotype characterized by a later onset, a slowly progressive spastic paraparesis with relapsing-remitting episodes, mild cognitive impairment and a long survival.
This study provides new insights into the molecular bases of NFU1 disease.
A fatal mitochondrial disease is associated with defective NFU1 function in the maturation of a subset of mitochondrial Fe-S proteins
Mutations in iron-sulfur cluster scaffold genes NFU1 and BOLA3 (zeige BOLA3 Proteine) cause a fatal deficiency of multiple respiratory chain and 2-oxoacid dehydrogenase enzymes [case report]
Analyses of genomic DNA, transcripts, and translation products indicate that alternative splicing of a common pre-mRNA results in synthesis of two Nfu isoforms with distinct subcellular localizations.
Laforin (zeige EPM2A Proteine) interacts with HIRIP5.
This gene encodes a protein that is localized to mitochondria and plays a critical role in iron-sulfur cluster biogenesis. The encoded protein assembles and transfers 4Fe-4S clusters to target apoproteins including succinate dehydrogenase and lipoic acid synthase. Mutations in this gene are a cause of multiple mitochondrial dysfunctions syndrome-1, and pseudogenes of this gene are located on the short arms of chromosomes 1 and 3. Alternatively spliced transcript variants encoding multiple isoforms have been observed for this gene.
HIRA-interacting protein 5
, NFU1 iron-sulfur cluster scaffold homolog, mitochondrial
, NifU-like C-terminal domain containing
, iron-sulfur cluster scaffold protein
, histone cell cycle regulation defective interacting protein 5