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NFU1 encodes a protein that is localized to mitochondria and plays a critical role in iron-sulfur cluster biogenesis. Additionally we are shipping NFU1 Iron-Sulfur Cluster Scaffold Homolog (S. Cerevisiae) Antibodies (5) and many more products for this protein.
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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 (show BOLA3 Proteins) 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 (show EPM2A Proteins) interacts with HIRIP5.
NFU binds to NifS (show NFS1 Proteins) and reduces the persulfide bond on activated NifS (show NFS1 Proteins) (following formation of the persulfide bond by abstraction of S from the Cys (show DNAJC5 Proteins) amino acid), yielding inorganic sulfide (show SQRDL Proteins) on a time frame that is compatible with Fe-S cluster assembly.
NFU is a functionally competent reducing agent for cysteinyl persulfide bond cleavage, releasing inorganic sulfide (show SQRDL Proteins) for incorporation into the iron-sulfur-bound [2Fe-2S] cluster, a reactivity that may be facilitated by flexibility of the C-terminal domain.
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