Tissue specificity of a human mitochondrial disease: differentiation-enhanced mis-splicing of the Fe-S scaffold gene ISCU renders patient cells more sensitive to oxidative stress in ISCU myopathy.

Tissue specificity of a human mitochondrial disease: differentiation-enhanced mis-splicing of the Fe-S scaffold gene ISCU renders patient cells more sensitive to oxidative stress in ISCU myopathy.

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http://www.wikidata.org/entity/Q36418756
Iron-sulfur cluster biogenesis in mammalian cells: New insights into the molecular mechanisms of cluster deliveryThe role of mitochondria in cellular iron-sulfur protein biogenesis: mechanisms, connected processes, and diseasesTurning Saccharomyces cerevisiae into a Frataxin-Independent Organism.Protein-mediated assembly of succinate dehydrogenase and its cofactorsSynthesis, delivery and regulation of eukaryotic heme and Fe-S cluster cofactors.Insertion mutants in Drosophila melanogaster Hsc20 halt larval growth and lead to reduced iron-sulfur cluster enzyme activities and impaired iron homeostasis.Elevated FGF21 secretion, PGC-1α and ketogenic enzyme expression are hallmarks of iron-sulfur cluster depletion in human skeletal muscleLipoic acid biosynthesis defects.Differential diagnosis of lipoic acid synthesis defects.Faster and stronger manifestation of mitochondrial diseases in skeletal muscle than in heart related to cytosolic inorganic phosphate (Pi) accumulation.Iron and copper in mitochondrial diseases.A novel de novo dominant mutation in ISCU associated with mitochondrial myopathy.Use of antisense oligonucleotides to correct the splicing error in ISCU myopathy patient cell lines.Acute loss of iron-sulfur clusters results in metabolic reprogramming and generation of lipid droplets in mammalian cells.NMR as a Tool to Investigate the Processes of Mitochondrial and Cytosolic Iron-Sulfur Cluster Biosynthesis
P2860

Tissue specificity of a human mitochondrial disease: differentiation-enhanced mis-splicing of the Fe-S scaffold gene ISCU renders patient cells more sensitive to oxidative stress in ISCU myopathy.

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http://www.wikidata.org/entity/Q36418756
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2012 nî lūn-bûn
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2012年の論文
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2012年学术文章
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name
Tissue specificity of a human ...... ative stress in ISCU myopathy.
@ast
Tissue specificity of a human ...... ative stress in ISCU myopathy.
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type
label
Tissue specificity of a human ...... ative stress in ISCU myopathy.
@ast
Tissue specificity of a human ...... ative stress in ISCU myopathy.
@en
prefLabel
Tissue specificity of a human ...... ative stress in ISCU myopathy.
@ast
Tissue specificity of a human ...... ative stress in ISCU myopathy.
@en
P1433
P1476
P2093
P2860
P304
P31
P356
P407
P433
P478
P50
P577
P698
P921
P932
P356
P1433
P1476
Tissue specificity of a human ...... ative stress in ISCU myopathy.
@en
P2093
P2860
P304
P31
P356
P407
P433
P478
P50
P577
P698
P921
P932