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Iron-sulfur (Fe-S) clusters are necessary for several mitochondrial enzymes and other subcellular compartment proteins. Zusätzlich bieten wir Ihnen ISCU Antikörper (59) und ISCU Proteine (7) und viele weitere Produktgruppen zu diesem Protein an.
We have shown that ASO treatment diminished aberrant splicing and increased ISCU protein levels in both patient fibroblasts and patient myotubes in a concentration dependent fashion. Upon ASO treatment, levels of SDHB (zeige SDHB ELISA Kits) in patient myotubular cell lines increased to levels observed in control myotubular cell lines
The NFS1 (zeige NFS1 ELISA Kits)/ISD11 (zeige LYRM4 ELISA Kits) complex further interacts with scaffold protein (zeige HOMER1 ELISA Kits) ISCU and regulator protein frataxin (zeige FXN ELISA Kits), thereby forming a quaternary complex for Fe-S cluster formation.
Molecular dynamics flexible fitting of protein structures docked into the EM map of the model revealed a [FXN (zeige FXN ELISA Kits)(42-210)]24.[NFS1 (zeige NFS1 ELISA Kits)]24.[ISD11 (zeige LYRM4 ELISA Kits)]24.[ISCU]24 complex, consistent with the measured 1:1:1:1 stoichiometry of its four components.
ISCU expression was decreased in the majority of human liver cancer tissues, and its reduced expression was significantly associated with p53 (zeige TP53 ELISA Kits) mutation.
Thus, driven by acquired (hypoxia) or genetic causes, the miR (zeige MLXIP ELISA Kits)-210-ISCU1/2 regulatory axis is a pathogenic lynchpin causing iron-sulfur deficiency and pulmonary hypertension.
The core Fe-S biosynthetic enzymatic complex generated [2Fe-2S] cluster intermediates that converted to stable [2Fe-2S] clusters bound to uncomplexed ISCU2.
IscU is a new substrate of MK2 (zeige KCNA2 ELISA Kits) both in Drosophila cells and in human cells
Fe-S assembly protein (ISCU2) and frataxin (zeige FXN ELISA Kits) convert substrates l-cysteine, ferrous iron, and electrons into Fe-S clusters.
the G50E iron-sulfur cluster scaffold protein (zeige NFU1 ELISA Kits) (ISCU) mutation has a role in mitochondrial myopathy
NFS1 (zeige NFS1 ELISA Kits) binds preferentially to the D-state of ISCU while mtHSP70 (zeige HSPA9 ELISA Kits) binds preferentially to the D-state of ISCU and HSC20 binds preferentially to the S-state of ISCU.
IscU is a marginally stable protein at low ionic strength to the point that undergoes cold denaturation at around -8 degrees C with a corresponding dramatic decrease of enthalpy, which is consistent with the fluxional nature of the protein.
mTORC1 associates with ISCU and phosphorylates ISCU at serine 14. This phosphorylation stabilized ISCU protein.
Data show that complete loss of ISCU results in early embryonic death and confirm a fundamental role for ISCU in mammals.
While IFN-gamma (zeige IFNG ELISA Kits) alone induced Nfs1 (zeige NFS1 ELISA Kits) protein instability, LPS (zeige TLR4 ELISA Kits) triggered a delayed decline of Nfs1 (zeige NFS1 ELISA Kits), rather involving transcriptional events or mRNA instability.
Iron-sulfur (Fe-S) clusters are necessary for several mitochondrial enzymes and other subcellular compartment proteins. They contain sulfur and iron, and are created via several steps that include cysteine desulfurases, iron donors, chaperones, and scaffold proteins. This gene encodes the two isomeric forms, ISCU1 and ISCU2, of the Fe-S cluster scaffold protein. Mutations in this gene have been found in patients with myopathy with severe exercise intolerance and myoglobinuria.
iron-sulfur cluster assembly enzyme ISCU, mitochondrial
, iron-sulfur cluster scaffold homolog (E. coli)
, iron-sulfur cluster assembly enzyme
, IscU iron-sulfur cluster scaffold homolog (E. coli)
, NifU-like N-terminal domain containing
, zC191D15.3 (novel protein with leucine-rich repeat domains)
, IscU iron-sulfur cluster scaffold homolog
, iron-sulfur cluster scaffold homolog
, nifU-like N-terminal domain-containing protein
, nifU-like protein