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Iron-sulfur cluster assembly enzyme

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Scaffold protein for the de novo synthesis of iron-sulfur (Fe-S) clusters within mitochondria, which is required for maturation of both mitochondrial and cytoplasmic [2Fe-2S] and [4Fe-4S] proteins. First, a [2Fe-2S] cluster is transiently assembled on the scaffold protein ISCU. In a second step, the cluster is released from ISCU, transferred to a glutaredoxin GLRX5, followed by the formation of mitochondrial [2Fe-2S] proteins, the synthesis of [4Fe-4S] clusters and their target-specific insertion into the recipient apoproteins. Cluster assembly on ISCU depends on the function of the cysteine desulfurase complex NFS1-LYRM4/ISD11, which serves as the sulfur donor for cluster synthesis, the iron-binding protein frataxin as the putative iron donor, and the electron transfer chain comprised of ferredoxin reductase and ferredoxin, which receive their electrons from NADH (By similarity).

Below are the list of possible Iron-sulfur cluster assembly enzyme products. If you cannot find the target and/or product is not available in our catalog, please click here to contact us and request the product or submit your request for custom elisa kit production, custom recombinant protein production or custom antibody production. Custom ELISA Kits, Recombinant Proteins and Antibodies can be designed, manufactured and produced according to the researcher's specifications.
 

Iron-sulfur cluster assembly enzyme ISCU

 Iron-sulfur cluster assembly enzyme ISCU ELISA Kit
 Iron-sulfur cluster assembly enzyme ISCU Recombinant
 Iron-sulfur cluster assembly enzyme ISCU Antibody
Also known as Iron-sulfur cluster assembly enzyme ISCU, mitochondrial (NifU-like N-terminal domain-containing protein) (NifU-like protein).
ISCU: Involved in the assembly or repair of the [Fe-S] clusters present in iron-sulfur proteins. Binds iron. Defects in ISCU are the cause of myopathy with exercise intolerance Swedish type (MEIS); also known as myopathy with deficiency of succinate dehydro
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genase and aconitase or myoglobinuria due to abnormal glycolysis or hereditary myopathy with lactic acidosis (HML). This autosomal recessive metabolic disease is characterized by lifelong severe exercise intolerance, in which minor exertion causes fatigue of active muscles, shortness of breath, and cardiac palpitations in association with lactic acidosis. The biochemical phenotype is characterized by a deficiency in mitochondrial iron-sulfur proteins and impaired muscle oxidative metabolism. Belongs to the NifU family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Mitochondrial

Cellular Component: cytoplasm; cytosol; mitochondrial matrix; mitochondrion

Molecular Function: 2 iron, 2 sulfur cluster binding; 4 iron, 4 sulfur cluster binding; ferrous iron binding; protein complex scaffold

Biological Process: cellular iron ion homeostasis
 Iscu ELISA Kit
 Iscu Recombinant
 Iscu Antibody
 Nifun ELISA Kit
 Nifun Recombinant
 Nifun Antibody
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