Iron-sulfur Cluster Scaffold Homolog Recombinant Protein | ISCU recombinant protein
Recombinant Human Iron-sulfur Cluster Scaffold Homolog
Sterile Filtered colorless solution.
Introduction: Iron-sulfur cluster assembly enzyme (ISCU) belongs to the nifU family. Iron-sulfur (Fe-S) clusters are required for several mitochondrial enzymes and other subcellular compartment proteins. ISCU interacts with ISCS (a cysteine desulfurase) to sequester inorganic sulfur for Fe-S cluster assembly. The ISCU-ISCS protein complex localizes in both mitochondria and cytosol, implying that Fe-S cluster assembly occurs in multiple subcellular compartments in mammalian cells.
NCBI and Uniprot Product Information
NCBI Description
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. A pseudogene of this gene is present on chromosome 1. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Jul 2014]
Uniprot Description
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 dehydrogenase 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
Chromosomal Location of Human Ortholog: 12q24.1
Cellular Component: mitochondrion; mitochondrial matrix; cytoplasm; nucleus; cytosol
Molecular Function: protein binding; iron ion binding; iron-sulfur cluster binding; protein complex scaffold
Biological Process: iron-sulfur cluster assembly; nitrogen fixation
Disease: Myopathy With Lactic Acidosis, Hereditary
Research Articles on ISCU
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Product Notes
The ISCU iscu (Catalog #AAA144588) is a Recombinant Protein produced from E Coli and is intended for research purposes only. The product is available for immediate purchase. The amino acid sequence is listed below: MGSSHHH HHH SSGLVPRGSH MYHKKV VDHY ENPRNVGSLD KTSKNVGTGL VGAPACGDVM KLQIQVDEKG KIVDARFKTF GCGSAIASSS LATEWVKGKT VEEALTIKNT DIAKELCLPP VKLHCSMLAE DAIKAALADY KLKQEPKKGE AEKK. It is sometimes possible for the material contained within the vial of "Iron-sulfur Cluster Scaffold Homolog, Recombinant Protein" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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