ISCU cdna clone
ISCU cDNA Clone
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. [provided by RefSeq, Jul 2008]
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; cytosol; nucleus
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 #AAA1270301) is a cDNA Clone and is intended for research purposes only. The product is available for immediate purchase. The amino acid sequence is listed below: atggcggcgg ctggggcttt ccgtctgagg cgggcggcat cggctctgct gctgcggagc ccccgcctgc ccgcccggga gctgtcggcc ccggcccgac tctatcacaa gaaggttgtt gatcattatg aaaatcctag aaacgtgggg tcccttgaca agacatctaa aaatgttgga actggactgg tgggggctcc agcatgtggt gacgtaatga aattacagat tcaagtggat gaaaagggga agattgtgga tgctaggttt aaaacatttg gctgtggttc cgcaattgcc tccagctcat tagccactga atgggtgaaa ggaaagacgg tggaggaagc cttgactatc aaaaacacag atatcgccaa ggagctctgc cttcctcccg tgaaactgca ctgctccatg ctggctgaag atgcaatcaa ggccgccctg gctgattaca aattgaaaca agaacccaaa aaaggagagg cagagaagaa atga. It is sometimes possible for the material contained within the vial of "ISCU, cDNA Clone" 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
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
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