Western Blot (WB)
(ISCU Antibody (C-term) western blot analysis in WiDr cell line lysates (35ug/lane).This demonstrates the ISCU antibody detected the ISCU protein (arrow).)
Rabbit anti-Human, mouse ISCU Polyclonal Antibody | anti-ISCU antibody
ISCU Antibody (C-term)
Western Blot (WB)
(ISCU Antibody (C-term) western blot analysis in WiDr cell line lysates (35ug/lane).This demonstrates the ISCU antibody detected the ISCU protein (arrow).)
Western Blot (WB)
(ISCU Antibody (C-term) western blot analysis in WiDr cell line lysates (35ug/lane).This demonstrates the ISCU antibody detected the ISCU protein (arrow).)
Western Blot (WB)
(ISCU Antibody (C-term) western blot analysis in mouse lung tissue lysates (35ug/lane).This demonstrates the ISCU antibody detected the ISCU protein (arrow).)
Western Blot (WB)
(ISCU Antibody (C-term) western blot analysis in mouse lung tissue lysates (35ug/lane).This demonstrates the ISCU antibody detected the ISCU protein (arrow).)
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.
Chan, S.Y., et al. Cell Metab. 10(4):273-284(2009)
Kollberg, G., et al. Brain 132 (PT 8), 2170-2179 (2009) :
Huang, J., et al. J. Biol. Inorg. Chem. 13(5):825-836(2008)
Mochel, F., et al. Am. J. Hum. Genet. 82(3):652-660(2008)
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 #AAA9209539) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The immunogen sequence is 138-167. The ISCU Antibody (C-term) reacts with Human, mouse and may cross-react with other species as described in the data sheet. AAA Biotech's ISCU can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), ELISA (EIA). WB~~1:1000. Researchers should empirically determine the suitability of the ISCU iscu for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "ISCU, Polyclonal Antibody" 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
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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