ISCU, cDNA Clone

For Research Use Only. Not for use in diagnostic procedures.

Chromosome: 12; NC_000012.11 (108956294..108963160). Location: 12q24.1

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Small volumes of ISCU cdna clone vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

17,999 Da

iron-sulfur cluster scaffold homolog (E. coli)

iron-sulfur cluster assembly enzyme ISCU, mitochondrial; nifU-like protein; NifU-like N-terminal domain containing; IscU iron-sulfur cluster scaffold homolog; nifU-like N-terminal domain-containing protein

Iron-sulfur cluster assembly enzyme ISCU, mitochondrial

NIFUN  [Similar Products]

ISCU_HUMAN

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]

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

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