Rabbit ISCU Polyclonal Antibody | anti-ISCU antibody

ISCU Rabbit Polyclonal

Cat. Num. AAA7600635

• Gene Names: ISCU; HML; ISU2; NIFU; NIFUN; hnifU; 2310020H20Rik
• Reactivity: Human, Mouse, Rat
• Applications: ELISA, Western Blot, Immunohistochemistry, Immunofluorescence, Immunoprecipitation
• Purity: >=95% as determined by SDS-PAGE; Immunogen Affinity Purified
• Synonyms: ISCU; Polyclonal Antibody; ISCU Rabbit Polyclonal; anti-ISCU antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: >=95% as determined by SDS-PAGE; Immunogen Affinity Purified
• Form/Format: Liquid
• Sequence Length: 129

• Applicable Applications for anti-ISCU antibody: ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF), Immunoprecipitation (IP)
• Immunogen: Iron-sulfur cluster scaffold homolog (E. coli)
• Preparation and Storage: PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded human lung cancer using MBS7600635 (ISCU antibody) at dilution of 1:50)

Western Blot (WB)

(mouse heart tissue were subjected to SDS PAGE followed by western blot with MBS7600635 (ISCU antibody) at dilution of 1:500)

NCBI and Uniprot Product Information

• NCBI GI #: 169261975
• NCBI GeneID: 23479
• Molecular Weight: 15,263 Da
• NCBI Official Full Name: ISCU
• NCBI Official Synonym Full Names: iron-sulfur cluster assembly enzyme
• NCBI Official Symbol: ISCU
• NCBI Official Synonym Symbols: HML; ISU2; NIFU; NIFUN; hnifU; 2310020H20Rik
• NCBI Protein Information: iron-sulfur cluster assembly enzyme ISCU, mitochondrial
• UniProt Protein Name: Iron-sulfur cluster assembly enzyme ISCU, mitochondrial
• Protein Family: Iron-sulfur cluster assembly enzyme
• UniProt Gene Name: ISCU
• UniProt Synonym Gene Names: NIFUN
• UniProt Entry Name: ISCU_HUMAN

NCBI Description

This gene encodes a component of the iron-sulfur (Fe-S) cluster scaffold. Fe-S clusters are cofactors that play a role in the function of a diverse set of enzymes, including those that regulate metabolism, iron homeostasis, and oxidative stress response. Alternative splicing results in transcript variants encoding different protein isoforms that localize either to the cytosol or to the mitochondrion. Mutations in this gene have been found in patients with hereditary myopathy with lactic acidosis. A disease-associated mutation in an intron may activate a cryptic splice site, resulting in the production of a splice variant encoding a putatively non-functional protein. A pseudogene of this gene is present on chromosome 1. [provided by RefSeq, Feb 2016]

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: cytoplasm; cytosol; mitochondrial matrix; mitochondrion; nucleus

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

Biological Process: cellular iron ion homeostasis; iron-sulfur cluster assembly; nitrogen fixation

Disease: Myopathy With Lactic Acidosis, Hereditary