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Rabbit Anti-ISCU Antibody (CSB-PA887955ESR2HU)
Supplier: CUSABIO BIOTECH CO.
Iron-sulfur cluster assembly enzyme ISCU Antibody,mitochondrial Antibody,NifU-like N-terminal domain-containing protein Antibody,NifU-like protein Antibody,ISCU Antibody,NIFUN AntibodyMore alternative names for the antibody
2310020H20Rik antibody|HML antibody|hnifU antibody|Iron sulfur cluster assembly enzyme ISCU mitochondrial antibody|Iron sulfur cluster scaffold homolog (E. coli) antibody|Iron sulfur cluster scaffold homolog antibody|Iron-sulfur cluster assembly enzyme ISCU antibody|Iscu antibody|IscU iron sulfur cluster scaffold homolog antibody|ISCU_HUMAN antibody|ISU2 antibody|MGC74517 antibody|mitochondrial antibody|NIFU antibody|NifU like N terminal domain containing antibody|NifU like N terminal domain containing protein antibody|NifU like protein antibody|NifU-like N-terminal domain-containing protein antibody|NifU-like protein antibody|NIFUN antibody|Nitrogen fixation cluster like antibody|OTTHUMP00000238760 antibody|OTTHUMP00000238761 antibody|OTTHUMP00000238762 antibody|OTTHUMP00000238764 antibody|OTTHUMP00000238765 antibody
Anti-ISCU antibody (ab154060)
Anti-ISCU antibody (ab154060)
Recommended applications: ELISA, IHC
Recommended dilution: Recommended dilution:IHC:1:20-1:200
Recommended protocols: check protocols
|Catalogue No.|| |
Recombinant human Iron-sulfur cluster assembly enzyme ISCU, mitochondrial protein (1-167AA)
|Recommended dilution|| |
|Molecular weight|| |
Antigen Affinity Purified
Shipped at 4 Celcius Degree. Upon delivery aliquot and store at -20 Celcius Degree or -80 Celcius Degree. Avoid repeated freeze.
|Database links|| |
Entrez Gene:514789( Cow), Entrez Gene:23479( Human), Entrez Gene:66383( Mouse), Entrez Gene:288740( Rat), Omim:611911( Human), SwissProt:Q9H1K1( Human), SwissProt:Q9D7P6( Mouse), Unigene:615131( Human), Unigene:29497( Mouse), Unigene:121( Rat)
|Protein function|| |
Scaffold protein for the de novo synthesis of iron-sulfur (Fe-S) clusters within mitochondria, which is required for maturation of both mitochondrial and cytoplasmic [2Fe-2S] and [4Fe-4S] proteins (PubMed:11060020). First, a [2Fe-2S] cluster is transiently assembled on the scaffold protein ISCU. In a second step, the cluster is released from ISCU, transferred to a glutaredoxin GLRX5, followed by the formation of mitochondrial [2Fe-2S] proteins, the synthesis of [4Fe-4S] clusters and their target-specific insertion into the recipient apoproteins. Cluster assembly on ISCU depends on the function of the cysteine desulfurase complex NFS1-LYRM4/ISD11, which serves as the sulfur donor for cluster synthesis, the iron-binding protein frataxin as the putative iron donor, and the electron transfer chain comprised of ferredoxin reductase and ferredoxin, which receive their electrons from NADH (By similarity). .
|Protein tissue specificity|| |
Detected in heart, liver, skeletal muscle, brain, pancreas, kidney, lung and placenta. .
|Involvement in disease|| |
Myopathy with exercise intolerance Swedish type (MEIS) [MIM:255125]: Autosomal recessive metabolic disease 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. . Note=The disease is caused by mutations affecting the gene represented in this entry.
|Protein sequence and domain|| |
Belongs to the NifU family.
|Protein cellular localization|| |
Isoform 1: Mitochondrion .; Isoform 2: Cytoplasm. Nucleus .
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CUSABIO BIOTECH CO.
|Product type|| |
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