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Mouse Anti-ISCU Recombinant Antibody (CBYY-I1355) (CBMAB-I2525-YY)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
CBYY-I1355
Antibody Isotype
IgG1
Application
WB

Basic Information

Specificity
Human
Antibody Isotype
IgG1
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
1% BSA, pH 7.3, 50% glycerol
Preservative
0.02% sodium azide
Concentration
1 mg/ml
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
iron-sulfur cluster scaffold homolog (E. coli)
Introduction
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.
Entrez Gene ID
23479
UniProt ID
Q9H1K1
Alternative Names
Iron-Sulfur Cluster Assembly Enzyme
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).
Isoform 2
Functions as a cytoplasmic scaffold protein for the de novo synthesis of iron-sulfur clusters in the cytoplasm.
Biological Process
Cellular iron ion homeostasisManual Assertion Based On ExperimentIBA:GO_Central
Iron-sulfur cluster assemblyManual Assertion Based On ExperimentTAS:UniProtKB
Negative regulation of iron ion import across plasma membraneManual Assertion Based On ExperimentIMP:ARUK-UCL
Positive regulation of aconitate hydratase activityManual Assertion Based On ExperimentIMP:ARUK-UCL
Positive regulation of mitochondrial electron transport, NADH to ubiquinoneManual Assertion Based On ExperimentIMP:ARUK-UCL
Cellular Location
Isoform 1: Mitochondrion
Isoform 2: Cytoplasm; Nucleus
Involvement in disease
Myopathy with exercise intolerance Swedish type (MEIS):
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.
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For research use only. Not intended for any clinical use.

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