Anti-ISCU Antibody (CAB4557)

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SKU:
CAB4557
Product type:
Antibody
Antibody Type:
Polyclonal Antibody
Reactivity:
Human
Reactivity:
Mouse
Host Species:
Rabbit
Isotype:
IgG
Synonyms:
ISCU
Synonyms:
2310020H20Rik
Synonyms:
HML
Synonyms:
ISU2
Synonyms:
NIFU
Synonyms:
NIFUN
Synonyms:
hnifU
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Description

Product Name:ISCU Rabbit pAb
Product Code:CAB4557
Size:100uL
Synonyms:ISCU, 2310020H20Rik, HML, ISU2, NIFU, NIFUN, hnifU
Applications:WB
Reactivity:Human, Mouse
Host Species:Rabbit
Immunogen:A synthetic Peptide of human ISCU
Applications:WB
Recommended Dilutions:WB 1:500 - 1:1000
Reactivity:Human, Mouse
Positive Samples:Mouse lung
Immunogen:A synthetic Peptide of human ISCU
Purification Method:Affinity purification
Storage:Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:Email for sequence
Gene ID:23479
Uniprot:Q9H1K1
Cellular Location:Cytoplasm, Mitochondrion, Nucleus
Calculated MW:15kDa/17kDa
Observed MW:26kDa
UniProt Protein Function: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.
UniProt Protein Details:

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

NCBI Summary: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 Code:Q9H1K1
NCBI GenInfo Identifier:313104118
NCBI Gene ID:23479
NCBI Accession:Q9H1K1.2
UniProt Related Accession:Q9H1K1
Molecular Weight:18kDa
NCBI Full Name:Iron-sulfur cluster assembly enzyme ISCU, mitochondrial
NCBI 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
UniProt Synonym Protein Names:NifU-like N-terminal domain-containing protein; NifU-like protein
Protein Family:Iron-sulfur cluster assembly enzyme
UniProt Gene Name:ISCU  
UniProt Entry Name:ISCU_HUMAN
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