Name: Anti-ISCU Antibody (CAB7082)
Brand: Cell Biology Antibodies 10
SKU: CAB7082
Availability: OutOfStock

Antibody Name:	Anti-ISCU Antibody
Antibody SKU:	CAB7082
Antibody Size:	20uL, 50uL, 100uL
Application:	WB
Reactivity:	Human
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-167 of human ISCU (NP_998760.1).

Application:	WB
Recommended Dilution:	WB 1:500 - 1:2000
Reactivity:	Human
Positive Samples:	HT-1080, U-87MG, 293T, A-549, HeLa

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-167 of human ISCU (NP_998760.1).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	MAAA GAFR LRRA ASAL LLRS PRLP AREL SAPA RLYH KKVV DHYE NPRN VGSL DKTS KNVG TGLV GAPA CGDV MKLQ IQVD EKGK IVDA RFKT FGCG SAIA SSSL ATEW VKGK TVEE ALTI KNTD IAKE LCLP PVKL HCSM LAED AIKA ALAD YKLK QEPK KGEA EKK
Gene ID:	23479
Uniprot:	Q9H1K1
Cellular Location:	Cytoplasm, Mitochondrion, Nucleus
Calculated MW:	15kDa/17kDa
Observed MW:	14-18kDa

Synonyms:

ISCU, 2310020H20Rik, HML, ISU2, NIFU, NIFUN, hnifU

Background:

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.

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

Western blot analysis of extracts of various cell lines, using ISCU Antibody (CAB7082) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Enhanced Kit (RM00021). Exposure time: 10s.

Antibodies

Anti-ISCU Antibody (CAB4557)

ISCU Antibody (PACO09996)

Secondary Antibody

Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014)