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Recombinant human ETHE1 protein

CAT: 0112-ATGP1382-100Size: 100 µgDry Ice: NoHazardous: No
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CAT#:0112-ATGP1382-100Size:100 µg
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24/48H Stock Items & 2 to 6 Weeks non Stock Items.
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Description
ETHE1 is a mitochondrial sulfur dioxygenase involved in catabolism of sulfide that accumulates to toxic levels in ethylmalonic encephalopathy. Mutations of ETHE1 were detected in all the typical ethylmalonic encephalopathy patients analysed, but no ETHE1 mutations were identified in patients presenting with early onset progressive encephalopathy with ethylmalonic aciduria. Recombinant human ETHE1 protein, fused to His-tag at N-terminus, was expressed in E. coli and purified by using conventional chromatography.
Product Name Alternative
Protein ETHE1 mitochondrial, Protein ETHE1, mitochondrial
Expression System
E.coli
Antigen Species
Human
Tag
His-Tag
Applications
SDS-PAGE
Concentration
1 mg/mL (determined by Bradford assay)
Purity
> 90% by SDS-PAGE
Molecular Weight
29.1 kDa (267aa) confirmed by MALDI-TOF
Additionnal Information
ETHE1, Protein ETHE1 mitochondrial, Protein ETHE1, mitochondrial, ATGP1382-10 µg, ATGP1382-20 µg, ATGP1382-50 µg, ATGP1382-100 µg, ATGP1382-250 µg, ATGP1382-500 µg, ATGP1382-1 mg, ATGP1382-10, ATGP1382-20, ATGP1382-50, ATGP1382-100, ATGP1382-250, ATGP1382-500, ATGP1382-1
References & Citations
Tiranti V. et al. (2009) Nat Med. 15:200-205.; ; Mineri R. et al. (2008) J Med Genet. 45:473-478
Storage Conditions
Can be stored at 2°C to 8°C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.
Formulation
Liquid in. 20 mM Tris-HCl buffer (pH 8.0) containing 10% glycerol, 0.1M NaCl
Scientific Category
Metabolism
NCBI Accession Number
NP_055112
Uniprot Accession Number
O95571
Species
Human
AA Sequence
LSQRG GSGAPILLRQ MFEPVSCTFT YLLGDRESRE AVLIDPVLET APRDAQLIKE LGLRLLYAVN THCHADHITG SGLLRSLLPG CQSVISRLSG AQADLHIEDG DSIRFGRFAL ETRASPGHTP GCVTFVLNDH SMAFTGDALL IRGCGRTDFQ QGCAKTLYHS VHEKIFTLPG DCLIYPAHDY HGFTVSTVEE ERTLNPRLTL SCEEFVKIMG NLNLPKPQQI DFAVPANMRC GVQTPTA

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