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ETHE1 Protein

Ethylmalonic Encephalopathy 1, HSCO, Hepatoma subtracted clone one protein, YF13H12, protein ETHE1 mitochondrial, D83198

Catalog No. Product Name Size List Price (US$) Quantity
BP002879-PRO-1027 Recombinant Human Ethylmalonic Encephalopathy 1 Protein 20 ug 160.00
BP002879-PRO-1027 Recombinant Human Ethylmalonic Encephalopathy 1 Protein 1 mg 3600.00
Description

Introduction
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.

BP002879-PRO-1027: Recombinant Human Ethylmalonic Encephalopathy 1 (ETHE1) Protein

Source: E. coli-derived.
A single, non-glycosylated polypeptide chain containing 267 amino acids (13-254) and having a molecular mass of 29.1 kDa. ETHE1 is fused to a 25 amino acid His-tag at N-terminus.
Purity: > 90% as determined by SDS-PAGE.
Formulation: The ETHE1 solution (1 mg/ml) contains 20 mM Tris-HCl buffer (pH 8.0), 100 mM NaCl and 10% glycerol.

Shipping: The product is shipped with ice pack. Upon receipt, store it immediately at the temperature recommended below.
Stability & Storage: Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
12 months from date of receipt, -20 to -70°C as supplied.

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