Goat Electron Transferring Flavoprotein Dehydrogenase ELISA Kit | ETFDH elisa kit
Goat Electron Transferring Flavoprotein Dehydrogenase ELISA Kit
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a component of the electron-transfer system in mitochondria and is essential for electron transfer from a number of mitochondrial flavin-containing dehydrogenases to the main respiratory chain. Mutations in this gene are associated with glutaric acidemia. Alternatively spliced transcript variants that encode distinct isoforms have been observed. [provided by RefSeq, Aug 2013]
Uniprot Description
ETFDH: Accepts electrons from ETF and reduces ubiquinone. Defects in ETFDH are the cause of glutaric aciduria type 2C (GA2C). GA2C is an autosomal recessively inherited disorder of fatty acid, amino acid, and choline metabolism. It is characterized by multiple acyl-CoA dehydrogenase deficiencies resulting in large excretion not only of glutaric acid, but also of lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids. Belongs to the ETF-QO/FixC family.
Protein type: EC 1.5.5.1; Oxidoreductase; Mitochondrial; Membrane protein, integral
Chromosomal Location of Human Ortholog: 4q32-q35
Cellular Component: mitochondrial matrix; mitochondrial membrane; integral to mitochondrial inner membrane
Molecular Function: ubiquinone binding; electron carrier activity; FAD binding; oxidoreductase activity, oxidizing metal ions with flavin as acceptor; metal ion binding; 4 iron, 4 sulfur cluster binding; oxidoreductase activity; quinone binding; electron-transferring-flavoprotein dehydrogenase activity
Biological Process: cellular metabolic process; response to oxidative stress; fatty acid beta-oxidation using acyl-CoA dehydrogenase
Disease: Multiple Acyl-coa Dehydrogenase Deficiency
Research Articles on ETFDH
Similar Products
Product Notes
The Goat ETFDH etfdh (Catalog #AAA093213) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA093213 ELISA Kit recognizes Goat ETFDH. It is sometimes possible for the material contained within the vial of "Electron Transferring Flavoprotein Dehydrogenase, ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
If you are ready to order, navigate to Shopping Cart and get ready to checkout.