Description
Key facts
Cell type:HEK-293T,
Species or organism:Human,
Tissue:Kidney,
Form:LiquidSee storage information,
Knockout validation:Sanger Sequencing,Western blot,
Mutation description:Knockout achieved by using CRISPR/Cas9, Homozygous: Insertion of the selection cassette in exon 1
Product details:
We will provide viable cells that proliferate on revival.
This product is subject to limited use licenses from The Broad Institute, ERS Genomics Limited and Sigma-Aldrich Co. LLC, and is developed with patented technology. For full details of the licenses and patents please refer to our
limited use license
patent pages
Properties and Storage Information:
Gene name-ETFA, Gene editing type-Knockout, Gene editing method-CRISPR technology, Knockout validation-Sanger Sequencing, Western blot, Zygosity-Homozygous, Shipped at conditions-Dry Ice, Appropriate short-term storage conditions--196°C, Appropriate long-term storage conditions--196°C
Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
ETFA or Electron-Transfer-Flavoprotein alpha subunit is an essential part of the mitochondrial respiratory chain. This protein which has a molecular mass of approximately 34 kDa functions in the transfer of electrons from acyl-CoA dehydrogenases to the main respiratory chain for energy production. ETFA is commonly expressed in tissues with high-energy demands such as the liver heart and skeletal muscle. The protein forms a heterodimeric complex with its counterpart ETFB providing a critical function in electron transfer during fatty acid oxidation.
Biological function summary
ETFA operates as an important part of the electron transfer process within the mitochondria. It acts as one-half of the heterodimeric electron-transfer flavoprotein complex teaming with ETFB. This complex facilitates electron transfer from a range of acyl-CoA dehydrogenases to ETF dehydrogenase which then continues the process of electron transfer to coenzyme Q in the respiratory chain. This action is key to the breakdown of fats enabling energy extraction and processing.
Pathways
ETFA has important roles in fatty acid beta-oxidation and amino acid catabolism. It engages in these pathways by transferring electrons as mentioned interfacing with other proteins like ETF dehydrogenase. This positioning within the mitochondrial matrix enables ETFA to assist in converting fat and protein substrates into energy which the cell can use. Its molecular interactions highlight its integral position in maintaining energy homeostasis.
Problems with ETFA lead to glutaric acidemia type 2 a metabolic disorder that impairs the body's ability to oxidize fatty acids and some amino acids. Deficiencies in ETFA function disrupt the electron transport to the respiratory chain causing an accumulation of intermediary metabolites. These disruptions can relate to or involve other mitochondrial components and proteins like ETFB or ETFDH. Correct diagnosis and understanding of ETFA's role in such conditions are instrumental for targeted therapeutic approaches.
