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: 20 bp deletion in exon 2
Product details:
We will provide viable cells that proliferate on revival.
This product is subject to limited use licenses from The Broad Institute and ERS Genomics Limited, and is developed with patented technology. For full details of the limited use licenses and relevant patents please refer to our
limited use license
patent pages
Properties and Storage Information:
Gene name-ECH1, 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.
ECH1 also known as Delta(35)-Delta(24)-dienoyl-CoA isomerase is a mitochondrial protein with a mass of approximately 31 kDa. It is expressed in a wide variety of tissues including high levels in the liver heart and muscle. This protein plays an important role in the beta-oxidation of unsaturated fatty acids inside mitochondria by catalyzing the conversion of 35-dienoyl-CoA to 24-dienoyl-CoA. Because of this function ECH1 contributes to the efficient metabolism of fatty acids.
Biological function summary
The ECH1 protein facilitates the breakdown and utilization of unsaturated fatty acids thereby providing energy for cellular processes. It acts independently and is not a part of a large protein complex. Mitochondria rely on ECH1 to handle specific intermediates in fatty acid metabolism which impacts how cells manage their energy resources. This makes ECH1 essential for maintaining energy homeostasis particularly during periods of increased metabolic demand.
Pathways
ECH1 is integral to the mitochondrial fatty acid beta-oxidation pathway. This pathway is important for converting fatty acids into acetyl-CoA units which then enter the Krebs cycle for further energy production. ECH1 interacts with other proteins like ACAA2 which contributes to the final step of fatty acid degradation. Alongside this it aligns with the peroxisomal biogenesis pathway influencing the balance between metabolic processes within mitochondria and peroxisomes.
ECH1 activity impacts conditions such as metabolic syndrome and inherited disorders of mitochondrial fatty acid oxidation including Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency. Changes in the function or expression of ECH1 can lead to accumulation of fatty acid intermediates causing cellular dysfunction. In diseases where energy balance is disrupted like metabolic syndrome ECH1's interaction with proteins like HADHA which assists in the final steps of beta-oxidation becomes significant for understanding and potentially targeting treatments.
