Abcam, ab228631, Anti-ECHS1 antibody

Size: 100µL
Rabbit Polyclonal ECHS1 antibody. Suitable for IP, WB, IHC-P, ICC/IF and reacts with Human, Mouse samples. Cited in 2 publications. Immunogen corresponding to Recombinant Fragment Protein within Human Enoyl-CoA hydratase, mitochondrial.
Key facts
Host species:Rabbit,
Clonality:Polyclonal,
Isotype:IgG,
Carrier free:No,
Reacts with:Mouse, Human,
Applications:IP, WB, IHC-P, ICC/IFSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:Recombinant Fragment Protein within Human Enoyl-CoA hydratase, mitochondrial. The exact immunogen used to generate this antibody is proprietary information.P30084

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Immunogen, Storage buffer-pH: 7Preservative: 0.01% Thimerosal (merthiolate)Constituents: 20% Glycerol (glycerin, glycerine), 1.21% Tris, 0.75% Glycine, Shipped at conditions-Blue Ice, Appropriate short-term storage duration-1-2 weeks, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze / thaw cycle

Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
ECHS1 Enoyl-CoA Hydratase mitochondrial also known as short-chain enoyl-CoA hydratase 1 is a mitochondrial enzyme involved in fatty acid metabolism. It has a molecular mass of approximately 30 kDa. This protein is expressed predominantly in tissues with high energy demands like heart and skeletal muscle. ECHS1 catalyzes the hydration of enoyl-CoA to 3-hydroxyacyl-CoA playing a significant role in the beta-oxidation pathway of fatty acid breakdown.
Biological function summary
ECHS1 functions as an important enzyme in the mitochondrial fatty acid degradation pathway. It participates as a component of the fatty acid beta-oxidation complex where it collaborates with other enzymes to facilitate energy production through lipids. ECHS1 ensures the conversion of unsaturated fatty acids into metabolites usable in subsequent steps of the energy cycle supporting cellular metabolism.
Pathways
ECHS1 lies within the beta-oxidation pathway of fatty acids important for the energy supply in mitochondria. This pathway directly links to the citric acid cycle (Krebs cycle) where acetyl-CoA produced through beta-oxidation gets further oxidized for energy generation. ECHS1 functions in concert with other enzymes such as Acyl-CoA dehydrogenases that precede its action in the pathway ensuring complete fatty acid degradation.
Defects in ECHS1 activity associate with rare metabolic disorders like short-chain enoyl-CoA hydratase deficiency. This deficiency leads to a build-up of toxic metabolites affecting the nervous system and muscle function. Moreover abnormalities in ECHS1 expression can relate to mitochondrial diseases impacting proteins such as Pyruvate dehydrogenase which further disrupts energy metabolism. Understanding ECHS1's role in these processes has important implications for diagnosing and developing treatments for metabolic conditions.