Abcam, ab243509, Anti-IVD antibody

Size: 100µL
Rabbit Polyclonal IVD antibody. Suitable for IHC-P, WB, ICC/IF and reacts with Human samples. Cited in 1 publication. Immunogen corresponding to Recombinant Fragment Protein within Human IVD aa 1-150.
Key facts
Host species:Rabbit,
Clonality:Polyclonal,
Isotype:IgG,
Carrier free:No,
Reacts with:Human,
Applications:IHC-P, WB, ICC/IFSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:Recombinant Fragment Protein within Human IVD aa 1-150. The exact immunogen used to generate this antibody is proprietary information.P26440

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Immunogen, Storage buffer-pH: 7.2Preservative: 0.02% Sodium azideConstituents: PBS, 40% Glycerol (glycerin, glycerine), 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.
The target IVD also known as isovaleryl-CoA dehydrogenase is an enzyme weighing approximately 49 kDa. It is a mitochondrial matrix enzyme where it is primarily expressed. IVD facilitates the third step of the leucine catabolic pathway by catalyzing the dehydrogenation of isovaleryl-CoA to 3-methylcrotonyl-CoA. This protein plays a critical role in the breakdown of leucine an essential amino acid contributing to energy production and metabolic processes.
Biological function summary
Isovaleryl-CoA dehydrogenase acts as an important enzyme in branched-chain amino acid metabolism. It is part of a complex enzyme system involved in leucine degradation. As such it contributes to maintaining energy homeostasis by converting leucine into acetoacetate and acetyl-CoA important components of energy production and lipid synthesis. IVD's functional activity supports metabolic processes and regulates amino acid levels in cells.
Pathways
Isovaleryl-CoA dehydrogenase participates in the leucine degradation pathway and the wider branched-chain amino acid degradation pathway. This pathway integrates with other metabolic pathways such as the tricarboxylic acid (TCA) cycle and the ketone body synthesis pathway. Related proteins in this context include enzymes such as 3-methylcrotonyl-CoA carboxylase and HMG-CoA reductase which also play roles in the metabolism of leucine and ketone body formation.
Disruptions in the activity of isovaleryl-CoA dehydrogenase can lead to isovaleric acidemia a metabolic disorder characterized by the inability to properly process leucine. This condition results in the accumulation of toxic metabolites leading to severe neurological deficits and metabolic crises. The protein is also linked to propionic acidemia where deficiencies in related enzymes like propionyl-CoA carboxylase or methylmalonyl-CoA mutase exacerbate symptoms highlighting the interconnected nature of metabolic pathways and the critical role of IVD.