Isovaleryl-CoA Dehydrogenase (Isovaleryl-Coenzyme A Dehydrogenase)

A mitochondrial flavoprotein, this enzyme catalyzes the oxidation of 3-methylbutanoyl-CoA to 3-methylbut-2-enoyl-CoA using FAD as a cofactor. Defects in the enzyme, is associated with isovaleric acidemia (IVA).

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Networked: 45 relevant articles (1 outcomes, 1 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Vockley, Jerry: 3 articles (09/2021 - 05/2006)
2.	Ensenauer, Regina: 2 articles (03/2018 - 05/2006)
3.	Sakamoto, Osamu: 2 articles (06/2017 - 12/2011)
4.	Vockley, J: 2 articles (03/2000 - 02/2000)
5.	Alodaib, Ahmad N: 1 article (09/2021)
6.	D'Annibale, Olivia M: 1 article (09/2021)
7.	Karunanidhi, Anuradha: 1 article (09/2021)
8.	Kochersperger, Catherine: 1 article (09/2021)
9.	Koppes, Erik A: 1 article (09/2021)
10.	Mohsen, Al-Walid: 1 article (09/2021)

Related Diseases

1.	isovaleric Acidemia 09/01/2023 - "Isovaleric acidemia (IVA) is a rare autosomal-recessive metabolic disorder caused by a genetic deficiency of isovaleryl-CoA dehydrogenase (IVD). " 09/01/2023 - "A Case Report of a Novel Isovaleryl-CoA Dehydrogenase Gene Mutation in a Chinese Family with Isovaleric Acidemia." 09/01/2021 - "Isovaleric acidemia (IVA) is an autosomal recessive inborn error of metabolism caused by deficiency of isovaleryl-CoA dehydrogenase (IVDH) in the Leu catabolism pathway. " 01/16/2021 - "Isovaleric acidemia (IVA) is a rare autosomal recessive inherited organic acidemia caused by a genetic deficiency of isovaleryl-CoA dehydrogenase (IVD). " 02/01/2020 - "Isovaleric acidemia (IVA) is an inborn error of metabolism caused by deficiency of isovaleryl-CoA dehydrogenase. "
2.	Seizures (Absence Seizure) 03/01/2008 - "Interestingly, down-regulation of stress protein mouse stress induced phosphoprotein 1, metabolic enzyme isovaleryl-CoA dehydrogenase, and the two in neuronal axon growth, collapsin response mediator protein 2 and fascin homolog 1 coincides with the results of our previous study on gamma-butyrolactone-induced transient absence seizure. "
3.	Metabolic Diseases (Metabolic Disease) 01/01/2022 - "Isovaleric acidaemia (IVA), characterized by an acute metabolic crisis and psychomotor delay, is a rare inherited metabolic disease caused by a deficiency in isovaleryl-CoA dehydrogenase (IVD). " 11/01/2016 - "Blood and urine screening and isovaleryl-CoA dehydrogenase (IVD) gene detection for inherited metabolic diseases were performed to clarify the etiology. " 07/25/2013 - "Isovaleric acidemia (IVA) is a rare inherited metabolic disease caused by a deficiency in isovaleryl-CoA dehydrogenase (IVD). "
4.	Inborn Genetic Diseases (Disease, Hereditary) 01/01/1971 - "The present concept agrees with our previous findings in isovaleric acidemia, a genetic disorder in which a specific defect of isovaleryl CoA dehydrogenase was observed. "
5.	Metabolic Syndrome (Dysmetabolic Syndrome X) 02/01/2010 - "These data led to the discovery of impaired branched chain amino acid catabolic enzyme isovaleryl-CoA dehydrogenase (encoded by gene At3g45300 or atIVD) as the cause of this metabolic syndrome. "

Related Drugs and Biologics

1.	Tritium
2.	Enzymes
3.	Proteins (Proteins, Gene)
4.	Semaphorin-3A
5.	4-Butyrolactone (gamma Butyrolactone)
6.	Phosphoproteins
7.	Heat-Shock Proteins (Heat-Shock Protein)
8.	fascin
9.	Leucine (L-Leucine)
10.	isovaleryl-coenzyme A