Enzyme

1.1.1.211 - Long-chain-3-hydroxyacyl-CoA dehydrogenase

Alternative Name(s)

Beta-hydroxyacyl-CoA dehydrogenase.

Catalytic Activity

long-chain (3S)-hydroxy fatty acyl-CoA + NAD(+) = a long-chain 3-oxo-fatty acyl-CoA + H(+) + NADH

Cofactors

There are no Cofactors for this Enzyme

Reaction Mechanism

There are no Reaction Mechanism for this Enzyme

Reaction Parameters

There are no kinetic parameters information for this Enzyme

Associated Proteins

Protein name	Organism
Trifunctional enzyme subunit alpha, mitochondrial	Human
LPS-assembly protein	Lettuce bottom rot fungus
Hydroxyacyl-CoA dehydrogenase, putative	Black-legged tick
Probable beta-hydroxyacyl-CoA dehydrogenase	Azoarcus sp. (strain BH72)
BcDNA.GH12558	Fruit fly

Citations

New Acylcarnitine Ratio as a Reliable Indicator of Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency.
An Autopsy Analysis of a Patient With Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency Caused by Compound Heterozygous HADHA Gene Mutations.
A G1528C Hadha knock-in mouse model recapitulates aspects of human clinical phenotypes for long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency.
Management of pregnancy in a patient with long-chain 3-hydroxyacyl CoA dehydrogenase deficiency.
CHOROIDAL NEOVASCULARIZATION ASSOCIATED WITH LONG-CHAIN 3-HYDROXYACYL-CoA DEHYDROGENASE DEFICIENCY.
Effectiveness of Robotic-Assisted Gait Training and Aquatic Physical Therapy in a Child With Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency: A Case Report.
Outcomes and genotype correlations in patients with mitochondrial trifunctional protein or isolated long chain 3-hydroxyacyl-CoA dehydrogenase deficiency enrolled in the IBEM-IS database.
Paternal uniparental disomy of chromosome 2 resulting in a concurrent presentation of Crigler-Najjar syndrome type I and long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency.
Retained visual function in a subset of patients with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD).
Fatal COVID-19 infection in a patient with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: A case report.
Impact of Newborn Screening and Early Dietary Management on Clinical Outcome of Patients with Long Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency and Medium Chain Acyl-CoA Dehydrogenase Deficiency-A Retrospective Nationwide Study.