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Qu'est-ce (qui) est 2-hydroxyphytanoyl-CoA lyase - définition
2-hydroxyphytanoyl-CoA lyase
CLASS OF ENZYMES
2-Hydroxyphytanoyl-CoA lyase
2-Hydroxyphytanoyl-CoA lyase is a peroxisomal enzyme involved in the catabolism of phytanoic acid by α-oxidation. It requires thiamine diphosphate (ThDP) as cofactor.
3-Hydroxy-3-methylglutaryl-CoA lyase
CLASS OF ENZYMES
Hydroxymethylglutaryl-CoA lyase; HMG-CoA lyase; EC 4.1.3.4; (S)-3-hydroxy-3-methylglutaryl-CoA acetoacetate-lyase (acetyl-CoA-forming); HMGCL; 3-hydroxy-3-methylglutaryl-CoA lyase
3-Hydroxy-3-methylglutaryl-CoA lyase (or HMG-CoA lyase) is an enzyme ( that in human is encoded by the HMGCL gene located on chromosome 1. It is a key enzyme in ketogenesis (ketone body formation).
3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
MEDICAL CONDITION
HMG-CoA lyase deficiency; Hydroxymethylglutaryl lyase deficiency; Hydroxymethylglutaric aciduria; Hydroxymethylglutaryl-CoA lyase deficiency; 3-hydroxy 3-methyl glutaryl-coa lyase deficiency; HMG lyase deficiency; HMG CoA lyase deficiency; Hydroxymethylglutaricaciduria; 3-hydroxy-3-methylglutaryl-CoA lyase deficiency
3-Hydroxy-3-methylglutaryl-CoA lyase deficiency is an uncommon inherited disorder in which the body cannot properly process the amino acid leucine. Additionally, the disorder prevents the body from making ketones, which are used for energy during fasting.
