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What is HMG CoA lyase deficiency?

What is HMG CoA lyase deficiency?

HMG CoA lyase deficiency is an inherited disorder in which the body cannot process a protein called leucine or make ketones. Ketones are used for energy during periods of fasting.

What is a lyase deficiency?

Adenylosuccinate lyase deficiency is a neurological disorder that causes brain dysfunction (encephalopathy) leading to delayed development of mental and movement abilities (psychomotor delay), autistic behaviors that affect communication and social interaction, and seizures.

Where is HMG CoA lyase found?

The HMGCL gene provides instructions for making an enzyme called 3-hydroxymethyl-3-methylglutaryl-CoA lyase (HMG-CoA lyase). This enzyme is found in mitochondria, which are the energy-producing centers inside cells. HMG-CoA lyase plays a critical role in breaking down proteins and fats from the diet.

What does HMG CoA stand for?

β-Hydroxy β-methylglutaryl-CoA (HMG-CoA), also known as 3-hydroxy-3-methylglutaryl-CoA, is an intermediate in the mevalonate and ketogenesis pathways. HMG-CoA is a metabolic intermediate in the metabolism of the branched-chain amino acids, which include leucine, isoleucine, and valine.

What is the function of mevalonic acid?

Mevalonic acid is a precursor in the biosynthetic pathway known as the mevalonate pathway that produces terpenes and steroids. Mevalonic acid is the primary precursor of isopentenyl pyrophosphate (IPP), that is in turn the basis for all terpenoids.

What does a lyase do?

Lyases are the enzymes responsible for catalyzing addition and elimination reactions. Lyase-catalyzed reactions break the bond between a carbon atom and another atom such as oxygen, sulfur, or another carbon atom.

What does CoA mean?

Certificate Of Authenticity
(Certificate Of Authenticity) A document that accompanies software which states that it is an original package from the manufacturer. It generally includes a seal with a difficult-to-copy emblem such as a holographic image.

How does HMG CoA work?

β-Hydroxy β-methylglutaryl-CoA (HMG-CoA) reductase inhibitors, more popularly known as statins, work by reducing the cholesterol levels in the body. HMG-CoA inhibitors interfere with the ability of the body to build cholesterol from dietary fat. Statins bind to the active site of the enzyme and change its structure.

What is the fate of HMG CoA?

Fates of 3-Hydroxy-3-Methylglutaryl CoA. In the cytosol, HMG-CoA is converted into mevalonate. In mitochondria, it is converted into acetyl CoA and acetoacetate. The synthesis of mevalonate is the committed step in cholesterol formation.

What is mevalonic acid made from?

As its name implies, the MVA pathway utilizes mevalonate (Mev) as an important biosynthetic intermediate. This compound is derived from the condensation of two molecules of acetyl coA followed by the condensation of acetoacetyl coA with another molecule of acetyl coA to produce hydroxymethylglutaryl coA (HMG coA) (Fig.