What does alpha keto acid dehydrogenase do?

The mammalian mitochondrial branched-chain α-keto acid dehydrogenase (BCKD) complex catalyzes the rate-limiting step in the catabolism of branched-chain amino acids, i.e., the oxidative decarboxylation of branched-chain α-keto acids derived from leucine, isoleucine, and valine.

What type of enzyme is acyl-CoA Dehydrogenase?

The acyl-CoA dehydrogenases are mitochondrial flavoenzymes involved in fatty acid and amino acid catabolism. The degradation of fatty acids, also known as β-oxidation, is a spiral reaction of four enzymatic steps that takes place in mitochondria and peroxisomes.

What does acyl-CoA dehydrogenase produce?

Oxidation at the β-carbon of the fatty acid occurs with reduction of FAD (creates a trans double bond) at the Δ2 position to produce Δ2-trans-enoyl-CoA. The electrons from FADH2 are subsequently transferred to ubiquinone in the electron transport chain.

What does the Bckdha gene do?

The BCKD enzyme complex is responsible for one step in the normal breakdown of three protein building blocks (amino acids). These amino acids—leucine, isoleucine, and valine—are obtained from the diet. They are present in many kinds of food, particularly protein-rich foods such as milk, meat, and eggs.

What is the role of thiamine in citric acid cycle?

Thiamine, as thiamine pyrophosphate (TPP), is an important coenzyme for essential steps in the Krebs cycle, including decarboxylation of pyruvate and oxidation of alpha-ketoglutamic acid; thiamine therefore provides an important link between the glycolytic and citric acid cycles.

What are the substrates of pyruvate dehydrogenase?

Principles in the Regulation of Cardiac Metabolism Pyruvate dehydrogenase (PDH) catalyzes an irreversible and no return metabolic step because its substrate pyruvate is gluconeogenic or anaplerotic, whereas its product acetyl-CoA is not [62–65].

What is MCAT disease?

Medium-chain ketoacyl-CoA thiolase deficiency (MCAT) is a condition in which the body is unable to breakdown certain fats. It is considered a fatty acid oxidation condition because people affected by MCAT are unable to change some of the fats they eat into energy the body needs to function.

How is acyl-CoA formed?

Acetyl-CoA is generated either by oxidative decarboxylation of pyruvate from glycolysis, which occurs in mitochondrial matrix, by oxidation of long-chain fatty acids, or by oxidative degradation of certain amino acids.

Is alpha-ketoglutarate A dehydrogenase?

Alpha-ketoglutarate dehydrogenase (α-KGDH) is a highly regulated enzyme, which could determine the metabolic flux through the Krebs cycle. It catalyses the conversion of α-ketoglutarate to succinyl-CoA and produces NADH directly providing electrons for the respiratory chain.

What is the role of α-ketoglutarate?

Alpha-ketoglutarate (AKG) is a key molecule in the Krebs cycle determining the overall rate of the citric acid cycle of the organism. It is a nitrogen scavenger and a source of glutamate and glutamine that stimulates protein synthesis and inhibits protein degradation in muscles.

What enzyme uses thiamine as a cofactor?

Thiamin (Vitamin B1) Thiamin pyrophosphate is a cofactor for a number of enzymes such as transketolase, pyruvate dehydrogenase and α-ketoglutarate dehydrogenase.

Which enzyme is the gateway to the TCA cycle?

Citrate synthase
Citrate synthase is a marker enzyme of the TCA cycle, at the gateway into the cycle from pyruvate via acetyl-CoA.

What are the three enzymes in the pyruvate dehydrogenase complex?

The PDCs in prokaryotes and eukaryotes are composed of multiple copies of three catalytic enzymes: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2), and dihydrolipoamide dehydrogenase (E3) (Fig.