Pyruvate dehydrogenase

Dihydrolipoyl transacetylase

Dihydrolipoyl dehydrogenase

Water is polar

Hydrogens are partially positive

Oxygen is partially negative

Caused by mutation or mitochondrial changes

Results in hyperammonemia

NAG analogue carbamoylglutamate restores urea cycle function

Protein-energy malfunction

Inadequate protein intake

Deficiency of nutrients, especially proteins, carbohydrates, and lipids

(Diagram from this study)

Preparatory Phase -- 5 enzyme-catalyzed reactions -- uses 2 ATP molecules -- makes two phosphorylated 3-carbon high-yield molecules -> glyceraldehyde 3-phosphate -> dihydroxyacetone phosphate

Payoff Phase -- 5 enzyme-catalyzed reactions -- makes 4 ATP molecules -- transfers two hydride ions to NAD+ -- this makes two molecules of pyruvate

Overall Reaction Glucose + 2 NAD+ + 2 ATP + 2 Pi -> 2 pyruvate + 2 NADH + 2 H+ + 2 ATP + 2 H2O

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