Carbohydrate Metabolism Flashcards
Low Km and Low Vmax; inhibited by G6P
Hexokinase
High Km and high Vmax; inhibited by F6P
Glucokinase
Inhibitors of PK (pyruvate kinase)
ATP, Glucagon, Alanine
Stimulators of PK (pyruvate kinase)
Insulin, F1-6 BP; dephosphorylation
Defects in glycolytic enzymes (such as PK, phosphoglucoisomerase and triosephosphate isomerase) malfunction of ATP pumps, intracellular NA+ concentration increases, cell swells, and bursts
Hemolytic anemia
Malfunction of GLUT 2; Impaired transport of fructose, glucose and galactose; fasting hypoglycemia
Fanconi Bickel
Deficiency in PFK 1 (rate limiting enzyme of glycolysis)
Tauri Disease
Rate limiting enzyme of gluconeogenesis
Fructose 1, 6 Bisphosphatase
Steps in Gluconeogenesis different from glycolysis
Pyruvate-Phosphoenolpyruvate (Pyruvate Carboxylase) and (PEPCK) OAA to PEP
Fructose 1,6 BP-Fructose 6P (Fructose 1,6 Bisphosphatase)
Glucose 6 Phosphate-Glucose (Glucose 6 Phosphatase)
Positive enzymatic regulators of gluconeogenesis
Malate Dehydrogenase; PEPCK, Pyruvate carboxylase, and fructose 1,6 bisphosphatase, glucose 6 phosphatase
Positive regulators of gluconeogenesis
Citrate, cortisol, thyroxine, acetyl coA
Negative regulators of gluconeogenesis
ADP, AMP, Fru 2-6 BP
Fructose 1,6 BP Deficiency
Can’t convert Fructose 1,6 BP to Fructose 6 Phosphate
Hypoglycemia
Lactic acidosis
Ketosis
Hyperventilation after artificial sweeteners
Deficiency in glucose 6 phosphatase
Fasting hypoglycemia
Von Gierke Disease
Fructose Metabolism
Usually used for fatty acid synthesis
Converted to sorbitol (sorbitol dehydrogenase) and to glucose (aldol reductase)-used for glycolysis