Carbohydrate Metabolism
Dietary carbohydrates Monosaccharides
Disaccharides
Disaccharides
Polysaccharides -1, 4 -1, 6 -1, 4
Carbohydrate digestion
Carbohydrate digestion
Lactose intolerance
Carbohydrate transport
The glucose transporter family members
Glucose intracellular metabolism Glucose-6 phosphate Glycolysis Pentose phosphate pathway Glycogen
Glycolysis
Aerobic and anaerobic conditions
1
2
3 – rate-limiting
4
5
6
7
8
9
10
11
Overall: aerobic Glucose + 2 NAD+ + 2 ADP + 2 Pi 2 pyruvate + 2 NADH + 2 H + + 2 ATP + 2 H 2 O anaerobic Glucose + 2 ADP + 2 Pi 2 lactate + 2 ATP + 2 H 2 O
Glycolysis
Regulation of glycolisis Enzyme Hexokinase Phosphofructokinase Pyruvate kinase Activator AMP/ADP, Fructose-2, 6 -bisphosphate AMP/ADP, Fructose-1, 6 -bisphosphate Inhibitor Glucose-6 -phosphate ATP, Citrate ATP, Acetyl Co. A, Alanine
Violation of glycolysis Enzyme deficiency Glucokinase Hexokinase Muscle phosphofructokin ase Pyruvate kinase Symptoms Hyperglycemia Increased lipogenesis in adiposities Overweight Low concentration of 2, 3 biphosphoglycerate Increased affinity to oxygen Abnormal oxygen supply Exercise intolerance Hemolytic anemia provoked by oxidants
Fructose metabolism aldolase B
Fructose metabolism in the liver
Fructose metabolism in muscles, adipose tissue, kidney Fructo se-6 Fructo phosp se-1, 6 hate bisph ospha Glycol te ysis
Fructose intolerance
Ø Fructosemia Ø Fructosuria Ø Problems after eating fruits and fructose/sucrosecontaining foods Ø Vomiting Ø Jaundice Ø Excessive sleepiness Ø Convulsions 38
2 3 Galactose metabolism 1
Galactosemia Enzyme Name Type I Galactose-1 -phosphate Classic galactosemia uridyl transferase Type II Galactokinase deficiency Type II UDP galactose Galactose epimerase deficiency ØAutosomal recessive ØCaused by the deficiency in an enzyme responsible for adequate galactose metabolism ØToxic levels of galactose-1 -phosphate result in hepatomegaly, cirrhosis, brain damage, cataracts, renal failure, hypoglycemia. ØWithout treatment, mortality in infants with galactosemia is about 40 75%.
Organs affected by galactosemia
Gerty Theresa Radnitz Cori (1896 -1957) Carl Ferdinand Cori (1896 -1984)
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