2.1 Fructose metabolism Flashcards
What are the two monosaccharides that make up Sucrose
Glucose and Fructose
Key sources of fructose in diet
Fructose
High Fructose Corn Syrup
Sucrose
What is the key difference between High Fructose Corn Syrup and Sucrose
Sucrose is a disaccharide of Glucose and Fructose
HFCS is a mixture of monosaccharides of Glucose and Fructose
Fructose absorption into the intestinal cells occurs how and through what type of transport?
Through GLUT 5
Facilitated Transport
Fructose absorption into the bloodstream from the intestinal cells occurs how and through what type of transport?
GLUT 5 or GLUT 2 (mainly GLUT 2)
Facilitated Transport
In the muscle, how in what structure does fructose enter glycolysis
Fructose –> Fructose 6-phosphate
What enzyme catalyzes the reaction of Fructose to Fructose 6-phosphate in the muscle?
Hexokinase
How does Fructose enter glycolysis in the liver
Fructose –> Fructose 1-phosphate –> Glyceraldehyde and Dihydroxyacetone phosphate –> Glyceraldehyde 3-phosphate
What enzyme catalyzes reaction of fructose to fructose 1-phosphate in the liver
Fructokinase
What enzyme catalyzes Fructose 1-phosphate to Glyceraldehyde and Dihydroxyacetone phosphate
Aldolase B (Fructose 1-phosphate aldolase)
What crucial step of normal glycolysis does Fructose skip as it enters glycolysis in the liver?
It bypasses PFK1
What is the result of Fructose bypassing PFK1 in the liver?
Increased lipogenesis and Increased appetite
Excessive dietary intake of fructose may lead to decrease in what?
available phosphate which means that it is insufficient to meet demands of ATP regeneration
What are the two major genetic disorders of fructose metabolism
Enzyme deficiency with Fructokinase
Enzyme deficiency with Aldolase B
Which genetic disorder of fructose metabolism is severe
Enzyme deficiency with Aldolase B or Hereditary fructose intolerance
What happens when there is an enzyme deficiency with fructokinase in the liver?
Fructose cannot be phosphorylated to fructose 1-phosphate and cannot be trapped in the cell. This could lead to Essential Fructosuria
What is the purpose of converting glucose to sorbitol?
To trap glucose in the cell without expending ATP.
What enzyme catalyzes the reaction of Glucose to Sorbitol?
Aldose Reductase
What coenzyme is used in the reaction of Glucose to Sorbitol?
NADPH
When is there a buildup of Sorbitol?
During hyperglycemia
In what cells does Sorbitol build up and why?
Lens, Kidney and Nerve
Sorbitol cannot be converted to Fructose in these cells
Sorbitol is converted to Fructose by what enzyme in the Liver?
Sorbitol Dehydrogenase
How does Mannose enter glycolysis?
By being converted to Fructose 6-phosphate
Reaction of Mannose to Mannose 6-phosphate is catalyzed by what enzyme?
Hexokinase
Mannose 6-phosphate to Fructose 6-phosphate reaction is catalyzed by what enzyme?
Phosphomannose isomerase
How does Galactose enter glycolysis?
Glucose 6-phosphate
Briefly outline the pathway of Galactose being converted to Glucose 6-phosphate
- Galactose –> Galactose 1-phosphate
- Galactose 1-phosphate + UDP-Glucose –> UDP-Gal + Glu 1-Phosphate
- Glucose 1-phosphate –> Glucose 6-phosphate
- *UDP-galactose –> UDP-glucose (to be used to produce glu-1-P)
What enzyme catalyzes the reaction of Galactose –> Galactose 1-phosphate
Galactokinase
What enzyme catalyzes the placement of uridine phosphate on Galactose 1-phosphate and the production of Glucose-1-phosphate
Galactose 1-phosphate uridylyl transferase
What enzyme catalyzes reaction of UDP-galactose to UDP-glucose?
UDP-Galactose-4-epimerase
What enzyme catalyzes reaction of Glucose 1-phosphate to Glucose 6-phosphate in the galactose metabolism
Phosphoglucomutase
Name the seven tissues that has the Pentose Phosphate Pathway
Mammary Gland Adipose tissue Adrenal Cortex Liver Testes Ovaries Red Blood Cells
What are the two major products of the Pentose Phosphate Pathway
NADPH and Ribose 5-Phosphate
Briefly outline the Oxidative pathway of PPP
Glucose 6-Phosphate –> 6 Phosphogluconolactone
6 Phosphogluconolactone –> 6 Phosphogluconate
6 Phosphogluconate –> Ribulose 5-Phosphate
Enzyme for conversion of 6 Phosphogluconolactone from G6P in oxidative PPP
GLucose 6-phosphate dehydrogenase
Enzyme that catalyzes reaction of 6 Phosphogluconolactone to 6 Phosphogluconate
Lactonase
6 Phosphogluconate to Ribulose 5 Phosphate is catalyzed by what enzyme?
6 Phosphogluconate Dehydrogenase
What enzyme catalyzes reaction of Ribulose 5 phosphate to Ribose 5-Phosphate
Phosphopentose Isomerase
When Ribose 5-Phosphate is needed more than NADPH in Non-Oxidative pathway, outline pathway
(2) Fructose 6-phosphate and (1) Glyceraldehyde 3-Phosphate –> (3) Ribose 5-phosphate
Outline the pathway when more NADPH is needed than Ribose 5-phosphate
(3)Ribose 5-Phosphate is recycled to form (2)Fructose 6-phosphate and (1)Glyceraldehyde 3-Phosphate
What does Glutathione do?
It reduces H202 to make 2 H20 and becomes oxidized in the meantine
When Glutathione is oxidized and needs to be become reduced, what enzyme and coenzyme help accomplish this.
Glutathione Reductase
NADPH
What enzyme is involved in oxidizing Glutathione when it gives up its 2 H ions to H2O2?
Glutathione Peroxidase
What is the effect of a Glucose 6 phosphate dehydrogenase deficiency?
Disrupts the reformation of NADPH that is used to reduce Glutathione. The ability to detoxify oxidizing agents is disrupted.