Metabolism of saccharides Flashcards

1
Q

Galactose

A
  • makes lactose
  • structural component for carbohydrates
  • energy source
  • not insulin dependent
  • metabolism is 2 steps to UDP-galactose
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2
Q

Fructose

A
  • found in sucrose
  • energy source
  • 10% of calories in western diet
  • transported by GLUC-5
  • not insulin dependent
  • does not promote insulin
  • bypasses PFK-1 so metabolizes more rapidly than glucose
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3
Q

Fructokinase

A
  • enzyme used in phosphorylation of fructose
  • only found in liver, kidney, and small mucosal cells in intestine
  • uses ATP
  • IRREVERSIBLE
  • makes 1P
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4
Q

Hexokinase

A
  • enzyme used in phophorylation of fructose(lower affinity than for glucose)
  • acts in all other tissues
  • makes 1-6bisP
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5
Q

Aldolase B

A
  • lots of aldolases can cleave fructose 1-6BisP

- ONLY aldolase B can cleave fructose 1P

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6
Q

Products from Cleavage of fructose 1P

A
  1. DHAP can directly enter glycolysis
  2. Glyceraldehyde: can be phosphorylated to glyceraldehyde 3P and enter glycolysis or gluconeogenesis
  3. glyceraldehyde can also be converted to glycerol of TAG synthesis
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7
Q

Aldose reductase

A
  • found in eyes, schwann cells of peripheral nerves, liver, kidney,ovaries and seminal cells
  • converts glucose to sorbitol
  • does not matter in galactose metabolism, until galactose levels are high
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8
Q

Sorbitol dehydrogenase

A
  • only found in liver(metabolize sorbitol in diet)
  • ovaries and seminal vesicles(energy source)
  • sorbitol to frutose
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9
Q

Conversion of glucose to sorbitol to fructose

A

-tissues that only make aldose reductase, will be damaged by longterm elevation of blood glucose (build up of sorbitol)

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10
Q

Sorbitol accumulation

A
  • sorbitol build up leads to osmotic uptake of water

- cataracts, retinopathy, nephropathy in diabetics

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11
Q

Mannose

A
  • C-2 epimer of glucose
  • very little in diet(mostly synthesized by fructose)
  • important component of glycoproteins
  • substrate for hexokinase
  • substrate for phosphomannoseisomerase to convert is to fructose 6P
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12
Q

Lack of fructokinase

A
  • fructosuria
  • autosoma recessive
  • 1:130,000 births
  • benign condition
  • fructose accumulates in urine
  • just dont eat fructose…
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13
Q

Fructose Poisoning

A
  • autosomal recessive
  • 1:20,000 births
  • lack aldolase B and results in intracellular trapping of fructose 1P
  • hypoglycemia, jaundice, hepatic failure
  • remove sucrose and fructose from diet
  • phosphates can’t make ATP so high AMP leads to hyperuricemia
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14
Q

Galactokinase

A
  • phosphorylation of galactose

- makes galactose 1P

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15
Q

What is the difference between galactose and glucose?

A

-position of hydroxyl group

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16
Q

Galactose 1P uridyltransferase

A

GALT

  • attaches galactose 1P to UDP glucose and frees glucose
  • creates UDP-galactose
  • UDP galactose can be converted into UDP-glucose and enter hlucose metabolic pathways
17
Q

GalactoseKinase deficiency

A
  • rare autosomal recessive disorder
  • elevation of galactose in urine and blood
  • without enzyme, galactose makes galactitol (causes cataracts)
18
Q

GALT deficiency

A
  • autosomal recessive
  • 1:30,000 births
  • prenatal diagnosis
  • ovarian failure
19
Q

Lactose

A
  • synthesized in golgi apparatus

- Enzyme: Lactose Synthase

20
Q

Lactose Syntase

A
  • complex of 2 proteins
    1. Beta-D-galactsyltransferase: found in all kinds of tissues
    2. Alpha-lactalbumin: its synthesis is stimulated by the peptide hormone Prolactin.enzyme is ONLY present when prolactin is there