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GINME > Week 4: Galactose and Fructose Metabolism > Flashcards

Week 4: Galactose and Fructose Metabolism Flashcards

1
Q

Lactose components

A

Glucose and Galactose

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2
Q

Sucrose components

A

Glucose and Fructose

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3
Q

Fructose transport

A

GLUT 5

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4
Q

GLUT 5 transport substrate

A

Fructose

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5
Q

GLUT 5 Transport type

A

Facilitated diffusion

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6
Q

How does fructose get into the cell

A

fructose -GLUT 5-> into cell

Fructose + ATP -fructokinase-> fructose-1-phosphate + ADP

fructose-1-phosphate -aldolase b-> glyceraldehyde + DHAP

DHAP -> glyceraldehyde-3-phosphate

Glyceraldehyde + Pi -Triose Kinase-> glyceraldehyde-3-phosphate

glyceraldehyde-3-phosphate -> glycolysis

The problem with fructose is that this pathway enters after the PFK-1 step so there is no going back to storing as glycogen

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7
Q

What is fructosemia

A

when there’s too much fructose that it exits back into the blood

gets excreted by the kidneys

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8
Q

Describe hereditary fructose intolerance

A

mutation in aldolase b

Fructose-1-phosphate concentration builds and is osmolyte so it holds a lot of water so water flows into cells as its concentration builds and can cause hepatocyte damage

Also significantly deplete phosphate by trapping it into fructose-1-phosphate

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9
Q

Treatment of hereditary fructose intolerance

A

remove fructose from diet

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10
Q

Symptoms of hereditary fructose intolerance

A

vomiting and diarrhea

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11
Q

Describe galactose metabolism

A

transport by GLUT 2 (concentration dependent)

Galactose -glactose kinase-> galactose-1-phosphate

galactose-1-phosphate + UDP-Glucose - galactose-1-phosphate uridyliltransferase -> glucose-1-phosphate + UDP Galactose

glucose-1-phosphate -phosphoglucomutase-> Glucose-6-phosphate

Glucose-6-phosphate -> glycolysis or glycogen synthesis

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12
Q

How is UDP-glucose regenerated

A

UDP-galactose -eperimase-> UDP-Glucose

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13
Q

Types of galactosemia

A

mild - galactokinase mutation

Severe - galactose-1-phosphate uridylyltransferase mutation

extremely rare - eperimase enzyme to convert UDP-galactose to UDP-Glucose

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14
Q

high concentrations of galactose and galactose-1-phosphate causes

A

Cataracts diagnosed in infants

can be processed by aldose reductase into galactital (alcohol form) which is an osmolyte and in the lens cells can cause damage (osmotic pressure increase in the lens causing proteins to precipitate causing cataracts)

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15
Q

Type 2 galactosemia

A

galactokinase mutation

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16
Q

Classical galactosemia

A

AKA Type 1 Galactosemia

mutation in galactose-1-phosphate uridylyltransferase

GINME (63 decks)

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