Enzymes
Intermediates
Galactosemia
100

What does PK do in glycolysis?

Converts PEP to pyruvate and produces ATP

100

Name 2 metabolic pathways G-6-P is involved in

Glycolysis, glucose production, hexosamine pathway, de novo lipogenesis, glycogen synthesis, pentose phosphate pathway

100
Where does galactose metabolism occur?

primarily liver and skeletal muscle

200

What is the common cofactor between PFK and PK?

Magnesium

200

Name 1 fate of pyruvate.

1.) Acetyl CoA Production: (Aerobic)

2.) Lactate Fermentation: (Anaerobic)

3.) Ethanol Fermentation: (Anaerobic)

200

List 2 galactosemia interventions

  • Eliminate lactose and galactose from diet until type is determined

  • Calcium/Vitamin D supplementation

  • In infants:

    • Eliminate breast milk

    • Recommend lactose-free formulas (soy, casein hydrolysate, elemental)

300

Describe the regulation of PFK (inhibition and stimulation)

  • Stimulated: High levels of ADP & AMP (low energy level); fructose 2,6-bisphosphate (hepatocytes)

  • Inhibited: High levels of ATP & citrate (high energy level); low pH in muscles (prevent lactic acid buildup)

300

Why is G-6-P important in the hexosamine pathway?

it produces fructose-6-P

300

Which enzyme is related to type 2 galactosemia?

Galactokinase (GALK)

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