Fructose

Question 1

Fructose pathway enzymes

Answer 1

Fructokinase ➡️ fructose 1 P

Aldolase B ➡️ glyceraldeido

Hexokinase ➡️ via Alternativa fructose 6 P

Question 2

Essencial fructosuria

Answer 2

Def fructokinase
-Aumento de frutose porem desviase para rota hexlkinase

Autossomal recessive
Dx: urine positive for reducing sugar

Question 3

Hereditary fructose intolerance

Answer 3

Autosomal recessive
Def aldolase B
Acumulo de fructose 1-P leva reducao de P no figado, ⬇️ ATP e ⬇️ dois procedienbtos feitos no figado:
Low gliconeogenese : hypoglicemia e seizures
Low glycongenolises: hepatomegalia