Galactose

Question 1

Galactose pathway enzymes

Answer 1

Galactokinase ➡️ galactose 1 P

Uridyltransferase ➡️ glucose 1 P

Nao possui via alternativa com hexokinase

Question 2

Galactokinase def

Answer 2

⬆️ galactose
Mild symptoms, cataracts infantile
Galatosuria e galactosemia
Autosomal recessive

Question 3

Classic galactosemia

Answer 3

Def uridyltransferase
⬆️Galactose 1 P

Liver failure, hepatomegaly, cataract, e E Coli sepsis

Question 4

Polyol Pathway

Answer 4

Alternative way to put glucose or galactose inside the Cel and goes to fructose pathway

-enzymes:
Aldolase reduxtase ➡️ sorbitol / galactiol
Sorbitol dehydrogenase ➡️ ambos em frutose

• cuidado com excessos de galactose:
  Aldolase reductase consegue trabalhar porem a sorbitol dehydrogenase nao comsegue, assim acumula sorbitol / galactitol inside the cell

Sorbitol and galactitol are osmotic active tissue damage:

Lens - cataracts
Retina - retinopathy
Kidney
Schwann cells peripheral

Tissues with HIGH sorbitol dehydrogenase : liver ovaries and seminal vesicles