GLycogen storage diseases Flashcards Preview

Biochemistry MBS > GLycogen storage diseases > Flashcards

Flashcards in GLycogen storage diseases Deck (7):
1

type 1

-glucose 6 phosphotase
-liver and kidney
-increased amount of normal glycogen
-severe hypoglycemia
-hepatomegaly
-Von Gierkes

2

type 2

-pompe
-alpha 1,4 glucosidase
-lysosomal
-effects the whole body
-large increase in normal structure glycogen
-cardiorespiratory failure

3

type 3

-cori
-debranching enzym
-left with dextrins
-muscle/liver
-increased amount of short outer branches
-like type 1 but milder

4

type 4

-andersen
-branching enzyme
-liver
-normal amount, long outer branches
-hepatomegaly, fatal

5

type 5

-mcArdle
-phosphorylase
-muscle
-increased amount of normal structure
-exercise problems
-cramps

6

type 6

-hers
-phosphorylase
-liver
-increased amount of normal structure
-like type 1, milder

7

type 7

-tauris
-PFK1
-muscle
-increased amount, normal structure
-like type 5 (exercise problems, cramps)