Exam 2 Zaidi Flashcards
1
Q
glucose sensor in liver cells is what
A
glycogen phosphorylase
2
Q
GSD 0
A
glycogen synthase
3
Q
GSD1 = von Gierke disease
A
glucose 6 phosphatase
insufficient release of free glucose into blood by liver
4
Q
GSD2 = pompe disease
A
acid maltase = a- glucosidase
used in lysosomal glycogen degradation pathway
ERT = enzyme replacement therapy (recombinant)
5
Q
GSD 3 = cori disease
A
debranching enzymes = a-1,6-glucosidase activity
6
Q
GSD 4 = andersen disease
A
glucosyl (4:6) transferase
7
Q
GSD 5 = mcardle disease
A
muscle glycogen phosphorylase
more serious than GSD 6
8
Q
GSD 6 = hers disease
A
liver glycogen phosphorylase
9
Q
disorders affecting glycogen breakdown lead to
A
hepatomegaly, hypoglycemia
10
Q
disorders affecting glycogen synthesis lead to
A
patients being dependent on glucose not glycogen
