Glycogen

Question 1

Basic function of glycogen

Answer 1

Storage for of glucose

Question 2

Where is abundant

Answer 2

Muscle and liver

Question 3

How does muscle use glycogen

Answer 3

For itself as energy!

Prolonged exercises

Question 4

How does the liver use glycogen

Answer 4

For others, to maintain blood glucose when needed

During fasting

Question 5

1, 4 linkage is ____

Answer 5

Straight

Question 6

1,6 linkage is ____

Answer 6

Branching

Question 7

Branching of glycogen

Answer 7

Makes it more soluble

Store more glycogen
increases rate of glycogen synthesis and breakdown

Question 8

Glycogen synthesis can be done from

Answer 8

Preexiting glycogen molecule
Or
Glycogenin center

Question 9

_____ forms a glycosidic bond via _____ to a glucose ______ end via autoGlycosylation

Answer 9

Glycogenin

Tyrosine

Reducing

Question 10

From G6P what can happen

Answer 10

Glycolysis
Or
Made to Glucose
Or
PPP

Question 11

Muscles do not have ____ so what is inevitable

Answer 11

No glucose 6 phosphatase

Which means G6P cannot go back to glucose, it must go to glycolysis

Question 12

____ is a high energy form of glucose

Answer 12

UDP glucose

Very reactive

Question 13

Glycogen synthase makes what kind of links

Answer 13

1,4

Question 14

Branching enzyme is aka and makes what links

Answer 14

4:6 transferase

1,6 links

Question 15

What is elevated in fasting or fight or flight

Answer 15

Glucagon
Epinephrine

Question 16

Glucagon and epinephrine favor

Answer 16

Glycogen to glucose

Question 17

Insulin favors

Answer 17

Glucose to glycogen

Question 18

What happens when glycogen phosphorylase gets phosphorylated

Answer 18

It turns more glycogen to glucose

Question 19

What do glucagon and epinephrine want to happen

Answer 19

Want glyc. Phosphorylase to get phosphorylated so it turns on and revs up the gly to glucose production

Question 20

What happens when glycogen synthase gets phosphorylated

Answer 20

It’s activity stops so it stops making glycogen

Question 21

Insulin wants ___ of glyc phosphorylase and glycogen synthase

Answer 21

Dephosphorylation

Question 22

What does Dephosphorylation of glycogen synthase do

Answer 22

Makes it more active. Make it want make more glycogen

Question 23

What does Dephosphorylation do to glycogen phosphorylase

Answer 23

Lowers activity so it will stop turning glycogen to glucose

Question 24

What does calcium/calmodulin do

Answer 24

Immediately breaks down glycogen by

Activate GPkinase A which phosphorylates glycogen phosphorylase

Which leads to glycogen break down

Question 25

Defective breakdown of glycogen in liver vs muscle

Answer 25

L= hypoglycemia M = weakness

Question 26

Glycogen storage disease type 1 aka Which enzyme

Answer 26

Von Gierke’s disease G6phosphatase deficiency

Question 27

Von gierkes enzyme does what normally

Answer 27

In the liver only and it turns G6P to glucose

Question 28

Logically describe von gierke’s

Answer 28

When the G6phosphatase no longer works, that means you cannot turn G6P to glucose Which means you cannot have glucose in the blood So you’ll be tired and you’ll have more lactic acid and excess glycogen (which means a large liver) SEE pic slide 52

Question 29

With von gierkes who will be ok

Answer 29

Muscles It’s a liver enzyme

Question 30

Type 2 aka and enzyme

Answer 30

Pompe Lysosomal α1,4 glucosidase If you spell it like the volcano POMPEII It has a 2 at the end

Question 31

What happens to glycogen in type 2 deficiency

Answer 31

It accumulates in lysosomes

Question 32

Clinical features of pompe disease

Answer 32

Cardiomegaly, muscle weakness, death by 2 yrs old

Question 33

Pompe wont have

Answer 33

Hypoglycemia in liver

Question 34

Enlarged tongue, hypotonia, no metabolic problems

Answer 34

Pompe disease

Question 35

Cori’s disease is type

Answer 35

III

Question 36

Type III is a disease of

Answer 36

The debranching enzyme Can’t break 1,6 to 1,4

Question 37

Mild hypoglycemia in Cori’s disease because

Answer 37

Liver can still make glucose cuz glucose 6 phosphatase is still intact

Question 38

Cori’s enzyme effects ___ mainly

Answer 38

Muscles

Question 39

Cori’s disease key point

Answer 39

Gluconeogenesis is intact

Question 40

Which disease has a HUGE round Santa belly

Answer 40

Type I

Question 41

Von gierke’s vs Cori

Answer 41

Muscle involvement apart of Cori’s Weak muscles

Question 42

Type V disease aka

Answer 42

McArdle’s disease McDonald’s 5 dollar meal

Question 43

McArdles deficient enzyme is

Answer 43

MUSCLE glycogen phosphorylase

Question 44

Symptoms of McArdles

Answer 44

Muscle cramps, weakness on exercise, myoglobinuria , dark pee after exercise

Question 45

type VI glycogen disease aka and enzyme

Answer 45

Hers HEPATIC Glycogen phosphorylase

Question 46

Glycogen diseases are _____

Answer 46

Inherited genetic disorders

Question 47

If liver is affected, _____ is common with hepatomegaly

Answer 47

Hypoglycemia