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CBFM Final > Glycogen > Flashcards

Glycogen Flashcards

1
Q

Basic function of glycogen

A

Storage for of glucose

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2
Q

Where is abundant

A

Muscle and liver

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3
Q

How does muscle use glycogen

A

For itself as energy!

Prolonged exercises

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4
Q

How does the liver use glycogen

A

For others, to maintain blood glucose when needed

During fasting

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5
Q

1, 4 linkage is ____

A

Straight

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6
Q

1,6 linkage is ____

A

Branching

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7
Q

Branching of glycogen

A

Makes it more soluble

Store more glycogen
increases rate of glycogen synthesis and breakdown

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8
Q

Glycogen synthesis can be done from

A

Preexiting glycogen molecule
Or
Glycogenin center

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9
Q

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

A

Glycogenin

Tyrosine

Reducing

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10
Q

From G6P what can happen

A

Glycolysis
Or
Made to Glucose
Or
PPP

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11
Q

Muscles do not have ____ so what is inevitable

A

No glucose 6 phosphatase

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

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12
Q

____ is a high energy form of glucose

A

UDP glucose

Very reactive

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13
Q

Glycogen synthase makes what kind of links

A

1,4

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14
Q

Branching enzyme is aka and makes what links

A

4:6 transferase

1,6 links

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15
Q

What is elevated in fasting or fight or flight

A

Glucagon
Epinephrine

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16
Q

Glucagon and epinephrine favor

A

Glycogen to glucose

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17
Q

Insulin favors

A

Glucose to glycogen

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18
Q

What happens when glycogen phosphorylase gets phosphorylated

A

It turns more glycogen to glucose

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19
Q

What do glucagon and epinephrine want to happen

A

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

20
Q

What happens when glycogen synthase gets phosphorylated

A

It’s activity stops so it stops making glycogen

21
Q

Insulin wants ___ of glyc phosphorylase and glycogen synthase

A

Dephosphorylation

22
Q

What does Dephosphorylation of glycogen synthase do

A

Makes it more active. Make it want make more glycogen

23
Q

What does Dephosphorylation do to glycogen phosphorylase

A

Lowers activity so it will stop turning glycogen to glucose

24
Q

What does calcium/calmodulin do

A

Immediately breaks down glycogen by

Activate GPkinase A which phosphorylates glycogen phosphorylase

Which leads to glycogen break down

25
Q

Defective breakdown of glycogen in liver vs muscle

A

L= hypoglycemia
M = weakness

26
Q

Glycogen storage disease type 1 aka

Which enzyme

A

Von Gierke’s disease

G6phosphatase deficiency

27
Q

Von gierkes enzyme does what normally

A

In the liver only and it turns G6P to glucose

28
Q

Logically describe von gierke’s

A

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

29
Q

With von gierkes who will be ok

A

Muscles

It’s a liver enzyme

30
Q

Type 2 aka and enzyme

A

Pompe

Lysosomal α1,4 glucosidase

If you spell it like the volcano
POMPEII
It has a 2 at the end

31
Q

What happens to glycogen in type 2 deficiency

A

It accumulates in lysosomes

32
Q

Clinical features of pompe disease

A

Cardiomegaly, muscle weakness, death by 2 yrs old

33
Q

Pompe wont have

A

Hypoglycemia in liver

34
Q

Enlarged tongue, hypotonia, no metabolic problems

A

Pompe disease

35
Q

Cori’s disease is type

A

III

36
Q

Type III is a disease of

A

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

37
Q

Mild hypoglycemia in Cori’s disease because

A

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

38
Q

Cori’s enzyme effects ___ mainly

A

Muscles

39
Q

Cori’s disease key point

A

Gluconeogenesis is intact

40
Q

Which disease has a HUGE round Santa belly

A

Type I

41
Q

Von gierke’s vs Cori

A

Muscle involvement apart of Cori’s

Weak muscles

42
Q

Type V disease aka

A

McArdle’s disease
McDonald’s 5 dollar meal

43
Q

McArdles deficient enzyme is

A

MUSCLE glycogen phosphorylase

44
Q

Symptoms of McArdles

A

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

45
Q

type VI glycogen disease aka and enzyme

A

Hers

HEPATIC Glycogen phosphorylase

46
Q

Glycogen diseases are _____

A

Inherited genetic disorders

47
Q

If liver is affected, _____ is common with hepatomegaly

A

Hypoglycemia

CBFM Final (18 decks)

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