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Neuro Week 5 > Glycogen Metabolism/Disorders > Flashcards

Glycogen Metabolism/Disorders Flashcards

1
Q

where is majority of glycogen stored?

A

liver

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2
Q

outside of liver, where else is glycogen?

A

muscle

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3
Q

where in cells does most glycogen reside?

A

in cytoplasm…but can be in lysosome

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4
Q

what is bond that links glucose to each other to form glycogen?

A

a1,4

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5
Q

what is bond that branches glucose in glycogen?

A

a1,6

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6
Q

what is enzyme in charge of linking glucose to form glycogen?

A

glycogen synthase

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7
Q

when does glycogen start being used after feeding?

A

2-3 hours after meal

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8
Q

what does insulin do to glycogen?

A

inhibits breakdown and promotes synthesis of it

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9
Q

what does insulin activate to start glycogen storage?

A

activates glycogen synthase a

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10
Q

what does glucagon do to glycogen?

A

leads to breakdown of it and inhibits synthesis

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11
Q

what enzyme is in charge of taking off glucose from glycogen in glycogen breakdown?

A

glycogen phosphorylase a

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12
Q

what is name of enzyme that transfers branches in glycogen breakdown?

A

debranching enzyme (4-a-glucanotransferaase)

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13
Q

what is name of enzyme that moves the very last branch point glucose to strand of glucose in glycogen break down?

A

debranching enzyme (a1,6glucosiase-

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14
Q

what is name of enzyme in charge of glycogen breakdown in lysosome?

A

a1,4 glucosidase

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15
Q

name the four glycogen storage diseases

A

Von gierke
pompe
cori
Mcardle

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16
Q

type I glycogen storage disease

A

Von gierke

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17
Q

type II glycogen storage disease

A

pompe

18
Q

type III glycogen storage disease

A

Curi

19
Q

type IV glycogen storage disease

A

McArdle

20
Q

what enzyme is deficient in von gierke?

A

glucose-6-phosphatase

21
Q

explain how glucose-6phosphatase deficiency leads to issues in glycogen storage?

A

glucose-6-phosphate needs to be changed into glucose to be transported out of the liver and that is done by the phosphatase..if it isnt changed into glucose then it enters the glycogen storage process instead…so lots of glycogen accumulates

22
Q

is blood sugar low or high in von gierke?

A

hypoglycemia

23
Q

name three things that increase in blood in von gierke

A

lactate, triglycerides and uric acid

24
Q

why do lactate and uric acid increase in von gierke?

A

because the glucose 6 phosphate is shunted down the krebs cycle or into the nucleotide cycle and that increases lactate and uric acid respectively

25
Q

what is another disorder associated with von gierke disease?

A

gout

26
Q

where is glycogen storage increased in von gierke?

A

liver and kidenys

27
Q

what is enzyme issue in pompe disease?

A

a-1,4-glucosidase

28
Q

where is issue in pompe disease?

A

lysosome enzyme

29
Q

name the four main symptoms of pompe disease

A

cardiomegaly, hypertrophic cardiomyopathy, exercise intolerance, hypotonia

30
Q

what is classic appearance of hypotonic baby?

A

floppy bby

31
Q

what is enzyme issue in cori disease?

A

debranching enzymes of glycogen

32
Q

what are the two main symptoms seen in cori disease?

A

hypoglycemia and hepatomegaly

33
Q

what is cardiac issue in cori disease?

A

cardiomyopathy

34
Q

what is issue in McArdle disease/

A

skeletal muscle glycogen phosphorylase

35
Q

where is issue in mcardles disease?

A

skeletal muscle

36
Q

what is main symptom of mcardles disease?

A

myopathy/muscle cramps and myoglobinuria

37
Q

what can be seen in urine in mcardles?

A

myoglobinuria

38
Q

does blood glucose go high or low in mcardles?

A

unchanged usually

39
Q

what disease is second wind phenomenon assoc with? why?

A

mcardle bc of increase in muscular blood flow during exercise

40
Q

how are all the glycogen storage diseases inherited?

A

all are autosomal recessive

Neuro Week 5 (4 decks)

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