Glycogen Flashcards

1
Q

Outline the flow of aqueous humor

A
  • formed in capillary bed of ciliary body
  • –> posterior chamber —>through angle formed by lens and iris diaphragm —>Anterior chamber —>absorped by canal of Schlemm
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2
Q

What is the MOA of metoclopramide

A
  • Antagonist at D2 receptor
  • Serotonin agonist
  • Increase contractility in GI tract
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3
Q

What is the rate limiter in glycogenesis?

A

Glycogen synthase

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4
Q

What linkages does glycogen synthase make

A

alpha-1,4 glycosidic

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5
Q

What linkages does Branching enzyme make

A

alpha-1,6 glycosidic

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6
Q

Describe McArdle Disease (type V)

A
  • Glycogen phosphorylase deficiency
  • Rhabdomyolysis
  • Myoglobinuria
  • Renal failure
  • Normal life expectency
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7
Q

Describe Von Gierke Disease (type I)

A
  • Glucose-6-phosphatase deficiency
  • muscles don’t have this anyway . . problem with liver, kidney, and enterocytes
  • SEVERE FASTING HYPOGLYCEMIA
  • Glycogen buildup in liver
  • Hepatosplenomegaly
  • Enlargement of kidneys
  • Increase serum lactate
  • Increase uric acid
  • hypertriglyceridemia
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8
Q

Treatment for Von Gierke Disease

A
  • Feed frequently

- Give corn starch at night

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9
Q

Describe Cori Disease (type III)

A
  • Deficiency of Debranching enzyme
  • Milder form of Von Gierke disease
  • Milder hypoglycemia
  • no elevation of lactate
  • No elevation in uric acid
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10
Q

Pompe Disease (type II) is a deficiency in what

A

-alpha-1,4-glucosidase in lysosomes

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11
Q

What are the two forms of Pompe disease

A
  • infantile

- Adult

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12
Q

Describe infantile Pompe disease

A
  • More severe
  • Severe muscle weakness
  • Cardiomegaly and heart failure
  • Shortened life expectancy
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13
Q

Describe adult form Pompe Disease

A
  • Same enzyme activity but less than normal
  • No cardiac involvement
  • Gradual onset of skeletal muscle weakness
  • Diaphragm weakness
  • Respiratory failure
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14
Q

Describe the role of Debranching enzyme

A
  • breaks apart alpha-1,6-glycosidic linkages
  • also takes 3 of last 4 glucoses before the branch point and puts them on the end (4-alpha-D-glucanotransferase activity)
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