Glycogen

Question 1

Glycogen is most abundant in muscle and liver.

Answer 1

True

Question 2

Glycogen is a storage form of glucose.

Answer 2

True
Polymer of glucose.

Question 3

Glycogen phosphorylase removes glucose from glycogen polymer.

Answer 3

True

Question 4

Enzyme that breakdown Glycogen?

Answer 4

Glycogen Phosphorylase

Question 5

What is the influence of Glucagon and Epinephrine at Glycogen?

Answer 5

They favor the breakdown of glycogen in glucose.

Question 6

Insuline - High blood sugar

Answer 6

Favors synthesys of glycogen from glucose.

Question 7

Is there a way to glycogen breakdown in the absence of hormones?

Answer 7

MUSCLE CONTRACTION
Calcium/calmodulin activate GP Kinase A which will phosphorylate glycogen phosohorylase - glycogen breakdown;

Question 8

High ATP (WORD) Glycogen Phosphorylase

Answer 8

Inhibits

Question 9

Glucose 6-P (word) Glycogen synthase

Answer 9

Stimulates

Question 10

AMP (WORD) Glycogen Phosphorylase

Answer 10

Stimulates

Question 11

Charactheristics of glycogen storage diseases:

Answer 11

Most autosomal recessive;
Defective breakdown of glycogen;
Liver: hypoglycemia;
Muscle: Weakness;
More than 14 described.

Question 12

Pompe’s Disease other name:

Answer 12

Glycogen Storage Disease type II

Question 13

Acid alpha-glucosidase deficiency is also known as…

Answer 13

Pompe Disease
Accumulation of glycogen in lysosomes

Question 14

Pompe’s Disease signals:

Answer 14

Enlarged Muscles
- Cardiomegaly;
- Enlarged tongue;
Hypotonia
Liver enlargement;
No hypoglycemia;
Death from heart failure

Question 15

Fasting hypoglycemia with ketosis causing weakness and lethargy in the morning is???

Answer 15

Glycogen Synthase deficiency

Question 16

FASTING HYPOGLYCEMIA + POST PRANDIAL HYPERGLYCEMIA REPRESENTS DEFICIENCY OF WHICH ENZYME?

Answer 16

Glycogen Synthase deficiency

Question 17

Fasting hypoglycemia occurs in GSDIII because the liver is ineffective at producing glucose from glycogen in absence of the debranching enzyme.

Answer 17

TRUE
Cori disease
Glycogen Storage Disease type III

Question 18

Glycogen storage disease type I (GSDI; von Gierke disease) is caused by a deficiency of the enzyme glucose-6-phosphatase. As a result, the liver cannot metabolize glycogen into glucose. Glycogen accumulates in the liver causing hepatomegaly. Fasting hypoglycemia with ketosis and lactic acidosis occurs due to inability to breakdown glycogen in the fasting state.

Answer 18

Vongierke Disease

To achieve these goals, frequent small meals are advised. Patients with GSDI often have to wake during the night to eat, one of many difficulties in living with the disorder. Foods containing complex carbohydrates are also recommended. These are slowly absorbed and metabolized into glucose allowing for longer periods of time between meals. Cornstarch is a mainstay of therapy for this reason (high-yield fact!). Other starch-containing foods like potatoes or rice are also recommended.

Question 19

The alanine cycle converts the amino acid alanine into glucose in the liver. This is disrupted in GSDI and, therefore, elevated serum alanine levels occur.

Answer 19

TRUE

Glycogen Storage Disease type I
VOn Gierke disease

Question 20

Myophosphorylase deficiency (McArdle disease) is a disorder, that myocytes cannot break down glycogen which leads to exercise intolerance, myalgias, and weakness. Muscle damage with exercise (rhabdomyolysis) may occur leading to myoglobinuria, dark urine, and an elevated creatine kinase level.

Answer 20

True

Question 21

Von Gierke’s Disease has deficiency of which enzyme?

Answer 21

Glucose 6 phosphatase deficiency

Question 22

Von Gierkes Disease signals:

Answer 22

Severe hypoglycemia between meals;
Lethargy, Seizures, Lactic acidosis;
Enlarged liver (excesses of glycogen);

Question 23

Treatment of Von Gierkes Disease:

Answer 23

Cornstarch + avoid sucrose lactose, fructose, galactose;

Question 24

Cori’s disease also known as…

Answer 24

Glycogen Diseas Type III

Question 25

Debranchung enzyme deficiency is cause of…

Answer 25

Coris Disease

Question 26

Gluconeogenesis is intact in Coris Disease.

Answer 26

TRUE

Question 27

Classic Presentation of Cori’s disease:

Answer 27

• Infant or child with hypoglycemia/hepatomegaly
• Hypotonia/weakness
• Possible cardiomyopathy with hypertrophy

Question 28

McArdle’s Disease also known as…

Answer 28

Glycogen Storage Disease Type V