Glycogenesis

Question 1

Glycogen

Answer 1

It is a storage form of glucose that are linked by 1,4 linear and 1,6 branched glycosidic bonds

Question 2

Non-reducing end

Answer 2

terminal glucose ends with free hydroxyl where additional glucose monomers are added. Extended/trimmed here.

Question 3

Reducing ends

Answer 3

The end that nothing is added but connected to glycogenin (primer for glycogen synthesis)

Question 4

Liver glycogen

Answer 4

Distributor that regulates blood glucose. If blood glucose, liver glycogen comes to rescue

Question 5

Muscle glycogen

Answer 5

Provides glucose for physical exercise. Selfish.

Question 6

Glycogen storage disease are ____

Answer 6

Autosomal recessive

Question 7

Glycogen storage disorder diseases that effect breakdown

Answer 7

hepatomegaly because storage of glycogen

Hypoglycemia because cant maintain blood sugar

Question 8

Glycogen storage disorder diseases that effect synthesis

Answer 8

Pts rely heavily on glucose

Question 9

GSD 0

Answer 9

Glycogen synthase deficiency

Need to eat freq.,muscle cramps due to no glycogen

Question 10

GSD1a or Von Gierke dx

Answer 10

Glucose-6-phosphatase deficiency

Cant release free glucose

Question 11

GSD II or Pompe Disease

Answer 11

deficiency in Acid Maltase or acid glucosidase

Glycogen build up in lysosomes

Question 12

GSD III or Cori Disease

Answer 12

Deficiency in 1,6 glucosidase (debranching enzyme)

Large glycogen molecules with short branches

Question 13

GSD IV or Andersen Dx

Answer 13

deficiency in glucosyl (4:6) transferase (branching enzyme)

Cirrhosis

Question 14

GS V or McArdle Disease

Answer 14

deficiency in muscle glycogen phosphorylase

Cant supply muscles with glucose

Question 15

GSD VI or Hers Disease

Answer 15

deficiency in liver glycogen phorsphorylase

Hepatomegaly