Other

Question 1

Alpha oxidation is used for

Answer 1

Phytanic acid

Question 2

Alpha oxidation deficiency

Answer 2

Refsum disease

Question 3

Omega oxidation

Answer 3

Unregulated in deficiencies of fatty acid oxidation

Question 4

Hers diseases

Answer 4

Liver glycogen phosphorylase defiency
Mild fasting hypoglycemia
Liver and leukocytes affected

Question 5

Mcardle syndrome

Answer 5

Muscle glycogen phosphorylase defiency
High levels of glycogen in muscle
Weakness or cramping in muscle after exercise

Question 6

Cori disease

Answer 6

Defiency in debranching enzyme
4 or 1 glucose glycogen branches
Fasting hypoglycemia

Question 7

Pompe disease

Answer 7

Lysosomal alpha glucosidase defiency
Excessive amounts of glycogen
Cardiomegaly and abnormal vacuoles in lysosomes

Question 8

Von Gierke disease

Answer 8

Glucose 6-phosphatase or glucose 6-P translocase
Increased stored glycogen
Fasting hypoglycemia, fatty liver, Hepato and renomegaly
Liver and kidney affected