TBL1: AA and Skeletal Muscle Metabolism

Question 1

How does peripheral tissue send N to liver?

Answer 1

As Ala and Gln using transamination and aminotransferases

Question 2

How does the liver send N to kidney and some to intestines?

Answer 2

Urea cycle

Question 3

Arginine Catabolism

Answer 3

• can enter TCA and nitrogen donated to urea cycle

Question 4

Arg anabolism

Answer 4

• proteins
• Creatine (skeletal muscle metabolism)
• NO signaling (cGMP)

Question 5

Starting material for Creatine

Answer 5

Arginine and glycine

Question 6

cGMP signaling

mnemonic

Answer 6

BAD GraMPa
BNP, ANP, EDRF (NO)

Question 7

Aminoacidemia

Answer 7

• caused by deficiency of enzyme or transporter involved in AA metabolism
• dietary management

Question 8

Organic Acidemia

Answer 8

• a group of AA disorders causing a buildup of acids which are usually not present in blood

Question 9

Maple Syrup Urine Disease

Question 10

B12 Deficiency (Initial)

Question 11

B12 Deficiency (Prolonged)

Question 12

B12 is a cofactor for

Answer 12

methionine synthase

Question 13

Methylmalonyl-CoA Acidemia

Question 14

Propionyl-CoA Carboxylase deficiency

Question 15

isovaleric acidemia

Question 16

Cystathionine beta synthase deficiency (Homocystinuria)

Question 17

Which amino acid carries nitrogen in circulation?

Answer 17

Glutamine

Question 18

Nitrogen waste (excess ammonia) from skeletal muscle amino acid catabolism is diverted to the liver by which amino acid?

Answer 18

Alanine

Question 19

When not needed, peripheral tissue send nitrogen to liver using which enzyme?

Answer 19

aminotransferase

Question 20

In propionyl-CoA carboxylase deficiency which substance should have reduced consumption?

Answer 20

In propionyl-CoA carboxylase deficiency which of the following substances should have reduced consumption?