TBL1: AA and Skeletal Muscle Metabolism Flashcards
How does peripheral tissue send N to liver?
As Ala and Gln using transamination and aminotransferases
How does the liver send N to kidney and some to intestines?
Urea cycle
Arginine Catabolism
- can enter TCA and nitrogen donated to urea cycle
Arg anabolism
- proteins
- Creatine (skeletal muscle metabolism)
- NO signaling (cGMP)
Starting material for Creatine
Arginine and glycine
cGMP signaling
mnemonic
BAD GraMPa
BNP, ANP, EDRF (NO)
Aminoacidemia
- caused by deficiency of enzyme or transporter involved in AA metabolism
- dietary management
Organic Acidemia
- a group of AA disorders causing a buildup of acids which are usually not present in blood
Maple Syrup Urine Disease
B12 Deficiency (Initial)
B12 Deficiency (Prolonged)
B12 is a cofactor for
methionine synthase
Methylmalonyl-CoA Acidemia
Propionyl-CoA Carboxylase deficiency
isovaleric acidemia
Cystathionine beta synthase deficiency (Homocystinuria)
Which amino acid carries nitrogen in circulation?
Glutamine
Nitrogen waste (excess ammonia) from skeletal muscle amino acid catabolism is diverted to the liver by which amino acid?
Alanine
When not needed, peripheral tissue send nitrogen to liver using which enzyme?
aminotransferase
In propionyl-CoA carboxylase deficiency which substance should have reduced consumption?
In propionyl-CoA carboxylase deficiency which of the following substances should have reduced consumption?
