Amino Acid Met 2 Flashcards
Can carbon from Acetyl CoA be used in gluconeogenesis?
No
Which are the only two pure ketogenic AA?
Leu, Lys
Where are BCAAs predominantly used?
Brain and muscle
What are the three BCAAs, and are they gluto or ketogenic?
Val-glutogenic
Ile-both
Leu-keto
What two enzymes are involved in BCAA metabolism?
Transaminase that needs B6, and BC a-ketoacid dehydrogenase
What disease has lethargy, dark sweet urine? Cause?
Maple syrup urine disease. Issue with BC a-ketoacid dehydrogenase
When Val and Ile are changed to propionyl CoA, they are further made into what? By what enzymes?
Methylmalonyl CoA by propionyl CoA Carboxylase. Then succinyl CoA by mutase needing b12
What does pernicious anemia cause?
B12 lack. Which causes folate lack
Phenylalanine is metsbolised to what AA? What cofactor is needed?
Tyrosine. THB
What are the symptoms of phenylketonuria? What is secondary phenylketonuria cause?
High phenylalanine and phenyl pyruvate, low tyrosine and melanin. Secondary caused by bad reduction of cofactor
Thyroxine is made from what AA? And what is its importance?
Tyrosine. Needs a digestion by proteolysis to be released.
What is a defect in tyrosinase called?
Albinism
Homogentistic acid is a midpoint in tyrosine metabolism. What enzyme breaks it down, and what disease results from enzyme issue? Symptoms?
Homogentisate oxidase. Alcaptonuria. Dark urine in air
Methionine metabolism makes what methyl donor?
SAM
Homocysteine needs what vitamin and enzyme to be made into cystathionine?
Cystathionine B synthase and B6
