Flashcards in Amino acid catabolism Deck (31):
Which amino acids are ketogenic?
L and K
Which amino acids are broken down into OAA?
Which amino acids are broken down into Fumarate?
W Y F
Which amino acids are broken down into succinyl-coa?
V I T M
Which amino acids can be broken down into aKG?
R H G P
Which amino acids are both ketogenic and glucogenic?
T W I F Y
What reaction does glutamate dehydrogenase catalyze? Why is this important?
the reaction from E to aKG. This is important since aKG is needed as an ammonium ion acceptor for aminotransferases.
What is glutamate dehyrdorgenase regulated by?
It is inhibited by GTP and NADH
It is activated by ADP
What is familial hyperinsulinemic hypoglycemia (HHF6)?
Hypoglycemiaand hyperammonemia after high protein meals due to caused by an insensitivity of Glutamate dehydro to GTP. Also reduces N-acetylglutamate synthesis (activator of urea cycle)
Which amino acids can be broken down to make pyruvate?
What is Glyoxalate formed from? Go to?
Formed from G, goes to Oxalate
What happens to Oxalate? What does it lead to?
It is a garbage molecule excreted through kidneys. Attaches to Ca, causes renal stones
G is cleaved in the mitochondira by what important carbon acceptor?
N gets its ammonium from where?
Q gets its ammonium from where?
Biotin is needed for which reaction?
Propionyl-CoA to Succinyl-CoA
Defects in Propionyl-Coa caboxylase will result in what condition?
Defects in racemase in the propionyl-CoA rxn will result in what condition?
Defects in mutase in the propionyl-CoA will result in what condition?
L-Methylmalonic acid uria
What protein binds to B12 in the intestine?
What transports the intrinsic factor-B12 across the intestines?
Pernicious anemia is the result of what?
Practical or actual B12 deficiency
Megaloblastic anemia is the result of what? Symptoms?
Deficiency of Vit B12 and no Homocystiene to M reaction.
Prevents DNA replication
Why does demyelination occur in B12 deficiency?
Impairment of the Methylmalonyl-CoA reaction (used to think, now different)
Branched chain V is turned to what?
alpha ketoisovalerate, then to propionyl-Coa
Branch chain Isoleucine is turned to what?
Branched chain Leucine is converted to what?
Tyrosenemia II is a defect in what enzyme? Symptoms?
Keratitis, photophobia, skin leisions
Alkaptonuria is a defect in what enzyme? Symptoms?
Homogentisate oxidase. Black urine, Ochronosis
What is the treatment for someone with Alkaptonuria?
lower F and Y levels, and admin Nitisione (inhibits p-hydoxyphenylpyruvate)