Individual Amino Acids Metabolism

Question 1

glycine

keto acid and functions

Answer 1

glyoxalate 
SCHGB PDG
Serine Synthesis
Creatine Biosynthesis
Heme Biosynthesis
Glutathione Biosynthesis
Bile Salt formation
Purine synthesis
Detoxification
Gives formyl THF

Question 2

glycine disorders

Answer 2

1. primary hyperoxaluria
   deficiency in enzymes that metabolize glyoxylate -> converted to oxalate which forms envelope shaped renal stones.
2. hyperglycemia
   defect in glycine cleavage system so too much glycine in blood - mental retardation bc glycine is inhibitory neurotransmitter
3. glycinuria
   renal fails to reabsorb glycine so too much glycine in urine. glycine goes to oxalate instead and makes renal stone.
   does nephrocalcinosis

Question 3

glutamic acid keto acid and functions of glutamate

Answer 3

alpha KG
1 A 2 G 3 S
1. activation of proteins ex: blood clotting factors
2. glutamate enters in structure of protein
3. glutathione synthesis
4. synthesis of arginine, proline and ornithine
5. synthesis of GABA
6. synthesis of glutamine by adding ammonia

Question 4

functions of glutamine and amide group of glutamine

Answer 4

1. protein synthesis
2. removes NH3 from brain
3. detoxification
4. increased during acidosis (makes urine basic)

amide group: PAPAN

1. purine synthesis
2. asparagine synthesis
3. pyrimidine synthesis
4. amino sugars synthesis
5. nicotinamide synthesis (B3)

Question 5

cysteine and cystine keto acid and functions

Answer 5

pyruvate
BACTP G
Bile Salt formation
Active site of enzymes
Cystine formation
Thioethanolamine
PAPS
Glutathione

Question 6

cystine and cystine diserases

Answer 6

1. cystinuria
   defect in transporting of dibasic amino acids : COLA
   so cystine goes to renal and forms hexagonal shape stones.
2. cystinosis
   lysosome storage diseases because no cystinosin. this makes cystine not leave lysosome so crystals form and damage the cell. result in death

Question 7

methionine keto acid and derivatives
carnosine
ethaolamine
guanidoacetate
and involves in synthesis of

Answer 7

alpha KB
anserine
choline
creatine
involved in spermine and spermidine which have + charge and bind with DNA to help with stabilization

Question 8

homocystinuria and hyperhomocystinuria
defect
cause
cysteine
treatment

symptoms

cystathioninuria

Answer 8

homo- defect in cystathionine synthase, genetic or from B6 deficiency
Cysteine becomes essenterial
give B6 and diet rich in cystine and poor in methionine

hyper- methionine synthase
genetic: deficiency of enzyme, b12, folic acid or MTHFR
cysteine is still non essential. treatment is folic acid & B12 and diet rich in methionine

symptoms: TDOM
thrombosis
dislocation of eyes
osteoprosis
mental retardation

cysta- genetic low activity of cystathionase or b6 deficiency so excess cystathionine in urine. Give VIT B6 and rich cysteine and poor methionine diet.