Biochem - Amino Acids

Question 1

Essential aas

Answer 1

PVT TIM HaLL

Phe
Val
Threonine

Trp
Isoleucine
Methionine

Histidine
Leucine
Lysine

Question 2

Basic aa

Answer 2

“HAL” +

Lysine and Arg – extra ammonia group
Arg and His – during periods of growth
Arg and Lys – nuclear localizing signals, high concentrations in histones

Histindine no charge at body pH

Question 3

Acidic aa

Answer 3

Aspartate and glutamate

Negative charge

Question 4

Arginine derivatives

Answer 4

Creatine
Urea
NO

Question 5

Vit B 6 cofactor dependent aa derivatives

Answer 5

Trp -> niacin (NAD+/NADP+)

Histidine -> histamine

Glycine -> porphyrin (to heme)

Glutamate -> GABA

Question 6

Trp derviatives

Answer 6

Niacine -> NAD+/NADP+ (need B6)

Serotonin -> melatoning (pineal gland)

Question 7

Glutamate derivatives

Answer 7

GABA (need B6)

Glutathione

Question 8

Deficiency of Ornithine transcarbamylase

Answer 8

Ornithine to citrulline

MC urea cycle disorder
X linked recessive
1st days of life

build up N and ammonia in body

Ornithine -> orotic acid in blood and urine
BUN low, can’t make urea

Sx:
Hyperammonemia – high ammonia levels
-> hepatoencephalopathy: slurred speech, somnolence, V, cerebral edema, blurring vision

Tx: low protein diet
Phenylbutyrate – binds gly and glutamine, helps excrete
Benzoate
Biotin – stimulates CPS1