amino acids

Question 1

Valine, Leucine, Isoleucine

Answer 1

Branched chain
MSUD (auto rec) - branched-chain alpha-keto acid dehydrogenase complex BCKDC enzyme deficiency
requires TLCFN cofactors (thiamine, lipoic acid, CoA, FAD (B2), NAD (B3)
Amish population!

Question 2

Glycine

Answer 2

Collagen
every 3rd a.a.
enables triple helix formation

Question 3

Alanine

Answer 3

major gluconeogenesis precursor

Question 4

Proline

Answer 4

collagen
hydroxylated while still in fibroblast by proline hydroxylase
deficiency due to vit C def or Ehrler-Danlos

Question 5

Phenylalanine

Answer 5

PKU
phenalanine dehydroxylase def - uses THB as cofactor
must supplement with tyrosine, RESTRICT (not omit) Phe

Question 6

Tyrosine

Answer 6

Alkaptonuria, albinism

target of tyrosine kinase

Question 7

Lysine, Arginine, Histadine

Answer 7

Positively charged
Lysine and Arginine more than histadine
as a result Lys and Arg components of Histones
His in active site of enzymes
Arg is urea precursor, NO precursor

Question 8

Aspartate

Answer 8

Urea cycle

Question 9

Glutamate

Answer 9

GABA precursor

Question 10

Serine, Threonine

Answer 10

targets of protein kinases, hydroxylated

O-glycosylation (post-transcriptional in Golgi)

Question 11

Cysteine

Answer 11

maintain tertiary structure of proteins with disulfide bonds

powerful reductants

Question 12

Methionine

Answer 12

SAM methyl donor

req B12 and THF for conversion back from L-homocysteine

Question 13

Asparagine

Answer 13

N-glycosylation (co-transcriptional in ER)

Question 14

Glutamine

Answer 14

NH3 carrier for excretion by kidney

no change in pH when ammonia group attached

Question 15

Essential?

Answer 15

PVT TIM HALL
phe, val, thr, trp, iso, met, his, arg (for growing child, preg), leu, lys

PKU? all + tyrosine

Question 16

Tryptophan

Answer 16

serotonin, niacin precursor