Protein and Amino Acid Metabolism III

Question 1

what are teh ways the body generates intermiedates

Answer 1

TCA cycle
converted into glucose
converted into keton bodies

Question 2

what are the amino acids that are purely ketogenic

Answer 2

leucine and lysine

Question 3

what is protein indicative of

Answer 3

low energy charge - catabolic state of body

Question 4

oxidative glutamate dehydrogenase reaction

Answer 4

glutamate goes into alphaketoglutatrate via glutamate dehdrogenase

Question 5

what is glutamate dehydrogenase regualted by

Answer 5

cellular energy chagne

Question 6

what inhibits glutamate dehydrogenase

Answer 6

GTP

NADH

Question 7

what stimulates glutamate dehydrogenase

Answer 7

ADP

Question 8

causes of familial hyperinsulinemia hypoglycemia type 6

Answer 8

mutant form of glutamate dehydrogenase that’s insensitive to inhibition by GTP

Question 9

symptom of HHF6

Answer 9

hypoglycermia

hyperammonemia

Question 10

what can be converted into pyruvate

Answer 10

alanine
serine
glycine
cysteinee

Question 11

what causes kidney stones

Answer 11

calcium oxalate precipitating in kidney tubules

Question 12

major route of glycine catabolism

Answer 12

mitochondrial glycine cleavage system

Question 13

where is the mitochondrial glycine cleavage system active

Answer 13

liver

Question 14

reaction of mitochondrial glycine cleaaver system

Answer 14

glycine + THF —-> N5, N10 methlene TNF

Question 15

is the mitochondrial glycine cleavage system reversible

Answer 15

yes

Question 16

glycine encephalophthy is caused by what

Answer 16

defect in glycine cleaavage system

Question 17

symptoms of glycine encephalophaty

Answer 17

lethargy, lac, o fmuscle tone, msucle twitching, death

Question 18

what can be used to make OAA

Answer 18

pyruvate, aspargine, asparate

Question 19

how is aspargine converted into asparate

Answer 19

asparaginase

Question 20

how is aspartate converted to OAA

Answer 20

AST

Question 21

what can be converted into alpha ketoglutarate

Answer 21

glutamate, glutamine, proline, arginine, ornithine, histdine

Question 22

what AA can be converted into propionyl CoA

Answer 22

isoleucine, threonine, methionine and valine

Question 23

what does the convertion of L methylmalonyl COA to succinyul CoA require as a coenzyme

Answer 23

vit B12

Question 24

what causes propionic acidemia

Answer 24

defect inpropinoyl CoA carboxylase

Question 25

what casues D methylmalonic aciduria

Answer 25

racemase deefect

Question 26

what causes methylmalonic acidura

Answer 26

defect in mutase of vit B12

Question 27

convertion of propionyl CoA to D methlmalonyl CoA requires what

Answer 27

biotin

Question 28

what is the ultimate source of vit B12

Answer 28

bacteria

Question 29

what is the best dietary sources of B12

Answer 29

meat, shellfish

Question 30

what does vit B12 bind to

Answer 30

intrinsic factor

Question 31

where does absorption of vit B12 happen

Answer 31

ileum

Question 32

once absorbed what does vit B12 bind to

Answer 32

transcobalamin

Question 33

what casues pernicious anemia

Answer 33

lack of intrinsic factor

Question 34

what is the only reaction that can convert N50methyl THF back to THF

Answer 34

conversion of homocysterine to methionine catalyzed by methionine synthase

Question 35

what does the convertino of N5 methyl THF back to THF require

Answer 35

vit B12

Question 36

what does vit B12 defiency result in

Answer 36

accumualtion of N5 mthyl THF

Question 37

what is N5N10 methylene THF required for

Answer 37

synthesis of thymidine and purine rings

Question 38

megaloblastic anemia

Answer 38

cells get super large because they can’t complete cell dvision