Metabolism I

Question 1

beta ox location

Answer 1

mitochondria

Question 2

TCA cycle location

Answer 2

mitochondria

Question 3

ox phos location

Answer 3

mito

Question 4

HMP shunt location

Answer 4

cytoplasm

Question 5

glycolysis location

Answer 5

cytoplasm

Question 6

kinase

Answer 6

add P using ATP

Question 7

phosphorylase

Answer 7

add P without using ATP

Question 8

phosphatase

Answer 8

remove P

Question 9

dehydrogenase

Answer 9

catalyze redox rxn

Question 10

hydroxylase

Answer 10

add OH group

Question 11

carboxylase

Answer 11

transfer CO2 group

require biotin

Question 12

mutase

Answer 12

relocate functional group

Question 13

glycolysis rate limiting step

Answer 13

PFK-1

Question 14

gluconeogenesis rate limiting step

Answer 14

fructose 1,6 bisphosphatase

Question 15

TCA cycle rate limiting step

Answer 15

isocitrate DH

Question 16

glycogenolysis rate limiting step

Answer 16

glycogen phosphorylase

Question 17

HMP shunt rate limiting step

Answer 17

G6PD

Question 18

de novo pyrimidine synthesis rate limiting step

Answer 18

CPS II

Question 19

de novo purine synthesis rate limiting step

Answer 19

PRPP amidotransferase

Question 20

urea cycle rate limiting step

Answer 20

CPS I

Question 21

fatty acid synthesis rate limiting step

Answer 21

acetyl CoA carboxylase

ACC

Question 22

fatty oxidation rate limiting step

Answer 22

carnitine acyltransferase I

Question 23

ketogenesis rate limiting step

Answer 23

HMG CoA synthase

Question 24

choletserol synthesis rate limiting step

Answer 24

HMG-CoA reductase

Question 25

fructose 2,6 bisphoshate regulation

Answer 25

stimulate PFK-1 - glycolysis | inhibit fructose 1,6 - bisphosphatase - gluconeogenesis

Question 26

regulation isocitrate dehydrogenase

Answer 26

rate limiting step - TCA cycle inhibitor - ATP and NADH stimulation - ADP

Question 27

malate aspartate shuttle

Answer 27

heart and liver produce 32 ATP from glucose

Question 28

glycerol-3 phosphate shuttle

Answer 28

muscle produce 30 ATP from glucose

Question 29

arsenic

Answer 29

cause glycolysis produce 0 ATP

Question 30

vit B3

Answer 30

source of NAD+

Question 31

vit B2

Answer 31

source of FAD+

Question 32

NADPH production

Answer 32

HMP shunt

Question 33

hexokinase

Answer 33

most tissue - not liver and beta cells low Km low Vmax feedback inhibition - glucose 6 P

Question 34

mutation with maturity onset diabetes of young

Answer 34

glucokinase

Question 35

glucokinase

Answer 35

liver and pancreatic beta cells high Km high Vmax induced by insulin

Question 36

hexokinase vs. glucokinase

Answer 36

low glucose - hexokinase sequester in tissue high glucose - glucokinase store in liver

Question 37

fructose 2,6 biphosphate regulation

Answer 37

FBP-ase 2 and PFK-2 - part of same enzyme - PFK-2 produces fructose 2,6 biphosphate (stimulate PFK-1) - FBPase-2 produces fructose 6-P (gluconeogenesis) fasting state - glucagon - enzyme P - FBP-ase 2 activity - more gluconeogenesis fed state - insulin - enzyme de-P - PFK-2 activity - more glycolysis

Question 38

pyruvate dehydrogenase

Answer 38

links glycolysis and TCA cycle -active in fed state pyruvate > acetyl CoA requires cofactors - TLCFN

Question 39

TLCFN

Answer 39

cofactors ``` thiamine -B1 lipoic acid CoA - B5 FAD - riboflavin - B2 NAD - niacin - B3 ``` necessary for - pyruvate DH - a-KG DH - TCA cycle

Question 40

increased serum alanin, neuro deficit, lactic acidosis

Answer 40

deficient pyruvate DH complex build up of pyruvate - shunted to lactate and alanine production

Question 41

tx pyruvate DH deficiency

Answer 41

high fat diet high lysine and leucine - ketogenic AAs low glucose

Question 42

pyruvate has what possible pathways

Answer 42

alanine OAA acetyl CoA lactate

Question 43

irreversible enzymes of TCA cycle

Answer 43

citrate synthase isocitrate DH - rate limiting a-KG DH

Question 44

products of TCA cycle

Answer 44

3 NADH 1 FADH2 2 CO2 1GTP occurs 2x - 2 acetyl CoA formed per glucose

Question 45

TCA cycle location

Answer 45

mitochondria

Question 46

pyruvate carboxylase

Answer 46

pyruvate > OAA

Question 47

pyruvate dehydrogenase

Answer 47

pyruvate > acetyl CoA

Question 48

alanine aminotransferase

Answer 48

pyruvate > alanine

Question 49

lactic acid dehydrogenase

Answer 49

pyruvate > lactate