Review Session Tidbits

Question 1

ACC is active when (phosphorylated/dephosphorylated)

Answer 1

dephosphorylated (by insulin stimulated phosphatase)

Question 2

phosphorylation of ACC by glucagon, epi, NE causes:

Answer 2

ACC to become inactive, can’t make malonyl CoA in FA synthesis

Question 3

cofactor used in ACC

Answer 3

biotin

Question 4

residue for attachment in Fatty Acid Synthase

Answer 4

phosphopantetheinyl residue

Question 5

most common desaturase enzyme

Answer 5

delta 9

Question 6

(aspirin/acetaminophen)=irreversible inhibition of COX 1 and COX 2 in cyclic pathway from arachidonic acid to prostaglandin and thromboxane

Answer 6

aspirin

Question 7

(cyclic/linear/cytochrome P450) pathway from arachidonic acid makes leukotrienes, involved in contraction of smooth muscle in the lungs

Answer 7

linear. leukotrienes can be inhibited by drugs to relax asthma spasms

Question 8

add which amino acid to IMP to get AMP and fumarate?

Answer 8

aspartate

Question 9

phospholipase (A2/D/C) cuts a phospholipid into a fatty acid and a glycerol-phosphate-head group-fatty acid

Answer 9

A2 (precursor to arachidonic acid

Question 10

which phospholipase is inhibited by glucocorticoids

Answer 10

A2 (prevents formation of arachidonic acid

Question 11

which phospholipase takes just the head group off the phospholipid

Answer 11

D

Question 12

which phospholipase takes the head group and phosphate off the phospholipid

Answer 12

C

Question 13

which phospholipase cleaves a phospholipid into phosphatidic acid/DAG, which stimulates PKC, and IP3, which stimulates calcium release?

Answer 13

C

Question 14

which phospholipase is especially important to cell signaling, cleaves off phosphoserine

Answer 14

D

Question 15

lysosomal storage disorder that is rapid, fatal neurodegeneration. GM2 ganglioside builds up

Answer 15

Tay Sachs

Question 16

lysosomal storage disorder def beta hexo aminidase A

Answer 16

Tay Sachs

Question 17

lysosomal storage disorder with hepatosplenomegaly, neurodegen, build up of sphingomyelin

Answer 17

Niemann Pick

Question 18

lysosomal storage disorder with def sphingomyelinase

Answer 18

Niemann Pick

Question 19

lysosomal storage disorder with neuro degen, skeletal deformities, hepatosplenomegaly, build up of GM1 and keratan sulfate

Answer 19

GM1 gangliosidosis

Question 20

lysosomal storage disorder with def beta galactosidase

Answer 20

GM1 gangliosidosis

Question 21

most common lysosomal storage disorder

Answer 21

Gaucher

Question 22

lysosomal storage disorder with hepatosplenomegaly, osteoporosis, treat with enzyme replacement therapy

Answer 22

Gaucher

Question 23

lysosomal storage disorder with build up of gluco cerebrosides

Answer 23

Gaucher

Question 24

lysosomal storage disorder with def beta glucosidase

Answer 24

Gaucher

Question 25

lysosomal storage disorder like Tay Sachs, with visceral involvement

Answer 25

Sandhoff

Question 26

lysosomal storage disorder with def beta hexo aminidase

Answer 26

Sandhoff

Question 27

lysosomal storage disorder with build up of GM2 and globosides

Answer 27

Sandhoff

Question 28

lysosomal storage disorder--X linked, skin rash, kidney and heart failure, burning pain in legs

Answer 28

Fabry's

Question 29

lysosomal storage disorder with build up of globosides and def alpha galactosidase

Answer 29

Fabry's

Question 30

lysosomal storage disorder with painful joint deformity, hoarse cry, lipid nodules under skin

Answer 30

Farber

Question 31

lysosomal storage disorder with ceramide build up and ceramidase def

Answer 31

Farber

Question 32

lysosomal storage disorder with demyelination, paralysis

Answer 32

Metachromatic Leukodystrophy

Question 33

lysosomal storage disorder with build up of sulphatides and def aryl sulfatase

Answer 33

Metachromatic Leukodystrophy

Question 34

lysosomal storage disorder with build up of galactocerebrosides and def beta galactosidase

Answer 34

Krabbe-Globoid cell Leukodystrophy

Question 35

lysosomal storage disorder with blindness, deafness, deterioration

Answer 35

Krabbe-Globoid cell Leukodystrophy

Question 36

(choline/amine) containing phospholipids are on the inner surface

Answer 36

amine

Question 37

(choline/glycolipids/amine) containing phospholipids are on the outer surface of the cell membrane

Answer 37

choline and glycolipids

Question 38

asymmetric cell membrane phospholipids maintained by (2 things)

Answer 38

flippases and ATP

Question 39

what type of linkage does amylase cleave

Answer 39

internal 1,4 alpha Glu-Glu

Question 40

how is glycogen different from amylopectin

Answer 40

same structure but much more branching

Question 41

which two steps in glycolysis have substrate level phosphorylation?

Answer 41

phosphoglycerate kinase and enolase--CREATE energy

Question 42

what enzyme is responsible for "glucose" entering the glycogen pathway

Answer 42

phosphoglucomutase (G6P G1P)

Question 43

what enzyme removes glucose from the terminal end of glycogen by cleaving the bond with a phosphate

Answer 43

glycogen phosphorylase

Question 44

major control enzyme for TCA cycle

Answer 44

isocitrate DHase

Question 45

NADH and ATP (activate/inhibit) isocitrate DHase

Answer 45

inhibit

Question 46

ADP (activates/inhibits) isocitrate DHase

Answer 46

activates

Question 47

pentose phosphate shunt takes place in the (mit/cytoplasm)

Answer 47

cytoplasm

Question 48

___ is formed from ornithine and then transported out of the mit and converted along with Asp into Arginosuccinate

Answer 48

citrulline

Question 49

ornithine is transported (into/out of) the mit

Answer 49

into

Question 50

treat (Hartnup's/Cystinuria) with penicillamine and increased fluid intake

Answer 50

Cystinuria

Question 51

treat (Hartnup's/Cystinuria) with niacin (B3)

Answer 51

Hartnup's

Question 52

a defect in Homogentisate Oxygenase results in ______, inability to metabolize Phe and Tyr, black cartilage build ups

Answer 52

alkaptonuria

Question 53

defective branched chain keto acid DHase results in which disease

Answer 53

maple syrup urine disease

Question 54

pyruvate (DHase/carboxylase) converts pyruvate into acetyl CoA, so excess acetyl CoA inhibits it

Answer 54

DHase

Question 55

pyruvate (DHase/carboxylase) produces OAA which condenses with acetyl CoA to form citrate and is stimulated by excess acetyl CoA

Answer 55

carbosylase

Question 56

in the presence of glucocorticoids, hepatic gluconeogenesis is (increased/decreased)

Answer 56

increased

Question 57

Deficiency of hypoxanthine-guanine phosphoribosyl transferase can lead to (Uric acid accumulation/ orotic acid accumulation)

Answer 57

uric acid accum. HGPRT deficiency. Also causes behavioral changes. Lesch Nyhan syndrome

Question 58

Methotrexate inhibits (ribonucleotide reductase/dihydrofolate reductase)

Answer 58

dihyrofolate reductase

Question 59

hydroxyurea inhibits (ribonucleotide reductase/dihydrofolate reductase)

Answer 59

ribonucleotide reductase