Biochemistry Metabolism 2

Front 1

site of FA synthesis

Back 1

cytosol

Front 2

site of FA breakdown

Back 2

mitochondria

Front 3

shuttle used in fa synthesis

Back 3

citrate shuttle

Front 4

shuttle used in fa breakdown

Back 4

carnitine shuttle

Front 5

citrate -> malate -> pyruvate shuttle

Back 5

citrate shuttle

Front 6

purpose is to shuttle AcoA to cytoplasm for fa synthesis

Back 6

citrate shuttle

Front 7

site of acoA formation

Back 7

mitochondria via pyruvate dehydrogenase

Front 8

AcoA + OAA ->

Back 8

citrate

Front 9

has ability to cross the membrane in fa synthesis by joining acco to OAA

Back 9

citrate

Front 10

once citrate enters cytoplasm from citrate shuttle, it can break down to these....

Back 10

OAA and AcoA

Front 11

after OAA is used in citrate shuttle, what does it become and where does it go?

Back 11

oaa -> malate -> pyruvate and then it reenters the mitochondria (in form of pyruvate)

Front 12

rate limiting step of f.a. synthesis

Back 12

AcoA carboxylase

Front 13

describe steps of fa synthesis

Back 13

-AcoA enters cytosol via citrate shuttle
-AcoA becomes malonyls CoA and is transferred to FAS (f.a. synthase)
-"OHOT" reactions occurs, and 2 c's of malonlylcoA are added to FA
-FAS is recharged with another malonyl coA and process continues.

Front 14

normal end product of fatty acid anything

Back 14

palmitic acid

Front 15

carbon # of palmitic acid

Back 15

16

Front 16

atp's needed for fa synthesis/breakdown

Back 16

c-1

Front 17

nadh's needed for fa synthesis/breakdown

Back 17

c-2

Front 18

round's needed for fa synthesis/breakdown

Back 18

c/2 - 1

Front 19

occurs when atp production gets toogh high, inhibiting pyruvate dehydrogenase and pfk1

Back 19

shunts to FA synthesis

Front 20

most # of c's body can make for a FA

Back 20

16 c's

Front 21

space between ='s in fa's

Back 21

3 c's apart

Front 22

last C with =

Back 22

C10

Front 23

no ='s

Back 23

saturated fa

Front 24

with ='s

Back 24

unsaturated FA

Front 25

shuttles LONG chain fatty acids from cytosol to mitochondria for fa breakdown

Back 25

carnitine shuttle

Front 26

needed as cofactor in malonylcoA formation

Back 26

biotin for AcoA carboxylase

Front 27

steps of carnitine shuttle

Back 27

-f.a. is enzymatically attached to carnitine and shuttled across mit. membrance to mitochondria.
-FA's are realeased for B-ox.

Front 28

defect of carnitine shuttle

Back 28

adrenal leukodystrophy

Front 29

steps of beta-oxidation

Back 29

-series of ox/hydration results in removal of 2-C groups as AcoA
-acoA -> kreb's
-if odd#, converted to propionyl coA and succinyl coA to enter krebs

Front 30

b-ox yields atps...

Back 30

1 nadh and 1 fadh2/turn = 5 atp
+ Acoa's that go to kreb's = 10 atp/molecule

Front 31

odd # FA's become.....in fa breakdown

Back 31

propionyl coA -> succinyl coA

Front 32

required with fatty acid breakdown of odd chained fa's

Back 32

biotin and b12

Front 33

inhibitor of carnitine shuttle

Back 33

malonyl coA

Front 34

stimulated beta oxidation

Back 34

epi

Front 35

inhibits beta oxidation

Back 35

insulin

Front 36

essential fatty acids

Back 36

linoeic and linolenic acid

Front 37

helps form arachodonic acid

Back 37

linoleic acid

Front 38

becomes essential if linoleic acid isn't present

Back 38

arachidonic acid

Front 39

dependent on essential fatty acids

Back 39

eiocosanoids

Front 40

compounds used to form ketone bodies

Back 40

fatty acids
amino acids

Front 41

ketones formed from ketogenesis

Back 41

acetone
acetoacetate
B-hydroxybutyrate

Front 42

ketone that can penetrate the brain

Back 42

B = beta hydroxybutyrate

Front 43

situations when ketone bodies form

Back 43

prolonged starbation
diabetic ketoacidosis

Front 44

ketone excretion

Back 44

urine

Front 45

rate limiting enzyme of ketogenesis

Back 45

hmgcoA synthase

Front 46

atp's needed for fa synthesis/breakdown

Back 46

c-1

Front 47

nadh's needed for fa synthesis/breakdown

Back 47

c-2

Front 48

round's needed for fa synthesis/breakdown

Back 48

c/2 - 1

Front 49

occurs when atp production gets toogh high, inhibiting pyruvate dehydrogenase and pfk1

Back 49

shunts to FA synthesis

Front 50

most # of c's body can make for a FA

Back 50

16 c's

Front 51

space between ='s in fa's

Back 51

3 c's apart

Front 52

last C with =

Back 52

C10

Front 53

no ='s

Back 53

saturated fa

Front 54

with ='s

Back 54

unsaturated FA

Front 55

shuttles LONG chain fatty acids from cytosol to mitochondria for fa breakdown

Back 55

carnitine shuttle

Front 56

needed as cofactor in malonylcoA formation

Back 56

biotin for AcoA carboxylase

Front 57

steps of carnitine shuttle

Back 57

-f.a. is enzymatically attached to carnitine and shuttled across mit. membrance to mitochondria.
-FA's are realeased for B-ox.

Front 58

defect of carnitine shuttle

Back 58

adrenal leukodystrophy

Front 59

steps of beta-oxidation

Back 59

-series of ox/hydration results in removal of 2-C groups as AcoA
-acoA -> kreb's
-if odd#, converted to propionyl coA and succinyl coA to enter krebs

Front 60

b-ox yields atps...

Back 60

1 nadh and 1 fadh2/turn = 5 atp
+ Acoa's that go to kreb's = 10 atp/molecule

Front 61

study ketogenesis on your own

Back 61

-

Front 62

study beta oxidation by yourself

Back 62

-

Front 63

only 2 organs that can mobilize glycogen into glucose

Back 63

adrenal cortex
kidney

Front 64

glycogenesis/lysis occurs here

Back 64

cytoplasm of muscle, liver, adipose, heart, adrenal cortex

Front 65

rapidly metabolizes glucose from glycogen during exercise

Back 65

skeletal muscle

Front 66

storage depot to maintain blood sugar at appropriate levels

Back 66

hepatocytes

Front 67

protein that is a precursor of glycogenesis

Back 67

glycogenin

Front 68

sugar carrier used with glycogen synthesis

Back 68

UDP

Front 69

rate limiting enzyme of glycogenesis

Back 69

glycogen synthase

Front 70

creates alpha 1,4 linkages

Back 70

glycogen synthase

Front 71

adds glucose units to nonreducing end of existing alpha 1,4 linkages

Back 71

glycogen synthesis

Front 72

transfers 7-glucose long residues from non-reducing ends of chain, creating intenal branches with alpha 1,6 linkages

Back 72

branching enzyme

Front 73

branching enzyme

Back 73

alpha 1,4 - alpha 1,6 glucosyl transferase

Front 74

allosteric activator of glycogen synthesis

Back 74

insulin

Front 75

allosteric inhibitor of glycogen synthesis

Back 75

glucagon/ epi (inhibits glycogen synthase)

Front 76

breaks alpha 1,4 linkages until 4c's before branch point

Back 76

glycogen phosphorylase

Front 77

breaks alpha 1,4's on branches chain

Back 77

debranching enzyme

Front 78

takes off final nub; breaks alpha 1,6 and takes off last glucose

Back 78

alpha 1,6 glucosidase

Front 79

coverts glucose-1-ph of glycogenolysis to glucose-6-ph

Back 79

phosphoglucomutase

Front 80

glucose-6-ph is coverted to glucose by....., which enters......

Back 80

glucose-6-phophorylase
glycolysis

Front 81

instead of alpha 1,6 glucosidase, the heart has.....

Back 81

alpha 1,4 glucosidase (yields free glucose directly)

Front 82

abnormal glycogen metabolism leading to accumulation of glycogen within cells

Back 82

glycogen storage diseases

Front 83

deficiency of glucose 6 phosphatase

Back 83

von gierke's type 1

Front 84

glucose 6 phosphatase deficieny affects these 2 organs

Back 84

liver
adrenal

Front 85

3-4 month old with hepatomegaly and hypoglycemia, "doll facies"

Back 85

type 1 glycogen storage disease = von gierke's

Front 86

alpha 1,4 glucosidase deficiency

Back 86

pompe's disease - type 2

Front 87

glycogen storage disease with earliest death

Back 87

pompe's

Front 88

inheritance of glycogen storage disease

Back 88

AR

Front 89

acid maltase aka

Back 89

alpha 1,4 glucosidase

Front 90

infant with hypertrophic cardiomyopathy, hypotonia, failure to thrive, macroglossia, hs-megaly

Back 90

pompe's disease - type 2

Front 91

deficient debranching enzym

Back 91

cori's disease

Front 92

debranching enzyme aka...

Back 92

alpha 1,6 blucosidase

Front 93

milder form of type 1 with normal blood lactate levels

Back 93

cori's

Front 94

on muscle biospy, see many short branches

Back 94

cori's

Front 95

deficient skeletal muscle phosphorylase

Back 95

mcardle's type 5

Front 96

see high glycogen in muscle, pain in muscles, rhabdomyolysis

Back 96

marcardle's

Front 97

sites in body of pentose phosphate pathway

Back 97

all sites of fatty acid or steroid synthesis -
lactating mammary glands
liver
adrenal cortex

Front 98

glucose may enter this pathway without any atp use of producation

Back 98

pentose phosphate pathway

Front 99

in pentose pathway, nadph produced is used for these function

Back 99

-fatty acid synthesis
-steroid synthesis
-glutathione reduction
-cytochrome p450

Front 100

2 products of pentose phophate pathway

Back 100

NADPH and
Ribose - 5 - ph

Front 101

an alternative route for glucose oxidation

Back 101

hmp shunt

Front 102

hmp shunt occurs here in the cellsis

Back 102

cytoplasm

Front 103

2 types of reactions that occur in hmp shunt

Back 103

oxidative and
nonoxidative

Front 104

irreversible part of hmp shunt

Back 104

oxidative reaction where glucose 6 ph -> ribose 5 phosphate

Front 105

reversible part of hmp shunt

Back 105

non-oxidative reaction where ribose 5 phosphate makes glyceraldehyde-3p and fructose-6-ph for entry into glycolysis

Front 106

rate limiting enzyme of hmp shunt

Back 106

glucose-6-phosphate dehydrogenase

Front 107

enzyme for non-oxidative part of hmp shunt

Back 107

transketolases

Front 108

transketolases require this vitamin to function

Back 108

thiamine

Front 109

ribose 5 ph may be used for these pathways

Back 109

ribose 5 ph -> nucleotide synthesis
g3p & F6p -> glycolytic intermediates

Front 110

necessary to keep glutathione reduced

Back 110

nadph

Front 111

detoxifies free radicals and peroxisomes

Back 111

glutathione

Front 112

low nadph in rbc's

Back 112

causes hemolytic anemia

Front 113

rbc defense against oxidizeing agents

Back 113

nadph

Front 114

sources that may oxidize rbc membranes

Back 114

fava beans
sulfonamides
primaquine
INH
antimalarials
metronidazole

Front 115

presents with pallor, hemoglobinuria, jaundice, and could develop after eating beans

Back 115

g6pd deficiency

Front 116

prevalent amount mediterranian people

Back 116

g6pd deficiency

Front 117

altered hb that precipitates within the rbc

Back 117

heinz bodies

Front 118

inheritance of g6pd deficiency

Back 118

x linked recessive

Front 119

increased malarial resistance

Back 119

g6pd deficiency

Front 120

triglyceride synthesis begins with this compound

Back 120

glycerol-3-ph

Front 121

g-3-p in triglyceride synthese originates from these pathways

Back 121

DHAP - glycolysis
from glycerol - glycerol kinase

Front 122

backbone for all phospholipids

Back 122

phosphatidic acid

Front 123

carrier complex for lipids

Back 123

CDP

Front 124

phos. acid + CDP-choline ->

Back 124

phosphatidyl choline/lecithin

Front 125

bridges 2 g-3-p

Back 125

cardiolipin

Front 126

multiple spontaneous abortions

Back 126

cardiolipin AD syndrome

Front 127

physical sign of triglyceridemia

Back 127

xantholesma

Front 128

triglyceridemia increases the risk of.....

Back 128

pancreatitis

Front 129

mc causes of pancreatitis

Back 129

alcohol
gallstones
trauma

Front 130

backbone of sphingolipids

Back 130

sphingosine

Front 131

ceramide + udp-glucose ->

Back 131

cerebroside

Front 132

ceramide + dolichol ->

Back 132

ganglioside