Xexam3 Mfm45

Front 1

fatty acids are the major source of fuel when:
1.
2.
3.

Back 1

1. in between meals
2. when fasting (e.g. overnight)
3. periods of physical exertion

Front 2

fatty acids are a fuel source for what kind of tissue:
1.
2.
3.

Back 2

skeletal muscle
cardiac muscle
liver

Front 3

fatty acids generate (more/less) fuel compared to glucose

Back 3

more

Front 4

dietary fatty acids are transported from intestine in the form of lipoprotein particles ... and ...

Back 4

chylomicrons
VLDL

Front 5

when fatty acids reach their target cells, they are cleaved from VLDL and the free fatty acid diffuses across the cell membrane where 2 things can happen:
1.
2.

Back 5

1. used immediately for energy
2. converted into fatty acyl CoA

Front 6

fatty acyl CoA combines with ... to from ... so it can be stored for later use as fuel for the body

Back 6

glycerol-3-phosphate
triglyceride

Front 7

excess glucose can be converted into ... and ...

this is stimulated by the hormone ...

Back 7

acetyl CoA
glycerol-3-phosphate
insulin

Front 8

look at the figure and use a ↑ or ↓ to indicate what is going on with the below listed hormones and enzymes:

glucagon / (insulin)
cAMP levels in adipocytes
protein kinase A (PKA)
hormone-sensitive lipase

Back 8

↑ glucagon (↓insulin)
↑ cAMP levels in adipocytes
↑ protein kinase A (PKA)
↑ hormone-sensitive lipase

Front 9

put the following in the correct order:

↑ cAMP levels in adipocytes
↑ glucagon (↓insulin)
↑ hormone-sensitive lipase
↑ protein kinase A (PKA)
fasting

Back 9

fasting
↑ glucagon (↓insulin)
↑ cAMP levels in adipocytes
↑ protein kinase A (PKA)
↑ hormone-sensitive lipase

Front 10

what are the function of lipases:

Back 10

to relase fatty acids from triglycerides

Front 11

lipases are ... sensitive

Back 11

hormone

Front 12

free fatty acids diffuse out of adipose cell and into the bloodstream where they travel attached to ...

Back 12

albumin

Front 13

glycerol derived from lipolysis enters bloodstream and can be used by the liver for ...

Back 13

gluconeogenesis

Front 14

Fatty acids enter cells through two routes:
1.
2.

Back 14

diffusion
transport via fatty acid binding protein (FaBP)

Front 15

where is the fatty acid binding protein located:

Back 15

plasma membrane

Front 16

fatty acids are ultimately oxidized to ... and ...

Back 16

CO2
H2O

Front 17

acetyl-CoA produced through β-oxidation of fatty acids in the liver can be:
1.
2.

Back 17

1. converted to ketone bodies
2. enter the TCA Cycle.

Front 18

where does β-oxidation of fatty acids occur:

Back 18

mitochondria

Front 19

in β-oxidation of fatty acids, free fatty acids are converted into ... before they cross the outer membrane of the mitochondria

Back 19

fatty acyl CoA

Front 20

... shuttles fatty acyl CoA across the ... membrane

Back 20

carnitine
mitochondrial inner

Front 21

what is the enzyme the catalyzes the following reaction:

fatty acid --> fatty acyl CoA

Back 21

acyl CoA synthetase + ATP

Front 22

what can we use fatty acyl CoA for:

1.
2.
3.

Back 22

1. energy
2. membrane lipids
3. storage

Front 23

carnitine comes from ... or can be synthesized in the ... from ... and

Back 23

red meats/dairy
liver
lysine

Front 24

synthesis of carnitine requires ... and ...

Back 24

vitamin C
S-adenosylmethionine (SAMe)

Front 25

... is on the outer mitochondrial membrane and is the rate-limiting enzyme (inhibited by malonyl CoA)

transfers the fatty acyl group to carnitine, forming fatty acylcarnitine, which is then translocated into the mitochondrial matrix

Back 25

Carnitine Palmitoyl Transferase I (CPT I)

Front 26

... on the inner mitochondrial membrane

transfers the fatty acyl group back to CoASH (re-forming the fatty acyl CoA) and releases the carnitine for recycling

Back 26

Carnitine Palmitoyl Transferase II (CPT II)

Front 27

identify the 3 enzymes labeled a, b, and c

Back 27

(see figure)

Front 28

what are the 3 most common diseases associated with carnitine metabolism:
1.
2.
3.

Back 28

1. Carnitine or carnitine uptake deficiency
2. CPT I deficiency
3. CPT II deficiency

Front 29

what happens in persons with carnitine deficiencies:

Back 29

inability to transport fatty acids into the mitochondria

Front 30

who is afflicted with carnitine deficiences:

Back 30

occurs in newborns (particularly pre-term infants), patients undergoing hemodialysis, patients with organic acidemia

Front 31

what are the symptoms in carnitine deficiencies:

Back 31

muscle weakness, muscle cramping, cardiomyopathy, hepatomegaly, encephalopathy, sudden death

Front 32

CPT I Deficiency is a (sex-linked/autosomal) (dominant/recessive) defect in CPT I gene that primarily affects ... in the liver

Back 32

autosomal
recessive
fatty acid oxidation

Front 33

the symptoms of CPT I Deficiency usually present after a period of ... or after a ... illness:

the symptoms include:
1.
2.
3.
4.

Back 33

fasting
gastrointestinal
1. hypoketosis and hypoglycemia
2. lethargy
3. seizures
4. coma

Front 34

CPT II Deficiency is a (sex-linked/autosomal) (dominant/recessive) defect in CPT II gene and has ...(number) subtypes

Back 34

autosomal
recessive
3

Front 35

the 3 subtypes of CPT-II deficiency are:
1.
2.
3.

Back 35

1. myopathic CPT-II deficiency (most common)
2. hepatic CPT-II deficiency (similar to CPT I)
3. multiorgan

Front 36

what are the symptoms of myopathic CPT-II deficiency:
1.
2.
3.

Back 36

1. adolescence or adulthood onset
2. recurrent muscle pain, fatigue, and myoglobinuria (particularly following exercise)
3. often misdiagnosed (fibromyalgia, chronic fatigue)

Front 37

The β-Oxidation Spiral is where ... are sequentially cleaved from ...

Back 37

2-carbon acetyl CoA units
fatty acyl group

Front 38

what are the 4 repeating steps in fatty acid oxidation:
1.
2.
3.
4.

Back 38

1. oxidation
2. hydration
3. oxidation
4. cleavage

Front 39

what are the 4 repeating steps in fatty acid synthesis:
1.
2.
3.
4.

Back 39

1. condensation
2. reduction
3. dehydration
4. reduction

Front 40

what is the most important (rate-limiting) step in fatty acid oxidation and what is the enzyme that catalyzes the reaction:

Back 40

1st oxidation
aceyl CoA dehydrogenase

Front 41

in oxidation, a ... is inserted into the fatty acid

Back 41

double bond

Front 42

there are several acyl CoA dehydrogenases, each works on a specific chain length. the most important is ... because it is most involved in pathologies and it works on fatty acids that are ... carbons in length

Back 42

MCAD - medium chain acyl CoA dehydrogenase
6-12

Front 43

what are the most frequently diagnosed of the fatty acid oxidation disorders

Back 43

MCAD Deficiency

Front 44

what accounts for about 1 in 100 SIDS deaths

Back 44

MCAD Deficiency

Front 45

... usually presents clinically between second month and second year of life following a period of fasting (even overnight fasting) or illness

Back 45

MCAD Deficiency

Front 46

how do you prevent Deficiency symptoms:

Back 46

frequent feedings, high-carb/low-fat diet

Front 47

...% of MCAD Deficiency patients will die with their first episode of illness

Back 47

20-25

Front 48

how would you manage MCAD Deficiency:
1.
2.
3.

Back 48

1. eat regularly, avoid fasting
2. diet high in carbs, low in medium and long chain fatty acids
3. oral carnitine

Front 49

where is the energy coming from in β-oxidation

Back 49

FADH2 and NADH from the 2 oxidation steps in the β-oxidation spiral

Front 50

the most common fatty acids used in β-oxidation are:
1.
2.
3.
4.

and they are the most common becuase:

Back 50

1. palmitate
2. stearate
3. oleate
4. linoleate

most common dietary fatty acids and also commonly synthesized in humans